Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene

 Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene

Waqas Tahir , Sandor Dudas , Renee Anderson , Jianmin Yang , Sarah Bogart , Kristina Santiago-Mateo, Yuanmu Fang & Roberta Quaghebeur

Pages 36-49 | Received 20 Feb 2025, Accepted 22 May 2025, Published online: 04 Aug 2025 Cite this article https://doi.org/10.1080/19336896.2025.2511933

ABSTRACT

Bovine Spongiform Encephalopathy (BSE) is a fatal neurodegenerative disease in cattle which can be either classical BSE (C-BSE) or atypical BSE (including H-BSE and L-BSE). Here, we report the results of our analyses of an H-BSE case found in Canada in 2021, indicating restriction of the pathological agent (PrPSc) mainly to the central nervous system with no or occasional weak involvement of peripheral tissues. Importantly, a non-synonymous mutation at codon 211 of the PRNP gene was detected and confirmed to be present as a germline mutation. This is the first case of BSE in Canada with a predisposing E211K mutation.

Introduction

The transmissible spongiform encephalopathies (TSEs) or prion diseases are fatal neurodegenerative diseases affecting both humans and animals [Citation1]. TSEs may occur sporadically, genetically or be acquired and are caused by a conformational change of a host encoded cellular prion protein (PrPC) into its pathological isoform called the scrapie prion protein (PrPSc) [Citation2]. This conformational shift to PrPSc leads to its deposition and aggregation in the central nervous system (CNS), resulting in neuronal loss and eventually death. Bovine spongiform encephalopathy (BSE) is a TSE of cattle. The first case of BSE (referred to as classical BSE; C-BSE) was identified in the UK in 1986 [Citation3] which evolved into an epidemic affecting more than 2 million cattle in the UK [Citation4]. Dietary exposure to C-BSE was later found to be the most likely cause of variant form of Creutzfeldt – Jacob disease (vCJD), an acquired form of prion disease in humans [Citation5,Citation6], indicating its zoonotic potential. The origin of the C-BSE epidemic in the UK was attributed to the recycling of TSE infected meat and bone meal (MBM) in animal feed [Citation7,Citation8]. This notion was supported by the reduction in the number of C-BSE cases worldwide when a ‘feed ban’ to eliminate MBM in animal feed was implemented in the affected countries. However, in 2004, two additional ‘atypical’ types of BSE, with different neuropathological and molecular characteristics from previous cases of C-BSE emerged in France and Italy [Citation9,Citation10]. These two atypical types of BSE were later designated as L-type (L-BSE) and H-type (H-BSE) based on the biochemical features of PrPSc (either lower or higher molecular weight un-glycosylated PrP in L-BSE and H-BSE respectively, compared to C-BSE) [Citation11,Citation12]. Since then, many other countries have reported atypical BSE cases. The exact cause of atypical BSE is unknown, however it is considered sporadic and linked to the old age of cattle since atypical BSE cases are mostly detected in cattle more than 8 years old with a few exceptions such as a Swiss H-BSE case in a cow of 6.5 years of age and a Japanese L-BSE case in a cow of 23 month old [Citation13,Citation14]. Moreover, a steady occurrence of atypical BSE cases, despite the feed ban, also favours their sporadic nature [Citation15].

The pathogenic PrPSc levels and distribution in various brain regions [Citation16] and peripheral tissues can be variable between C-BSE and atypical forms of BSE. Considering the public health concerns associated with BSE, it is highly pertinent to characterize any newly identified BSE case for the spatial pattern of PrPSc. Such information can help to determine if the distribution of the pathogenic PrPSc is the same as in previously reported cases or if there has been an evolution in prion strain biology and disease phenotype in newly identified BSE cases. Additionally, a set of genetic risk factors including a 23-bp indel in the promoter region, a 12-bp indel in intron 1 or an increase in the number of octa-peptide repeats (OPRs) on the N-terminal [Citation17–19] of the prion protein (PRNP) gene have also been identified to be associated with BSE cases. In 2006, the US reported a H-BSE case associated with a novel mutation in the bovine PRNP gene leading to a switch of Glutamic acid (E) to Lysine (K) at amino acid residue 211 of PrPC – referred to as the E211K mutation [Citation18]. The E211K mutation in the PRNP gene of cattle is analogous to the E200K mutation in the most prevalent genetic form of human TSE; genetic Creutzfeldt-Jakob’s disease (gCJD) [Citation20]. This E211K mutation in cattle was later found to be heritable [Citation21] and associated with an increased susceptibility to H-BSE [Citation22]. These findings underscore the importance to evaluate the presence of genetic risk factors in any newly identified case of BSE to aid in designing or modifying mitigation strategies to prevent future BSE cases.

Canada has reported 3 cases of atypical BSE (in addition to 17 cases of C-BSE) to date, with 1 case of L-BSE (in December 2007) and 2 cases of H-BSE (first one in July 2006 and the most recent in December 2021). There were no genetic risk factors or pathogenic mutations identified in the first two atypical BSE cases in Canada [Citation23]. Here, we report the results of our analyses of the most recent H-BSE case (Canadian BSE case 20), where we investigated, i) the spatial distribution of PrPSc to assess any changes in specified risk material (SRM), and ii) the genetic risk factors contributing to the sporadic disease onset in this case of H-BSE.

Materials and methods

Animal information

In December 2021, an 8-year-old beef cow of Red Poll breed from central Alberta, under the fallen stock stream of Canada and Alberta BSE surveillance programme, tested positive for BSE. The clinical presentation included loss of body weight, abnormal posture, ataxic and uncoordinated or exaggerated gait, and falling down or stumbling. The initial BSE surveillance testing of the brain stem from the cow was performed at a network laboratory in Alberta, Canada using World Organization for Animal Health (WOAH) approved rapid surveillance test for BSE. After repeating initial results, the sample was referred to the Canadian BSE Reference Lab; BSE-RL (Canadian Food Inspection Agency, National Center for Animal Disease, Lethbridge Laboratory, Lethbridge, Alberta, Canada) for confirmatory testing. After the sample tested positive at the BSE-RL, the obex from that cow was further analysed for the type of BSE by immunoblotting, presence of vacuolation by histopathology (H&E) and deposition of PrPSc by immunohistochemistry (IHC). After confirmation and typing, the carcass of the cow (Canadian BSE case 20) was brought to the BSE-RL for additional sample collection. Upon arrival at the BSE-RL, the carcass was decomposing, but all available tissues were harvested and analysed by IDEXX HerdChek (Bovine Spongiform Encephalopathy-Scrapie Antigen Test Kit, EIA), H&E and IHC for the presence and distribution of the pathological agent. Additionally, DNA from various tissues of the animal was also analysed to identify genetic variation in the PRNP gene.

Snip…

Identification of E211K mutation in the PRNP gene of the Canadian BSE case 20

To determine if the Canadian BSE case 20 was associated with any mutation in the PRNP gene, the PRNP gene was amplified from DNA extracted from brainstem and sequenced. The sequence alignment with a known bovine PRNP sequence showed that the Canadian BSE case 20 had a functional PRNP gene indicated by no mutation in either start or stop codon. An increase in the number of octa-peptide repeat regions on N- terminal of the PRNP gene has been shown to enhance susceptibility to BSE in transgenic rodent models [Citation17], however both alleles contained six octa-peptide repeats (OPRs), indicative of the wild type bovine genotype. However, sequence analysis of the coding region revealed a synonymous mutation at codon 192 (AAC/AAT > N192N) and a non-synonymous mutation at codon 211 (GAA/AAA > E211K) (Supplementary Figure S4 and Figure 4). The change in the nucleotide sequence at codon 211 from GAA to AAA resulted in a switch of amino acid sequence at codon 211 from glutamic acid (E) to lysine (K) amino acid. As this change in the nucleotide sequence from GAA to AAA was present on one allele of the PRNP gene only, so E211K mutation in the Canadian BSE 20 was heterozygous in nature. The finding of the E211K mutation was of significant interest because an analogous mutation, E200K, at codon 200 in human PRNP gene is associated with genetic CJD in humans [Citation28].

Figure 4. DNA sequencing results of the PRNP gene of the Canadian BSE case 20. DNA sequence chromatograms of the PRNP gene from A) brain stem, B) retropharyngeal lymph node, C) quadriceps, and D) tongue of the Canadian BSE case 20. The site of mutation is highlighted by a boxed area with a black arrow. Encoded amino acid sequence is mentioned at the bottom of each chromatogram by capital letters indicating a change of amino acid code from GAA to AAA resulting in a switch of amino acid from glutamic acid (E) to lysine (K). DNA sequence chromatograms are shown for forward (top) and reverse (bottom) primers for each tissue tested.

Figure 4. DNA sequencing results of the PRNP gene of the Canadian BSE case 20. DNA sequence chromatograms of the PRNP gene from A) brain stem, B) retropharyngeal lymph node, C) quadriceps, and D) tongue of the Canadian BSE case 20. The site of mutation is highlighted by a boxed area with a black arrow. Encoded amino acid sequence is mentioned at the bottom of each chromatogram by capital letters indicating a change of amino acid code from GAA to AAA resulting in a switch of amino acid from glutamic acid (E) to lysine (K). DNA sequence chromatograms are shown for forward (top) and reverse (bottom) primers for each tissue tested. Display full size The change in the amino acid sequence at codon 211 of the bovine PRNP gene has been shown to increase the susceptibility of cattle to H-BSE making it a pathogenic mutation in cattle. The E211K mutation was previously reported in an American H-BSE case in 2006 [Citation18], which was later found to be genetically inherited to its offspring (a heifer) [Citation21] indicating that the US E211K mutation is a germ line mutation. To determine if the E211K mutation identified in the Canadian BSE case 20 was a somatic or germline in nature, additional tissues were analysed. The DNA sequencing results on retropharyngeal lymph node, tongue and quadriceps also showed a change in the nucleotide sequence at codon 211 resulting in the E211K mutation (Figure 4). These results strongly suggest that E211K in the Canadian BSE case 20 was present as a germline mutation. Due to the poor condition of the carcass at post-mortem sample collection, neither blood nor gonadal tissues were available for further analyses.

Discussion

Here we describe a case of H-BSE confirmed in December 2021 in Canada (referred to as the Canadian BSE case 20). This was an 8-year-old dairy cow of Red Poll breed, born and raised in the C-BSE affected region in Canada. However, it was born after the reinforced feed ban (BARB) on feeding meat and bone meal to ruminants which supported the notion that this was not a C-BSE case. The laboratory confirmatory tests also verified that this was an H-BSE case based on the higher molecular weight of unglycosylated PrP in comparison to C-BSE and L-BSE controls, both before and after PNGase F treatment. Further analyses of glycoform ratios and the presence of a fourth band in the Canadian BSE case 20 were also consistent with the H-BSE control, as well as previously reported cases of H-BSE (Table 4) in the literature [Citation26]. Immunoreactivity with an N-terminal anti-PrP P4 antibody and increased sensitivity to stringent PK digestion conditions also confirmed this to be an H-BSE case (Table 4). These results were further supported by the presence of intra-neuronal, intra-microglial, and absence of linear and stellate PrPSc immunostaining pattern, which are characteristic features of H-BSE cases [Citation29]. Immediately, after the confirmation and typing of the Canadian BSE case 20, a notification was sent to WOAH and all the activities required for compliance with WOAH standards were conducted to ensure that the carcass was secured on the farm from the time of euthanasia until laboratory confirmation of BSE status and type. The carcass was then transported to the Canadian BSE RL for necropsy and incineration following post-mortem sample collection. During these activities, no part of the carcass of the Canadian BSE case 20 was rendered or entered the human food nor animal feed chain.

Table 4. Summary of comparison of characteristics of Canadian BSE cases to date.

Download CSVDisplay Table Speculating about the distribution of PrPSc in the peripheral tissues of any atypical BSE case is difficult because published data on the tissue tropism of PrPSc in cattle affected by atypical BSE are limited. However, this information is important for developing a good understanding of the evolution in prion strain biology and disease phenotype in newly identified BSE cases, particularly for atypical BSE cases. According to experimental transmission studies of atypical BSE, PrPSc has been reported in peripheral ganglia and nerves, muscles (muscle spindles), adrenal glands and retina for both H-BSE and L-BSE. No lymphoid tissues or gastrointestinal tissues have previously tested positive in atypical BSE cases [Citation30–34]. In the Canadian BSE case 20, the peripheral nervous system (PNS) including optic nerve was found to be 8.8% positive (relative to the obex) in IDEXX-EIA whereas the trigeminal ganglion and vagus nerve showed granular coarse PrPSc staining only in occasional neurons, as confirmed with two anti-PrP antibodies. However, brachial nerve plexus and sciatic nerve, which have been previously shown to be positive for PrPSc during experimental challenge studies and in natural cases [Citation35], were found to be negative in both IDEXX-EIA and IHC in the Canadian BSE case 20. Additionally, all lymphoid tissues as well as endo/exocrine glands examined were also negative in both IDEXX-EIA and IHC. Furthermore, striated muscles (including tongue and masseter muscles), nasal and buccal cavities, conjunctiva and ileocaecal junction also appeared negative in both tests. Amongst the muscles tested, the quadriceps and biceps brachii were negative in IDEXX-EIA; but showed inconclusive results in IHC. Overall, these results indicate that the distribution of the pathological agent is mainly restricted to the CNS with no or occasional weak involvement of the peripheral tissues in the Canadian BSE case 20. This also suggests the unlikely involvement of any new or unusual tissues during the disease pathogenesis of this Canadian BSE case 20, indicating no concerns that would require the revision of existing mitigation strategies nor the list of specified risk materials.

Regarding the origin of H-BSE cases, most of those reported worldwide have been sporadic except for one in the 2006 US H-BSE case associated with the E211K mutation [Citation18]. Canada has reported a total of 20 cases of BSE to date which included 17 C-BSE, 1 L-BSE, and 2 H-BSE cases. The only L-BSE case was identified in December 2007. The first case of H-BSE was identified in July 2006 and the latest case in December 2021.

To determine the involvement of host genetic risk factors, every field case of BSE is sequenced to analyse mutations in the PRNP gene. None of the previously reported pathogenic variations such as, i)-E211K mutation, ii)-23-bp indel in the promoter region, iii)-12-bp indel in intron 1, or iv)-increase in the number of OPRs on the N-terminal [Citation17–19] of the PRNP gene that are linked with enhanced susceptibility to BSE have been found in the first 19 BSE cases (including the first two atypical BSE cases) in Canada. However, synonymous mutations at codon 78 (Q78Q; CAG/CAA), 113 (P113P; CCC/CCT) and 192 (N192N; AAC/AAT), which have no known association with BSE, have been previously identified (Table 4) in some Canadian BSE cases [Citation23].

The DNA sequencing results of the Canadian BSE case 20 also showed no variation in the number of OPRs of the PRNP gene just like the previous Canadian BSE cases indicating a very low risk of such genetic variations in the Canadian cattle herd. Similarly, none of the synonymous mutations at codon 23 (L23L; CTC/CTA or CTG), 78 (Q78Q; CAG/CAA), 113 (P113P; CCC/CCT) and 185 (N185N; AAC/AAT), which were reported previously in the French, American, Swedish, Canadian and Portuguese H-BSE cases [Citation23,Citation35–38], were identified in the Canadian BSE case 20. Though a synonymous mutation at codon 192 (N192N; AAC/AAT) was detected in the Canadian BSE case 20, it was not a major concern because this mutation was also described previously in above mentioned studies and has no association with BSE. However, the detection of the E211K mutation in the Canadian BSE case 20 raises concerns because the E211K mutation has been associated with an increased susceptibility to H-BSE [Citation22] and is genetically inheritable to its offspring [Citation21], indicating that the E211K mutation could result in additional BSE cases.

This is the first time that the E211K mutation has been found in a Canadian BSE case. Additionally, the detection of the E211K mutation in multiple tissues strongly suggested that the E211K mutation in the Canadian BSE case 20 is a germline mutation. This implies that the E211K mutation in the Canadian BSE case 20 is most likely heritable and may be present in 50% of the offspring of this cow. However, it is difficult to speculate from where the Canadian BSE case 20 acquired the E211K mutation. The mutation could be either spontaneous or inherited from a parent. Possible theories about the origin of this E211K mutation in the Canadian BSE case 20 could include that, i) this cow acquired E211K mutation spontaneously in its early embryonic development, ii) germ cells from one of its parents acquired this mutation spontaneously, iii) ancestors of this cow had this mutation in their line, or iv) this mutation was present in a population of this breed. To explore these possibilities, a strategic testing of various cattle breeds (including Red Poll) from different geographic regions within Canada is important and being planned. However, none of its ancestors were traceable or available for testing to explore if the Canadian BSE case 20 acquired the E211K mutation from its ancestors. On the other hand, irrespective of the detection of the E211K mutation in this Canadian cow, there will always be a slight possibility for the E211K mutation to occur independently in any cattle. Nonetheless, the presence of the E211K mutation in cattle makes them highly susceptible to develop H-BSE [Citation22]. While strong BSE mitigation measures such as specified risk material removal, and ruminant to ruminant feed bans play a major role in preventing future BSE cases, the potential for animals with the E211K mutation and an increased risk of developing atypical BSE in the Canadian cattle population cannot be ruled out.

The E211K mutation associated with the US H-BSE case was heritable [Citation21] and associated with an increased susceptibility to H-BSE, represented by a shorter incubation time in cattle with K211 allele during the experimental challenge studies [Citation22,Citation39], and suggested a higher propensity for the K211 prion protein to misfold. Identification of E211K mutation in the 2006 US and 2021 Canadian H-BSE cases represent independent existence of this pathogenic mutation in time and space. This is further supported by the comparative analysis (Supplementary Table S1) of both cases showing minor dissimilarities, though both cases were confirmed as H-type BSE. Those dissimilarities included the presence of 4th band of PK resistant PrP at 10 kDa in western-blot and a synonymous mutation at codon 192 of the PRNP gene in Canadian BSE cases 20 only. Additionally, proven heritability and germline nature of E211K mutation in the 2006 US and 2021 Canadian H-BSE cases respectively, indicate familial nature of E211K mutation just like familial Creutzfeldt Jakob’s disease in humans. This highlights the importance of screening each identified case for genetic risk factors specifically and cattle population to determine the prevalence rate of E211K mutation in general, to eradicate this pathogenic mutation form the national herd.

After the detection of E211K mutation in the US H-BSE case in 2006, DNA testing of cattle populations was conducted in various countries including USA (n = 6062 cattle tested) [Citation40], China (n = 349) [Citation41], Poland (n = 105) [Citation42], Pakistan (n = 236 cattle and n = 281 buffalo) [Citation43] and Korea (n = 536) [Citation44], but no E211K mutation could be detected in any sample. This indicates that the E211K mutation in cattle is very rare. Notably, all the previously mentioned studies have largely analysed blood samples with only occasional testing of muscle or brain tissues to detect the E211K mutation, which might not be detected if it was present as a somatic or postzygotic mutation within a particular tissue. Recently, Won SY and colleagues novelly reported that the E211K mutation was present only in the medulla oblongata of three Holstein Korean slaughter cattle, was present at high rates and was a somatic mutation. In silico analyses of the biological impact of the E211K mutation in these cattle predicted that E211K was damaging in nature despite being a somatic mutation [Citation45]. Somatic mutations in the PRNP gene at codon D178N in sporadic CJD and P102L associated with sporadic Gerstmann – Sträussler – Scheinker (GSS) syndrome have also been documented in humans [Citation46,Citation47]. This indicates that the E211K mutation can be pathogenic irrespective of whether it is germline or somatic in origin. However, PrPSc was not detected in the medulla oblongata of the three Holstein Korean cattle carrying E211K as a somatic mutation, using highly sensitive in-vitro amplification assays including real-time quaking-induced conversion reactions (RT-QuIC) and protein misfolding cyclic amplification (PMCA) [Citation48]. Though more objective evidence is required to explore the possibility that a somatic E211K mutation can cause H-BSE, results from Kim YC and colleagues show that the level of somatic mutation required to induce H-BSE could be a determining factor. On the other hand, it could be argued that the three Holstein Korean cattle carrying a somatic E211K mutation had an average age of less than 5 years, and they might have acquired atypical BSE if they were let to live longer. While scientific evidence exist that E211K mutation increases susceptibility to H-BSE, however it is difficult to objectively conclude that E211K mutation is a bona fide mutation responsible for the onset of H- BSE. In this regard, a long-term project is already underway by the USDA to investigate the possibility of a genetic aetiology for H-BSE in cattle expressing the K211 allele. Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.

Supplemental material Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene

KEYWORDS:

Atypical BSEBovine Spongiform Encephalopathycentral nervous systemE211K mutationprion diseasesprion protein genesynonymous mutation

https://www.tandfonline.com/doi/full/10.1080/19336896.2025.2511933#d1e1606

“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”

See Canadian 2021 Mad Cow 

SATURDAY, DECEMBER 18, 2021 

CFIA Canada Alberta Laboratory detection of atypical bovine spongiform encephalopathy 

https://bovineprp.blogspot.com/2021/12/cfia-canada-alberta-laboratory.html

MONDAY, JUNE 09, 2025 

The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE) The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)

Component 6: Transmissible Spongiform Encephalopathies (TSEs)

Problem Statement 6A: Determine pathobiology of prion strains.

The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).

Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa

Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.

https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/Final%20NP103%20FY2024%20Annual%20Report.updated%205.30.25.pdf

Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation

Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University

Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.

Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.

Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.

DOI: https://doi.org/10.1080/19336896.2022.2091286

Interpretive Summary:

Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-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 the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).

This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. 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.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351

Highlights

This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. 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.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351

Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211 cattle following intracranial inoculation

Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin

Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.

A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211 cattle following intracranial inoculation.

Frontiers in Veterinary Science. 3:78. Interpretive

Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.

Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle 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 BSE-H 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 BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=326785

Atypical BSE in cattle

THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.

Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.

Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.

Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous

VET RECORD | 29 March–12 April 2025

disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.

The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8

Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.

In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.

Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.

Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.

“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”

Timm Konold, TSE lead scientist

Brenda Rajanayagam, workgroup leader for the data systems group

APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk

Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX

References

1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)

Atypical BSE In Cattle

https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1002/vetr.5400?campaign=woletoc

THURSDAY, JUNE 5, 2025

World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible, what could go wrong?

https://bovineprp.blogspot.com/2025/06/world-organisation-for-animal-health.html

Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle

Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, * Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada. *Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca

Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.

Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.

Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.

Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.

Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.

Presentation Type: Oral Presentation Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute

Grant Number: ALMA/APRI: 201400006, HC 414250

Acknowledgement: TSE unit NCAD, Lethbridge (Jianmin Yang, Sarah Bogart, Rachana Muley, Yuanmu Fang, Keri Colwell, Renee Anderson, John Gray, Rakhi Katoch) (CFIA, Canada), Dr. Catherine Graham (NSDA, Canada), Dr. Michel Levy (UCVM, Canada), Dr. Martin Groschup (FLI, Germany), Dr. Christine Fast (FLI, Germany), Dr. Bob Hills (Health Canada, Canada) Theme: Animal prion diseases

"After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE. "

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PRION 2023 CONTINUED;

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure

Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA

Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.

Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).

Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.

Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.

Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.

"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

2023

OIE Conclusions on transmissibility of atypical BSE among cattle

Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.

https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf

Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019

34 Scientific Commission/September 2019

3. Atypical BSE

The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.

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

http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf

Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle

Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *

Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.

*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca

Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.

Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.

Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.

Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.

Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.

Presentation Type: Oral Presentation

Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute

Grant Number: ALMA/APRI: 201400006, HC 414250

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

Molecular phenotype shift after passage of low-type bovine spongiform encephalopathy (L-BSE).

Zoe J. Lambert, M. Heather West Greenlee, Jifeng Bian, Justin J. Greenlee Ames, USA

Aims: The purpose of this study is to compare the molecular phenotypes of L-BSE in wild type cattle and cattle with the E211K polymorphism to samples of other cattle TSEs, such as classical BSE (C-BSE), hightype BSE (H-BSE), and transmissible mink encephalopathy (TME).

Materials and Methods: Two wild type cattle (EE211 PRNP) and one steer with the E211K polymorphism (EK211) were intracranially inoculated with 1 mL of brain homogenate that originated from a 2005 French L-BSE case. Multiple assays were used to compare and differentiate tissues, including enzyme immunoassay, western blot (Sha31, 12B2, SAF84), stability (Sha31), and immunohistochemistry (F99/97).

Results: Approximately 16.6 months post-inoculation, Steer 6 (EK211 L-BSE) developed neurologic signs, including agitation, difficulty eating accompanied by weight loss, head tremor, ataxia, and fasciculations in the forelimbs, and was necropsied. Enzyme immunoassays demonstrated misfolded prion protein in the brainstem (4.0 O.D) but not in peripheral tissues, such as the retropharyngeal lymph node and palatine tonsil. When compared by western blot, the molecular phenotype of the brainstem of Steer 6 (EK211 L-BSE) is higher than that of wildtype cattle inoculated with L-BSE, requiring careful differentiation from C-BSE. Ongoing mouse studies in bovinized mice (K211 and TgBov) will provide data to compare to all other BSE strains available, including L-BSE, C-BSE, H-BSE, E211K H-BSE, and TME.

Conclusions: Further study of L-BSE in EK211 cattle with a higher molecular phenotype in the brainstem may give more insight into the origin of C-BSE.

Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript. This research was supported in part by an appointment to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE.

Grant number: DOE contract number DE-SC0014664 Acknowledgements: NA Theme: Animal prion diseases

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PRION 2023 CONTINUED;

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion

https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article

Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/

Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/

Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate

Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata

Affiliations expand

PMID: 21266763

Abstract

A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.

https://pubmed.ncbi.nlm.nih.gov/21266763/

see full text;

https://www.niid.go.jp/niid/images/JJID/64/81.pdf

THURSDAY, JUNE 25, 2020

First Report of the Potential Bovine Spongiform Encephalopathy (BSE)-Related Somatic Mutation E211K of the Prion Protein Gene (PRNP) in Cattle

https://bovineprp.blogspot.com/2020/06/first-report-of-potential-bovine.html

WEDNESDAY, AUGUST 15, 2018

The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challenge

http://bovineprp.blogspot.com/2018/08/the-agent-of-h-type-bovine-spongiform.html

PRION 2018 CONFERENCE

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

https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094

https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf

WEDNESDAY, OCTOBER 24, 2018

Experimental Infection of Cattle With a Novel Prion Derived From Atypical H-Type Bovine Spongiform Encephalopathy

https://bse-atypical.blogspot.com/2018/10/experimental-infection-of-cattle-with.html

WEDNESDAY, OCTOBER 24, 2018

Experimental Infection of Cattle With a Novel Prion Derived From Atypical H-Type Bovine Spongiform Encephalopathy

https://bse-atypical.blogspot.com/2018/10/experimental-infection-of-cattle-with.html

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.

http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156

http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF

THE last two mad cows documented in the USA were in Alabama and Texas, both of which were atypical h-BSE.

SINGE then, the surveillance for TSE in cattle in the USA has been reduced to a number of which detecting any TSE would almost impossible.

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end

https://www.upi.com/Health_News/2006/03/15/Analysis-What-that-mad-cow-means/UPI-12841142465253/ 

In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

http://web.archive.org/web/20080521165659/http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm

PLEASE SEE EVIDENCE 

imo, THAT THE USDA ET AL COVERED UP MAD COW DISEASE IN TEXAS ; PAGE 43; Section 2. Testing Protocols and Quality Assurance Controls 

snip... 

FULL TEXT 130 PAGES 

http://web.archive.org/web/20080920075242/http://www.usda.gov/oig/webdocs/50601-10-KC.pdf

TUESDAY, JULY 18, 2017

USDA announces Alabama case of Bovine Spongiform Encephalopathy Alabama

http://bovineprp.blogspot.com/2017/07/usda-announces-alabama-case-of-bovine.html

2012 

ATYPICAL L-TYPE BASE BSE TSE PRION CALIFORNIA ‘confirmed’ 

Saturday, August 4, 2012

*** Final Feed Investigation Summary - California BSE Case - July 2012

http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/final-feed-investigation-summary.html

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

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)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

TUESDAY, AUGUST 22, 2006

BSE ATYPICAL TEXAS AND ALABAMA UPDATE JANUARY 20, 2007

https://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

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

https://www.nature.com/articles/4571079b.pdf

https://www.nature.com/articles/4571079b

Epidemiological investigations conducted by USDA personnel failed to reveal any evidence of a feed source contaminated with TSE material fed to this animal

http://www.aphis.usda.gov/newsroom/hot_i​ssues/bse/downloads/EPI_Final5-2-06.pdf

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2525843/

''This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. 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.''

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

______________________________

http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.html

http://web.archive.org/web/20080229052729/http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.html

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

###

http://www.fda.gov/bbs/topics/ENFORCE/2006/ENF00964.html

http://web.archive.org/web/20070223174152/http://www.fda.gov/bbs/topics/ENFORCE/2006/ENF00964.html

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

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html

http://web.archive.org/web/20060821195949/http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html

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

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00960.html

http://web.archive.org/web/20070223180551/http://www.fda.gov/bbs/topics/enforce/2006/ENF00960.html

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

http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm

http://web.archive.org/web/20091104111717/http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm

***> The U.S. cases were animals born and raised in the U.S. (Texas, Alabama).

SEE HISTORY AT THE BOTTOM...TSS

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

THURSDAY, OCTOBER 18, 2007

BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA, A REVIEW OF SORTS

http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html

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)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY, what if?

BSE Case Associated with Prion Protein Gene Mutation

http://bovineprp.blogspot.com/2015/05/bse-case-associated-with-prion-protein.html

WEDNESDAY, AUGUST 15, 2018

***> The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challenge

http://bovineprp.blogspot.com/2018/08/the-agent-of-h-type-bovine-spongiform.html

From: TSS 

Subject: Transcript of Tele-News Conference regarding The inconclusive BSE Rapid Test Result With Chief Veterinary Officer Dr. John Clifford, March 13, 2006 

Date: March 14, 2006 at 10:31 am PST

Transcript

Release No. 0084.06 Contact: Office of Communications (202) 720-4623

Transcript of Tele-News Conference regarding The inconclusive BSE Rapid Test Result With Chief Veterinary Officer Dr. John Clifford, March 13, 2006 MR. JIM ROGERS: Hi, everybody. This is Jim Rogers with the Animal Plant Health Inspection Service Legislative and Public Affairs Office. I appreciate you all calling in today. We have here the USDA chief veterinarian, Dr. John Clifford. He is also known as the deputy administrator for the Veterinary Services Program under APHIS. And at this time I will turn the call over to him.

DR. CLIFFORD: Thanks, Jim. Thanks, everybody for joining us this afternoon. We received a positive result on a Western blot confirmatory test conducted at our USDA laboratories in Ames, Iowa, on samples from an animal that had tested inconclusive on a rapid screening test performed on Friday, March 10.

The samples were taken of a nonambulatory animal on a farm in Alabama. A local private veterinarian euthanized and sampled the animal and sent the samples for further testing, which was conducted at one of our contract diagnostic laboratories at the University of Georgia.

The animal was buried on the farm and did not enter the animal or human food chains. We are now working with Alabama Animal Health officials to conduct an epidemiological investigation to gather any further information we can on the herd of origin of this animal.

The animal had only resided on the most recent farm in Alabama for less than a year. We will be working to locate animals from this cow's first cohort and any offspring. We will also work with Food and Drug Administration officials to determine any feed history that may be relevant to the investigation.

Experience worldwide has shown us that it's highly unusual to find BSE in more than one animal in a herd or in affected animal's offspring. Nevertheless, all animals of interest will be tested for BSE.

Under USDA's testing protocols, surveillance samples are sent to contract laboratories for screening tests. If the sample is found to be inconclusive on a screening test, it is then shipped to our National Veterinary Services Laboratory in Ames, Iowa, for an additional rapid test and two confirmatory tests-- the immunohistochemistry test which is conducted by APHIS scientists, and the Western blot test which is conducted by scientists with USDA's Agricultural Research Service.

USDA considers an animal positive if either of these two confirmatory tests returns a positive result. In this instance the inconclusive result from the contract lab in Georgia was confirmed through a second rapid test at NVSL. Now the Western blot test has returned a positive result, and that is sufficient for us to confirm this animal to be positive for BSE, which is why we are making the announcement today.

The IHC tests are still pending, and we will release those results as soon as they are available, which we expect to be later this week.

I want to emphasize that human and animal health in the United States are protected by a system of interlocking safeguards and we remain very confident in the safety of U.S. beef.

Again, this animal did not enter the human food or animal feed chains while epidemiological work to determine the animal's precise age is just getting underway and is ongoing, the attending veterinarian has indicated that based on dentition it was an older animal, quite possibly upwards of 10 years of age.

This would indicate that this animal would have been born prior to the implementation of the Food and Drug Administration's 1997 feed ban.

Older animals are more likely to have been exposed to contaminated feed circulating before FDA's '97 ban on ruminant-to-ruminant feed practices which scientific research has indicated is the most likely route for BSE transmission.

By any measure the incidence of BSE in this country is extremely low. Our enhanced surveillance program was designed as a one-time snapshot to provide information about the level of prevalence of BSE in the United States. Since June 2004 all sectors of the cattle industry have cooperated in this program by submitting samples from more than 640,000 animals from the highest risk populations and more than 20,000 from clinically normal older animals as part of our enhanced BSE surveillance program.

To date, including the animal in today's announcement, only two of these highest risk animals have tested positive for the disease, as part of our enhanced surveillance program.

As we approach the conclusion of our enhanced surveillance program, let me offer a few thoughts regarding surveillance going forward. I can assure you that we will continue to base our maintenance surveillance testing on international guidelines. Though the nature and extent of maintenance surveillance has not yet been finalized, the incidence of BSE in this country remains extremely low and our interlocking safeguards are working to protect both human and animal health, and we remain very confident in the safety of U.S. beef.

As we move forward with the epidemiological investigation that's been initiated today in this case of BSE, we will continue to be very transparent in sharing information with the public and with our trading partners around the world.

With that, I'm happy to take any questions that you may have.

OPERATOR: At this time we are ready to begin the question and answer session. If you'd like to ask a question, please press *1 on your touch-tone phone. You will be announced prior to asking your question and to withdraw your question you will press *2.

Our first question comes from Beth Grohem (sp). Your line is open.

REPORTER: Yes. Hi, there. I'm with the Canadian Press. Thanks for taking my question. You said you were investigating the herd of origin. I'm wondering if there's any idea yet whether the animal was born in Canada or the United States.

DR. CLIFFORD: Thank you for the question. At this time we don't, we'd not be able to indicate whether it's of U.S. origin or Canadian origin. As I'd indicated, the animal was really at this particular location for less than a year, and it will require us to complete our investigation before we can determine the actual farm of origin or birth origin.

MR. ROGERS: Next question, please.

OPERATOR: Catherine Hunter, your line is open.

REPORTER: Hi, there. I was wondering if you could tell me how you expect this result to impact your ongoing negotiations to open up the Japanese import beef market.

DR. CLIFFORD: We would not anticipate that this would impact our ongoing negotiations. As I'd indicated our product is safe, we've got a number of interlocking safeguards, and Japan themselves has had 20-plus cases of BSE. And we believe their product is safe with regards to the safeguards they've in place in that country. We have a ruminant-to-ruminant feed ban to protect animal health in this country as well.

MR. ROGERS: Next question, please.

OPERATOR: Peter Shinn, your line's open.

REPORTER: Yes, thank you. Peter Shinn with the National Association of Farm Broadcasting. My question is simply, at what point do you think that you will know more relative to specifics about this animal's life? Do you have any kind of timeline?

DR CLIFFORD: We'll work as quickly as we can. I really can't give you a specific timeline, but I can tell you that we're very transparent with our information, and as soon as we have the completion of that and more information we can share we will certainly do that.

MR. ROGERS: Next question, please.

OPERATOR: Philip Brasher, your line's open.

REPORTER: Yes. Can you say anything about the breed of this animal? Do you have any idea how many farms it was on? And what's the state of the record keeping associated with this animal?

DR. CLIFFORD: I missed the second part of that, that the breed itself -- it's a beef breed. It's actually a Santa Gertrubis, has been identified. As far as the record keeping, I can't really speak to any of the record keeping at this point in time other than the fact that we know this animal was at this location for less than a year.

MR. ROGERS: Next question, please.

OPERATOR: Pete Heisey, your line's open.

REPORTER: Hi. How about the effect on trade with South Korea? They've announced since this turned positive that they were likely to suspend their reopening, which is scheduled for a month from now.

DR. CLIFFORD: Again we would hope this wouldn't affect any trade. We have a number of safeguards based upon the estimation of age and based on dentition from the private veterinarian this animal should have been born before the feed ban went into place, and we have effective safeguards in the U.S. in the ruminant-to-ruminant feed ban as well as SRM removal.

So we would not anticipate that it would affect trade and as well, we would request other countries and our trading partners as well as ourselves to move to the international standards and OIE guidelines that are based upon safe trade in commodities.

MR. ROGERS: Next question, please.

OPERATOR: Jeff Wilson, your line's open.

REPORTER: Yes. I'd like to know whether or not it's standard practice to bury any of these dead animals on the farm at all times or are they supposed to be incinerated, or is there some other means of disposal?

DR. CLIFFORD: Actually with regards to disposal methods, there are several different possibilities and options. But with regards to burying on-farm, I think that's probably specific to state issue and possibly EPA guidelines as well with regards to particular states. So I couldn't speak to the state itself.

But with regards to disposal methods we use for animals, we would use incineration, burial in lime type of burial sites as well as alkaline tissue digestion. So there's more than one method that would be available.

MR. ROGERS: Before we go to the next question, Operator, I'd like to ask everybody please state your organization that you represent before asking your question. Next question, please.

OPERATOR: Chris Clayton, your line's open.

REPORTER: Hi. I'm with DTN in Omaha, Nebraska. I wanted to just clarify the animal had died on the farm, and that was this animal already had been buried or was in the process of being buried? And have you quarantined the farm that it was on? And what kind of herd was that, how big size, that sort of thing?

DR. CLIFFORD: This particular animal, as I'd indicated, was at this farm for less than a year. Obviously they did not get, would not have been born on that farm; so therefore would not likely to have other animals of interest. So therefore it's not necessary to quarantine the farm. But whether or not it is quarantined or not I'd have to check with state officials to see if they have put a quarantine in place.

With regard to the status of the animal itself, the animal was nonambulatory. I believe the animal was treated by the veterinarian initially and the veterinarian returned on the following day and actually euthanized the animal and took the sample.

MR. ROGERS: Next question, please.

OPERATOR: Daniel Goldstein, your line's open.

REPORTER: Yeah, hi, Dr. Clifford. Dan Goldstein with Bloomberg. You said that this animal is going to go through the IHC test or the brain tissue's going to go through the IHC test. Are there any plans to send the brain tissue to Weybridge, England, for a second confirmatory IHC test?

DR. CLIFFORD: No, there's not. We don't feel that would be necessary, and as I've indicated we use both now-- the Western blot as well as the IHC. And either one of those findings to be positive, we consider that to be a positive result. So we don't feel the need in sending the sample to Weybridge.

MR. ROGERS: Next question, please.

OPERATOR: Elizabeth Weiss with USA Today. Your line's open.

REPORTER: Hey. It's Beth Weiss with USA Today. So a 10-year-old. Can you spell the name of that breed, or at least say it slowly?

DR. CLIFFORD: Santa Gertrubis. I may have get the exact spelling. It's been awhile since I've spelled it. I think it's, the first name's Santa, which is S-A-N-T-A. And the last one is G-E-R-T-R-U-B-I-S.

MR. ROGERS: All right. Spelling questions. I guess that means we're going to probably wrap it up in about two more questions, Operator. Next question, please.

OPERATOR: Wyatt Andrews with CBS News. Your line's open.

REPORTER: Thanks. It's Wyatt Andrews from CBS. Doctor, could you go in a little deeper about how you were going to look for the offspring of this cow? I mean obviously it's a cow; she's 10 years old. She clearly must have had quite a bit of offspring. First of all, do you have the records to track all the young calves that this cow had over her lifespan, number one? And number two, would those cows be presumptively contagious, and how do you look for BSE in them?

DR. CLIFFORD: Let me start by talking about records. Basically we'll have to do an epi investigation as I indicated. This particular cow was on this farm for less than a year, so therefore we will have to do an epi investigation, which will require us to try to trace her back to her farm of origin.

Once we've determined the farm of origin and then through that we would determine what other locations she may have been as well. We would determine through that then if we're able to determine that we will determine animals of interest. We would go back to the farm of origin and talk to that particular owner about what records they have or may have relative to time of birth and her first year of life on that farm.

The offspring issue, let me point out while we would still trace her two last offspring if we're able to identify those animals, it's very highly unlikely and extremely rare that either of those animals would even have the potential of having BSE while it is part currently of the OIE code, there's little science that supports that that disease is transmitted from the dam to the offspring while in the womb. So basically there's very little -- or we'll have to complete our epi investigation before we can give you more details relative to that.

MR. ROGERS: Last and final question, please, Operator?

OPERATOR: Elizabeth Lee with Atlanta Journal Constitution, your line's open.

REPORTER: Hi. It's Elizabeth Lee with the Atlanta Journal Constitution. I wanted to ask about the conclusion of the expanded surveillance program, if you could talk a little bit about when that is supposed to end and what the proposals are, how many fewer animals might be tested going forward?

DR. CLIFFORD: I think, we indicated in our statement that I'd given earlier you know as we talk about the conclusion of our enhanced surveillance program I wanted to reiterate and state that program was to take a snapshot in time to give us an estimate of prevalence.

Having said that, we will be continuing to do a level of surveillance for a long period of time within the U.S. So we will make sure that those standards meet international standards. We'll be working with scientists and others and having input in that, but at this point in time the nature and extent of that surveillance program has not yet been finalized. And when we do that we'll certainly share that publicly.

MR. ROGERS: I'd like to thank everybody very much for attending the call today. Just a few points of clarification. There's been some confusion out there about the number of inconclusives that have been found under the enhanced surveillance program. That number is four.

All of that testing data is available on our website at WWW.APHIS.USDA.GOV.

There's a picture of a cow down at the bottom, click on that and it will tell you everything you need to know.

And before we go, Dr. Clifford has one final statement.

DR. CLIFFORD: I also want to clarify for you there may be some confusion relative to the number of cases found within the U.S. for BSE. The Washington state cow, which was a Canadian origin animal, was actually found prior to our enhanced surveillance effort. So that's why when we talk about two cases, that's during the enhanced surveillance program.

MR. ROGERS: Then I guess just because I always have to have the last word, the testing numbers are now over 650,000, the new numbers are posted today.

Thank you everybody. There will be a transcript posted on our website as soon as we can make one available. This concludes our call.

Last Modified 3/14/2006

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB/.cmd/ad/.ar/sa.retrievecontent/.c/6_2_1UH/.ce/7_2_5JM/.p/5_2_4TQ/.d/2/_th/J_2_9D/_s.7_0_A/7_0_1OB?PC_7_2_5JM_contentid=2006%2F03%2F0084.xml&PC;_7_2_5JM_navtype=RT&PC;_7_2_5JM_parentnav=TRANSCRIPTS_SPEECHES&PC;_7_2_5JM_navid=TRANSCRIPT#7_2_5JM

WONDER if it was typical BSE or atypical BSE/BASE/TSE ??? where is the pathology, or is burying them on the farm and no weybridge going to be the norm now?

must give them credit for confirming the cow though, i am still in shock over that. ...TSS

2025, PLEASE NOTE, the above link told me ;

Forbidden

You don't have permission to access this resource.

Rather infuriating if you ask me…terry

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

03-025IFA 03-025IFA-2 Terry S. Singeltary

Page 1 of 17

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

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

To: fsis.regulationscomments@fsis.usda.gov

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

for the Disposition of Non-Ambulatory Disabled Cattle

Greetings FSIS,

I would kindly like to submit the following to [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and

Requirements for the Disposition of Non-Ambulatory Disabled Cattle

THE BSE/TSE SUB CLINICAL Non-Ambulatory Disabled Cattle

Broken bones and such may be the first signs of a sub clinical BSE/TSE Non-Ambulatory Disabled Cattle ;

snip...FULL TEXT ;

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

http://web.archive.org/web/20060316114732/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

=====

From: TSS 

Subject: TRANSCRIPT FROM ALABAMA AI COMMISSIONER RON SPARKS ON BSE (a must see) 

Date: March 14, 2006 at 11:39 am PST

March 13, 2006 - Second BSE Results Show Positive, Cow on Alabama Farm MONTGOMERY – Agriculture & Industries Commissioner Ron Sparks has confirmed that a second test for bovine spongiform encephalopathy (BSE) is positive. The cow was a downed animal and did not enter the human or animal food supply. The samples were taken from a cow that was on an Alabama farm within the last year.

http://www.agi.state.al.us/press_releases

March 13, 2006 - Second BSE Results Show Positive, Cow on Alabama Farm

Videos from yesterdays BSE news conference: WATCH AND LISTEN TO THIS LOAD OF BSeee, BEEF IS SAFE PR at it's finest. NO more animals are at risk on this farm? COW was 10 years old, and in Alabama for less than a year? was it ever in TEXAS and did it feed from TEXAS? mama cow. only transmitted through spinal cord and brain, firewalls in place and working. cannot be passed on to other cattle, and be very clear, IS NOT IN MUSCLE. NO COWS EATING THIS PARTICULAR TYPE FOOD. she was a DOWNER. it is not a contagious disease, no way any of the other animals can contact this disease. absolutely as safe today as it is tomorrow. nvCJD bull sh!t, and cjd in the USA is different. safe, safe, safe, safe, safe i bet this guy says it 20 times. lets compliment the cattleman, this guy, that guy, enhanced surveillance is working all the time (he does not have a clue), call in, more pats on the back. (this is the biggest hoot i have heard in a while, you must listen, safe, safe, safe). we been talking about bse in Alabama for a few years, and i have to be honest, i feel good about finding it, firewalls in place. not under quarantine, but no animals will go or come for now. not contagious. muscle tissue is safe again. cow is not shedding any organism that is dangerous to other cows. somebody in background whispering answers to commissioner sparks. Alabama food supply is safe. safe safe safe. animals cannot eat materials that they ate in 1997.............huh????? again, safe, safe, safe. (holy mad cow, these folks are oblivious) Canada has same feed ban, safe, safe, safe (commissioner is more whispers and answers, he is oblivious). we will possibly give the location of the farm later. for the security of the farmer, we will not release that information now. consumers by 93% in food supply, they vote with there pocket book (it's time the consumer showed them finally just what that is all about, show them we demand safer beef, TEST ALL CATTLE...TSS). another person speaking about the firewalls, and no risk to human health. random testing, why would i test you for flu, if bse is not contagious. clear message. transmission of cow to cow is not possible. speaker confuses himself on program and has to get someone else, he is not sure either, but insists brain and spinal is not going into feed chain. there is a testing for normal animals, we tested 20,000. (WHOOPY...tss)

Windows Media Player:

http://video1.adph.state.al.us/alphtn/newscasts/agnewscast.wmv.asx

Real Player:

http://video1.adph.state.al.us/alphtn/newscasts/agnewscast.ram

“I was very concerned to find out that the samples that tested positive for BSE were from a cow in Alabama, but this is exactly the reason that we emphasis the importance of BSE surveillance,” said Sparks. “The cow was tested as part of the enhanced BSE surveillance program that has been in place in Alabama,” said Sparks. “Even cows brought in from other states get tested for BSE before they would have a chance to be sold as food. I cannot stress enough how important this testing is to protect consumers. Also, having the Premises ID program in place in Alabama means we are able to trace the origin of a diseased animal. The cattle producers of Alabama understand the need for these precautions as well and we will continue to work together closely to protect consumers.”

The cow had been purchased by an Alabama producer and was examined and treated by a local veterinarian. After failing to respond to medication, the cow was humanely euthanized by the veterinarian and a routine sample was collected to test for BSE. Following an inconclusive test result from a rapid BSE test, the samples were tested at the National Veterinary Services Laboratories in Ames, Iowa, . The Western blot test produced a positive result. A third test, the immunohistochemistry test, is in progress and will be completed later this week. BSE is not a contagious disease that spreads animal to animal, or animal to human. BSE spreads in cattle through feed containing meat and bone meal derived from BSE infected cattle. The United States banned the use of such protein supplements in cattle feed since 1997. Commissioner Sparks stresses that beef consumption in this country is safe and there are measures in place to see that they continue. For example, downer animals are not allowed to enter commerce for human consumption and there is a ban on feeding ruminant derived protein to cattle.

http://www.agi.state.al.us/press_releases/second-bse-results-show-positive-cow-on-alabama-farm?pn=2

http://web.archive.org/web/20060604180930/http://www.agi.state.al.us/press_releases/second-bse-results-show-positive-cow-on-alabama-farm?pn=2

TSS

=====

From: TSS 

Subject: USDA/Alabama BSE Epidemiological Update 

Date: March 16, 2006 at 5:51 pm PST

Jim Rogers (202) 690-4755 U.S. Department of Agriculture

Christy Rhodes (334) 240-7103 Alabama Department of Agriculture & Industries

USDA/Alabama BSE Epidemiological Update

WASHINGTON , March 16, 2006 --Today, officials with the state of Alabama and the U.S. Department of Agriculture have completed work at the farm in Alabama to recover the remains of the cow that tested earlier this week as positive for BSE.

Federal and state agriculture workers excavated the remains of the animal, which had been buried on the farm and did not enter the animal or human food chain, in accordance with USDA protocols. While the carcass matches the description provided by the owner, samples are being sent to USDA's National Veterinary Services Laboratory (NVSL) in Ames , Iowa to match DNA with the positive sample. The DNA will also be used to match suspected siblings and offspring found during the epidemiological investigation.

After further examination, experts confirmed through dentition that the animal was at least 10 years of age. This means the animal would have been born prior to the implementation of the Food and Drug Administration's 1997 feed ban. Human and animal health in the United States is protected by a system of interlocking safeguards, which ensure the safety of U.S. beef. The most important of these safeguards is the ban on specified risk materials from the food supply and the FDA's ruminant-to-ruminant feed ban.

In addition to the carcass, federal agriculture officials located a six-week-old calf belonging to the BSE positive animal. The calf has been quarantined and is being moved to NVSL for further observation.

The USDA's Animal and Plant Health Inspection Service will begin tomorrow to post daily updates on the progress of the epidemiological investigation on its website between 4 and 5 p.m. EST. The updates will be available at:

http://www.aphis.usda.gov/newsroom/hot_issues/bse.shtml

http://web.archive.org/web/20061027224321/http://www.aphis.usda.gov/newsroom/hot_issues/bse.shtml

http://www.aphis.usda.gov/newsroom/content/2006/03/bse_al-epi_vs.shtml

http://web.archive.org/web/20060922193758/http://www.aphis.usda.gov/newsroom/content/2006/03/bse_al-epi_vs.shtml

=====

From: TSS 

Subject: SECOND USDA CONFIRMATORY TEST RESULTS POSITIVE FOR BSE 

Date: March 16, 2006 at 7:22 am PST

Release No. 0090.06 Contact: Jim Rogers (202) 690-4755 USDA Press Office (202) 720-4623

SECOND USDA CONFIRMATORY TEST RESULTS POSITIVE FOR BSE

WASHINGTON, March 15, 2006- The U.S. Department of Agriculture's Animal and Plant Health Inspection Service (APHIS) today announced that the second of two bovine spongiform encephalopathy (BSE) confirmatory tests conducted on an Alabama cow has returned a positive result.

Earlier this week, USDA announced that an Alabama cow was positive for BSE after receiving the results of a Western blot confirmatory test. APHIS' National Veterinary Services Laboratories in Ames, Iowa, which conducted a second confirmatory test, the immunohistochemistry (IHC), received positive results today. Under APHIS protocols, if either the IHC or the Western blot returns a positive result the animal is considered positive for BSE.

APHIS is currently conducting an epidemiological investigation into the animal's origin in order to attempt to trace the animal to its place of birth. It had been on the Alabama farm less than a year. One of the first steps in this investigation will be the recovery of the carcass for examination to allow APHIS investigators to directly examine the breed and age of the animal as well as check the animal for any form of identification such as ear-tags. The recovery will be completed within the next day.

The cow, initially reported to be a Santa Gertrudis, is now believed to be a red crossbred (possibly crossed with a Santa Gertrudis or similar breed). This animal was non-ambulatory on the farm and examined by a local, private veterinarian. The veterinarian returned to the farm the following day, euthanized the animal and collected a sample, which was submitted for testing. The animal was buried on the farm at that time.

This animal did not enter the animal or human food chain, in accordance with USDA protocols. Human and animal health in the United States is protected by a system of interlocking safeguards, which ensure the safety of U.S. beef. The most important of these safeguards is the ban on specified risk materials from the food supply and the Food and Drug Administration's ruminant-to-ruminant feed ban.

As part of USDA's BSE enhanced surveillance program, more than 650,000 samples have been tested since June 2004. Throughout this effort, APHIS has noted the likelihood of finding additional cases of BSE. To date, only two of these highest risk animals has tested positive for the disease as part of the surveillance program, for a total of three cases of BSE in the United States. The enhanced surveillance program was designed as a one-time, intensive effort to provide a snap shot of the U.S. cattle population, in order to determine the prevalence of BSE in this country. This second case does not change the fact that BSE prevalence in the United States remains extremely low.

APHIS will continue to work closely with the state of Alabama to learn more about this animal's history, and the results of our epidemiological investigation will be shared with the public. All animals of interest will be tested for BSE.

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB/.cmd/ad/.ar/sa.retrievecontent/.c/6_2_1UH/.ce/7_2_5JM/.p/5_2_4TQ/.d/3/_th/J_2_9D/_s.7_0_A/7_0_1OB?PC_7_2_5JM_contentid=2006%2F03%2F0090.xml&PC;_7_2_5JM_navtype=RT&PC;_7_2_5JM_parentnav=LATEST_RELEASES&PC;_7_2_5JM_navid=NEWS_RELEASE#7_2_5JM

TSS

2025 link is Forbidden…terry

-------- 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

<<Asante et al 2002.pdf>>

____________________________________

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)

____________________________________

''This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. 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.''

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

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

https://www.nature.com/articles/srep11573

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

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

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

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

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

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

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

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

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

===============

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

===============

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

==============

PRION 2015 CONFERENCE

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/

***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.

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

PRION 2016 TOKYO

Saturday, April 23, 2016

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

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

Taylor & Francis

Prion 2016 Animal Prion Disease Workshop Abstracts

WS-01: Prion diseases in animals and zoonotic potential

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

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

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

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.

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160

Sunday, January 10, 2021 

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

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

Greetings APHIS et al,

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

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

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

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

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

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

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

https://www.regulations.gov/comment/APHIS-2018-0087-0002

https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf

http://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.230.8886&rep=rep1&type=pdf

https://bovineprp.blogspot.com/2020/12/bse-research-project-final-report-2005.html

http://bovineprp.blogspot.com/2020/12/

Control of Chronic Wasting Disease OMB Control Number: 0579-0189APHIS-2021-0004 Singeltary Submission

https://www.regulations.gov/comment/APHIS-2021-0004-0002

https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf

Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification

https://www.regulations.gov/document/APHIS-2018-0011-0003

https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf

Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE

Friday, May 19, 2023 | 04:12pm NASHVILLE — The Tennessee State Veterinarian is confirming a case of atypical bovine spongiform encephalopathy (BSE) in a cow with ties to Tennessee.

The cow appeared unwell after arriving at a packing company in South Carolina. In alignment with the United States Department of Agriculture’s BSE surveillance program, the animal was isolated and euthanized. It did not enter the food supply. Preliminary investigation has determined the cow originated in southeast Tennessee.

“We are working closely with our federal partners and animal health officials in South Carolina for this response,” State Veterinarian Dr. Samantha Beaty said. “That includes determining prior owners and locations where the affected cow lived in Tennessee and tracing siblings and offspring for testing.”

BSE is a chronic degenerative disease affecting the central nervous system of cattle. It is caused by an abnormal prion protein. The atypical form occurs spontaneously at very low levels in all cattle populations, particularly in older animals. Atypical BSE poses no known risk to human health. It is different from the classical form of BSE, which has not been detected in the U.S. since 2003.

BSE is not contagious and therefore is not spread through contact between cattle or with other species. There is no treatment for or vaccine to prevent BSE. The U.S. has a strong surveillance program in place for early detection and to prevent suspect cattle from entering the food supply chain.

Cattle owners are always advised to monitor their herds for health. Cattle affected by BSE may display changes in temperament, abnormal posture, poor coordination, decreased milk production, or loss of condition without noticeable loss of appetite. Owners should report any herd health concerns to their veterinarian or to the State Veterinarian’s office at 615-837-5120.

The Tennessee Department of Agriculture Animal Health Division is responsible for promoting animal health in Tennessee. The State Veterinarian’s office seeks to prevent the spread of disease through import and movement requirements, livestock traceability, disaster mitigation, and the services of the C.E. Kord Animal Health Diagnostic Laboratory. The division collaborates with other health-related stakeholders, academic institutions, and extension services to support One Health, an initiative to improve health for people and animals.

https://www.tn.gov/agriculture/news/2023/5/19/state-veterinarian-alerts-cattle-owners-to-disease-detection.html

USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection

The U.S. Department of Agriculture (USDA) is announcing an atypical case of Bovine Spongiform Encephalopathy (BSE), a neurologic disease of cattle, in an approximately five-year-old or older beef cow at a slaughter plant in South Carolina. This animal never entered slaughter channels and at no time presented a risk to the food supply or to human health in the United States. Given the United States’ negligible risk status for BSE, we do not expect any trade impacts as a result of this finding.

USDA Animal and Plant Health Inspection Service’s (APHIS) National Veterinary Services Laboratories (NVSL) confirmed that this cow was positive for atypical L-type BSE. The animal was tested as part of APHIS’s routine surveillance of cattle that are deemed unsuitable for slaughter. The radio frequency identification tag present on the animal is associated with a herd in Tennessee. APHIS and veterinary officials in South Carolina and Tennessee are gathering more information during this ongoing investigation.

Atypical BSE generally occurs in older cattle and seems to arise rarely and spontaneously in all cattle populations.

This is the nation’s 7th detection of BSE. Of the six previous U.S. cases, the first, in 2003, was a case of classical BSE in a cow imported from Canada; the rest have been atypical (H- or L-type) BSE.

The World Organization for Animal Health (WOAH) recognizes the United States as negligible risk for BSE. As noted in the WOAH guidelines for determining this status, atypical BSE cases do not impact official BSE risk status recognition as this form of the disease is believed to occur spontaneously in all cattle populations at a very low rate. Therefore, this finding of an atypical case will not change the negligible risk status of the United States, and should not lead to any trade issues.

The United States has a longstanding system of interlocking safeguards against BSE that protects public and animal health in the United States, the most important of which is the removal of specified risk materials - or the parts of an animal that would contain BSE should an animal have the disease - from all animals presented for slaughter. The second safeguard is a strong feed ban that protects cattle from the disease. Another important component of our system - which led to this detection - is our ongoing BSE surveillance program that allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population.

More information about this disease is available in the BSE factsheet.

#

https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse

May 2, 2023

Docket No. APHIS–2023–0027 Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission

ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023.

ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo.

Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission

https://www.regulations.gov/comment/APHIS-2023-0027-0002

see full submission;

https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf

Mission, Texas, scrapie transmission to cattle atypical BSE.

IBNC Tauopathy or TSE Prion disease, it appears, no one is sure

Terry S. Singeltary Sr., 03 Jul 2015 at 16:53 GMT

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 ***

https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/5adef4ac-a7e4-46a4-8806-c8533d5c862c

WEDNESDAY, DECEMBER 23, 2020

Idiopathic Brainstem Neuronal Chromatolysis IBNC BSE TSE Prion a Review 2020

https://bse-atypical.blogspot.com/2020/12/idiopathic-brainstem-neuronal.html

***Our transmission study demonstrates that CH 1641-like scrapie is likely to be more virulent than classical scrapie in cattle.

In the US, scrapie is reported primarily in sheep homozygous for 136A/171Q (AAQQ) and the disease phenotype is similar to that seen with experimental strain CH1641.

http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=182469

***Our transmission study demonstrates that CH 1641-like scrapie is likely to be more virulent than classical scrapie in cattle.

P-088 Transmission of experimental CH1641-like scrapie to bovine PrP overexpression mice

Kohtaro Miyazawa1, Kentaro Masujin1, Hiroyuki Okada1, Yuichi Matsuura1, Takashi Yokoyama2

1Influenza and Prion Disease Research Center, National Institute of Animal Health, NARO, Japan; 2Department of Planning and General Administration, National Institute of Animal Health, NARO

Introduction: Scrapie is a prion disease in sheep and goats. CH1641-lke scrapie is characterized by a lower molecular mass of the unglycosylated form of abnormal prion protein (PrpSc) compared to that of classical scrapie. It is worthy of attention because of the biochemical similarities of the Prpsc from CH1641-like and BSE affected sheep. We have reported that experimental CH1641-like scrapie is transmissible to bovine PrP overexpression (TgBoPrP) mice (Yokoyama et al. 2010). We report here the further details of this transmission study and compare the biological and biochemical properties to those of classical scrapie affected TgBoPrP mice.

Methods: The details of sheep brain homogenates used in this study are described in our previous report (Yokoyama et al. 2010). TgBoPrP mice were intracerebrally inoculated with a 10% brain homogenate of each scrapie strain. The brains of mice were subjected to histopathological and biochemical analyses.

Results: Prpsc banding pattern of CH1641-like scrapie affected TgBoPrP mice was similar to that of classical scrapie affected mice. Mean survival period of CH1641-like scrapie affected TgBoPrP mice was 170 days at the 3rd passage and it was significantly shorter than that of classical scrapie affected mice (439 days). Lesion profiles and Prpsc distributions in the brains also differed between CH1641-like and classical scrapie affected mice.

Conclusion: We succeeded in stable transmission of CH1641-like scrapie to TgBoPrP mice. Our transmission study demonstrates that CH 1641-like scrapie is likely to be more virulent than classical scrapie in cattle.

snip...

In the US, scrapie is reported primarily in sheep homozygous for 136A/171Q (AAQQ) and the disease phenotype is similar to that seen with experimental strain CH1641.

http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=182469

CH1641

http://scrapie-usa.blogspot.com/2019/11/review-update-on-classical-and-atypical.html

WEDNESDAY, JULY 31, 2019

The agent of transmissible mink encephalopathy passaged in sheep is similar to BSE-L

49. The agent of transmissible mink encephalopathy passaged in sheep is similar to BSE-L

E. D. Cassmanna,b, S. J. Moorea,b, R. D. Kokemullera, A. Balkema-Buschmannc, M. H. Groschupcand J. J. Greenleea

aVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA (EDC, SJM, RDK, JJG); bOak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. (EDC, SJM), Department of Veterinary Pathology, Iowa State University, Ames, IA, USA (JDS); cInstitute of Novel and Emerging Infectious Diseases, Friedrich-Loeffler-Institut, Federal Research Institute for Animal Health, Greifswald – Isle of Riems, Germany (ABB, MHG)

CONTACT E. D. Cassmann eric.cassmann@usda.gov

ABSTRACT

Introduction: Transmissible mink encephalopathy (TME) is a fatal neurologic prion disease of farmed mink. Epidemiologic and experimental evidence following a Wisconsin outbreak in 1985 has linked TME to low-type bovine spongiform encephalopathy (BSE-L). Evidence suggests that farmed mink were likely exposed through feeding of BSE-L infected downer cattle. The interspecies transmission of TME to cattle has been documented. Recently, we demonstrated the susceptibility of sheep to cattle passaged TME by intracranial inoculation. The aim of the present study was to compare ovine passaged cattle TME to other prion diseases of food-producing animals. Using a bovine transgenic mouse model, we compared the disease phenotype of sheep TME to BSE-C and BSE-L.

Materials and Methods: Separate inoculants of sheep passaged TME were derived from animals with the VRQ/VRQ (VV136) and ARQ/VRQ (AV136) prion protein genotype. Transgenic bovinized mice (TgBovXV) were intracranially inoculated with 20 µl of 1% w/v brain homogenate. The disease phenotypes were characterized by comparing the attack rates, incubation periods, and vacuolation profiles in TgBovXV mice.

Results: The attack rate for BSE-C (13/13), BSE-L (18/18), and TMEVV (21/21) was 100%; whereas, the TMEAV group (15/19) had an incomplete attack rate. The average incubation periods were 299, 280, 310, and 541 days, respectively. The vacuolation profiles of BSE-L and TMEVV were most similar with mild differences observed in the thalamus and medulla. Vacuolation profiles from the BSE-C and TMEAV experimental groups were different than TMEVVand BSE-L.

Conclusion: Overall the phenotype of disease in TME inoculated transgenic mice was dependent on the sheep donor genotype (VV vs AV). The results of the present study indicate that TME isolated from VRQ/VRQ sheep is similar to BSE-L with regards to incubation period, attack rate, and vacuolation profile. Our findings are in agreement with previous research that found phenotypic similarities between BSE-L and cattle passaged TME in an ovine transgenic rodent model. In this study, the similarities between ovine TME and BSE-L are maintained after multiple interspecies passages.

Prion2019 Conference

https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197

2007

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2876762/

August 1988

Evidence That Transmissible Mink Encephalopathy Results From Feeding Infected Cattle

https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. snip... The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf

https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf

NOW, in 1979, it was proven that indeed U.S. scrapie strain that was transmitted to U.S. cattle, did NOT produce a Transmissible Spongiform Encephalopathy (TSE) like the U.K. B.S.E., but a TSE unlike the U.K. B.S.E. SO what does all this tell us? it tells me that there is a possibility that a strain of mad cow disease was circulating in the U.S.A. long, long, before originally thought, only left to be ignored, while incubating and spreading.

3.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE.339 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to DrWatson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture.340 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre.341 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

3.58 There are several possible reasons why the experiment was not performed in the UK. It had been recommended by Sir Richard Southwood (Chairman of the Working Party on Bovine Spongiform Encephalopathy) in his letter to the Permanent Secretary of MAFF, Mr (now Sir) Derek Andrews, on 21 June 1988,342 though it was not specifically recommended in the Working Party Report or indeed in the Tyrrell Committee Report (details of the Southwood Working Party and the Tyrell Committee can be found in vol. 4: The Southwood Working Party, 1988–89 and vol. 11: Scientists after Southwood respectively). The direct inoculation of scrapie into calves was given low priority, because of its high cost and because it was known that it had already taken place in the USA.343 It was also felt that the results of such an experiment would be hard to interpret. While a negative result would be informative, a positive result would need to demonstrate that when scrapie was transmitted to cattle, the disease which developed in cattle was the same as BSE.344 Given the large number of strains of scrapie and the possibility that BSE was one of them, it would be necessary to transmit every scrapie strain to cattle separately, to test the hypothesis properly. Such an experiment would be expensive. Secondly, as measures to control the epidemic took hold, the need for the experiment from the policy viewpoint was not considered so urgent. It was felt that the results would be mainly of academic interest.345

3.59 Nevertheless, from the first demonstration of transmissibility of BSE in 1988, the possibility of differences in the transmission properties of BSE and scrapie was clear. Scrapie was transmissible to hamsters, but by 1988 attempts to transmit BSE to hamsters had failed. Subsequent findings increased that possibility.

337 Fraser, H., Bruce, M., Chree, A., McConnell, I. and Wells, G. (1992) Transmission of Bovine Spongiform Encephalopathy and Scrapie to Mice, Journal of General Virology, 73, 1891–7; Bruce, M., Chree, A., McConnell, I., Foster, J., Pearson, G. and Fraser, H. (1994) Transmission of Bovine Spongiform Encephalopathy and Scrapie to Mice: Strain Variation and the Species Barrier, Philosophical Transactions of the Royal Society of London, Series B, Biological Sciences, 343, 405–11 338 Bruce, M., Will, R., Ironside, J., McConell, I., Drummond, D., Suttie, A., McCordie, L., Chree, A., Hope, J., Birkett, C., Cousens, S., Fraser, H. and Bostock, C. (1997) Transmissions to Mice Indicate that ‘New Variant’ CJD is Caused by the BSE Agent, Nature, 389, 498–501 339 Clark, W., Hourrigan, J. and Hadlow, W. (1995) Encephalopathy in Cattle Experimentally Infected with the Scrapie Agent, American Journal of Veterinary Research, 56, 606–12 340 YB88/10.00/1.1 341 Cutlip, R., Miller, J., Race, R., Jenny, A., Katz, J., Lehmkuhl, H., Debey, B. and Robinson, M. (1994) Intracerebral Transmission of Scrapie to Cattle, Journal of Infectious Diseases, 169, 814–20 342 YB88/6.21/1.2 343 YB88/11.17/2.4

https://web.archive.org/web/20090505200149/http://www.bseinquiry.gov.uk/pdf/volume2/Chapter3.pdf

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html

31

Appendix I VISIT TO USA - OR A E WRATHALL — INFO ON BSE AND SCRAPIE

Dr Clark lately of the scrapie Research Unit, Mission Texas has

successfully transmitted ovine and caprine scrapie to cattle. The

experimental results have not been published but there are plans to do

this. This work was initiated in 1978. A summary of it is:-

Expt A 6 Her x Jer calves born in 1978 were inoculated as follows with

a 2nd Suffolk scrapie passage:-

i/c 1ml; i/m, 5ml; s/c 5ml; oral 30ml.

1/6 went down after 48 months with a scrapie/BSE-like disease.

Expt B 6 Her or Jer or HxJ calves were inoculated with angora Goat

virus 2/6 went down similarly after 36 months.

Expt C Mice inoculated from brains of calves/cattle in expts A & B were resistant, only 1/20 going down with scrapie and this was the reason given for not publishing.

Diagnosis in A, B, C was by histopath. No reports on SAF were given.

Dr Warren Foote indicated success so far in eliminating scrapie in offspring from experimentally— (and naturally) infected sheep by ET. He had found difficulty in obtaining embryos from naturally infected sheep (cf SPA).

Prof. A Robertson gave a brief accout of BSE. The us approach was to

32

accord it a very low profile indeed. Dr A Thiermann showed the picture in the "Independent" with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs.

BSE was not reported in USA.

4. Scrapie incidents (ie affected flocks) have shown a dramatic increase since 1978. In 1953 when the National Control scheme was started there were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.

5. Scrapie agent was reported to have been isolated from a solitary fetus.

6. A western blotting diagnostic technique (? on PrP) shows some promise.

7. Results of a questionnaire sent to 33 states on the subject of the national sheep scrapie programme survey indicated

17/33 wished to drop it

6/33 wished to develop it

8/33 had few sheep and were neutral

Information obtained from Dr Wrathall‘s notes of a meeting of the u.s.

Animal Health Association at Little Rock, Arkansas Nov. 1988.

33

In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...

http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

VISIT TO USA - DR AE WRATHALL - INFO ON BSE AND SCRAPIE

1. Dr. Clark lately of the Scrapie Research Unit, Mission Texas has successfully transmitted ovine & caprine Scrapie to cattle. The experimental results have not been published but there are plans to do this. This work was initiated in 1978. A summary of it is;

snip...see handwritten notes from this here;

http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

https://web.archive.org/web/20090505234344/http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

IN CONFIDENCE

Perceptions of an unconventional slow virus diseases of animals in the U.S.A. G A H Wells

Report of a Visit to the USA April-May 1989

http://webarchive.nationalarchives.gov.uk/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

http://bseusa.blogspot.com/2018/

Thursday, June 09, 2016

Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie Experiment 1964

How Did CWD Get Way Down In Medina County, Texas?

Confucius ponders...

Could the Scrapie experiments back around 1964 at Moore Air Force near Mission, Texas, could this area have been ground zero for CWD TSE Prion (besides the CWD cases that have waltzed across the Texas, New Mexico border near WSMR Trans Pecos region since around 2001)?

Epidemiology of Scrapie in the United States 1977

snip...

Scrapie Field Trial Experiments Mission, Texas A Scrapie Field Trial was developed at Mission, Texas, to provide additional information for the eradication program on the epidemiology of natural scrapie. The Mission Field Trial Station is located on 450 acres of pastureland, part of the former Moore Air Force Base, near Mission, Texas.

It was designed to bring previously exposed, and later also unexposed, sheep or goats to the Station and maintain and breed them under close observation for extended periods to determine which animals would develop scrapie and define more closely the natural spread and other epidemiological aspects of the disease.

The 547 previously exposed sheep brought to the Mission Station beginning in 1964 were of the Cheviot, Hampshire, Montadale, or Suffolk breeds.

They were purchased as field outbreaks occurred, and represented 21 bloodlines in which scrapie had been diagnosed.

Upon arrival at the Station, the sheep were maintained on pasture, with supplemental feeding as necessary.

The station was divided into 2 areas:

(1) a series of pastures and-pens occupied by male animals only, and

(2) a series of pastures and pens occupied by females and young progeny of both sexes.

... snip...

see full text ;

http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf

Thursday, June 09, 2016

Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie TSE Prion Experiment 1964 How Did CWD Get Way Down In Medina County, Texas?

http://chronic-wasting-disease.blogspot.com/2016/06/how-did-cwd-get-way-down-in-medina.html

http://scrapie-usa.blogspot.com/2016/06/scrapie-field-trial-experiments-mission.html

doi:10.1016/S0021-9975(97)80022-9 Copyright © 1997 Published by Elsevier Ltd.

Second passage of a US scrapie agent in cattle

R.C. Cutlip, J.M. Miller and H.D. Lehmkuhl

United States Department of Agriculture, Agricultural Research Service, National Animal Disease Center, Ames, Iowa, USA

Received 10 September 1996; accepted 31 July 1997. Available online 25 May 2006.

Summary

Scrapie and bovine spongiform encephalopathy are similar chronic neurodegenerative diseases of sheep and cattle. An earlier study showed that, on first passage in cattle, a US scrapie agent caused an encephalopathy that was distinct from bovine spongiform encephalopathy (BSE). The present report describes a second passage in cattle, carried out because diseases caused by the spongiform encephalopathy agents often change in character with additional passages in abnormal hosts. For this work, young calves were inoculated intracerebrally with a pooled suspension of brain from cattle that had died of encephalopathy after experimental inoculation with brain from scrapie-affected sheep. The second passage disease was essentially identical with the first passage disease, as judged by clinical signs, histopathological findings and distribution of "prion protein scrapie" (PrPsc). This represents additional evidence to suggest that the US sheep scrapie agent tested is incapable of causing BSE in cattle.

http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WHW-4K1V3NT-9&_user=10&_rdoc=1&_fmt=&_orig=search&_sort=d&view=c&_version=1&_urlVersion=0&_userid=10&md5=01b6b1aef2a83ea797b17fc4305a4752

(b) the epidemiological and laboratory studies in the USA suggest the possibility of an occurrence of BSE infection in cattle as the origin of outbreaks of TME.

{c) there is also evidence from two experiments conducted in the USA that cattle, though susceptible to scrapie inocula prepared from sheep, express a pathology quite different from that of BSE and not convincingly diagnostic of an SE by histopathological criteria. Furthermore, neither of these studies can be regarded as a basis for extrapolation to the situation in the UK because the inocula used were either experimentally passaged or natural scrapie originating from Suffolk sheep; a minority breed in this country.

https://webarchive.nationalarchives.gov.uk/20080102191344/http://www.bseinquiry.gov.uk/files/yb/1993/09/21002001.pdf

Is There a Scrapie-Like Disease in Cattle? R.F. Marsh*, DVM, PhD and G.R. Hartsough, DVM

Transmissible mink encephalopathy (TME) is a rare disease of ranch-reared mink which is indistinguishable from sheep scrapie. Previous studies on the epidemiology of TME have not identified a definite source of infection for mink. Studies on experimental transmission have shown that mink are susceptible to intracerebral inoculation of American Suffolk scrapie, but that the incubation periods are longer (>1 year) than those observed in natural outbreaks of TME (<1 year).

In April of 1985, a mink rancher in Wisconsin reported a debilitating neurologic disease in his herd which we diagnosed as TME by histopathologic findings confirmed by experimental transmission to mink and squirrel monkeys. The rancher was a “dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed.

We believe that these findings may indicate the presence of a previously unrecognized scrapie-like disease in cattle and wish to alert dairy practitioners to this possibility.

* Department of Veterinary Science, University of Wisconsin-

- Madison, Madison, WI 53706, .

* Director of the GLMA/EMBA Ranch Service, P.0. Box 342, Thiensville, WI 53092.

PROCEEDINGS OF THE SEVENTH ANNUAL WESTERN CONFERENCE FOR FOOD ANIMAL VETERINARY MEDICINE, University of Arizona, March 17-19, 1986

August 1988

Evidence That Transmissible Mink Encephalopathy Results From Feeding Infected Cattle

https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

Mission, Texas, scrapie transmission to cattle atypical BSE.

http://web.archive.org/web/20060925205531/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

THURSDAY, JUNE 5, 2025

World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible, what could go wrong?

https://bovineprp.blogspot.com/2025/06/world-organisation-for-animal-health.html

MONDAY, JUNE 09, 2025

The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE), Singeltary Review

https://bse-atypical.blogspot.com/2025/06/the-naturally-occurring-lysine-to.html

WEDNESDAY, JULY 16, 2025

Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field

https://bse-atypical.blogspot.com/2025/07/classical-bse-emergence-from.html

2001 Singeltary on CJD

JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214

February 14, 2001

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Terry S. Singeltary, Sr

Author Affiliations

JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

https://jamanetwork.com/journals/jama/article-abstract/1031186

Prion Scientific Advisors and Consultants Staff Meeting Singeltary Submission Freas Monday, January 08,2001 3:03 PM

Scientific Advisors and Consultants Staff 2001 Advisory Committee TSE PRION Singeltary Submission Freas Monday, January 08,2001 3:03 PM

FDA Singeltary submission 2001

Greetings again Dr. Freas and Committee Members,

I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee (short version). I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products. I cannot explain the 'real' risk of this in 5 or 10 minutes at some meeting, or on 2 or 3 pages, but will attempt here: fda link is dead in the water;

http://web.archive.org/web/20041212232925/http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

https://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html

26 MARCH 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

Terry S. Singeltary, retired (medically)

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6

Tracking spongiform encephalopathies in North America 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

Volume 3, Number 8 01 August 2003

Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.

49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.

Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.

Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.

To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.

Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.

Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.

Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.

Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.

CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009 Plos Singeltary August 10, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

August 10, 2009

Greetings,

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date.

But, while sub-clinical, how many can one exposed human infect?

Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas?

why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved?

would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite?

The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.

The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

please see history, and the ever evolving TSE science to date ;

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd

Singeltary Submission SEAC 2007

SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission

This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry

SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”

41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.

http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf

The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases, by Philip Yam, Answering critics like Terry Singeltary 2007

The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases

by Philip Yam

''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...

Revisiting Sporadic CJD

It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow.org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that

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prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.

Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on vegsource and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.

Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.

224 CHAPTER 14

Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.

Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.

Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.

Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows

Laying Odds 225

(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).

Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4

The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to

226 CHAPTER 14

infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.

A Case for Undercounting

The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7

The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year

Laying Odds 227

(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)

In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8

One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a one in-a-million lottery, it’s more like one-in-2.5-million for African Americans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?

Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9

SNIP...SEE FULL TEXT;

http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf

Singeltary 2000

BMJ 2000; 320 doi: https://doi.org/10.1136/bmj.320.7226.8/b (Published 01 January 2000) Cite this as: BMJ 2000;320:8

02 January 2000 Terry S Singeltary retired

Rapid Response:

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well...

In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.

The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;

"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."

Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.

Something else I find odd, page 16;

"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."

A few more factors to consider, page 15;

"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."

"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."

"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."

Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.

Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.

To be continued...

Terry S. Singeltary Sr. Bacliff, Texas USA

Competing interests: No competing interests

https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well

BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999) Cite this as: BMJ 1999;319:1312

Rapid Response:

Re: vCJD in the USA * BSE in U.S.

In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease.

Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through. Sporadic, simply means, they do not know.

My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following;

vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD.

The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?

CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997.

So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.

No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;

Since 1990 the U.S. has raised 1,250,880,700 cattle;

Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;

There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;

Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;

Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.

I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endescopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.

Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.

It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........

The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.

Terry S. Singeltary Sr.

Bacliff, Texas 77518 USA

flounder@wt.net

Competing interests: No competing interests

https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us

1999 Re: vCJD in the USA * BSE in U.S. BMJ 1999;319:1312 Singeltary

https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us

FRIDAY, JANUARY 15, 2021

CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire?

if not, why not?

https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html

CJD TSE Prion Questionnaire USA, UK, Singeltary

CJD FOUNDATION Questionnaire

https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf

See archived url link;

http://web.archive.org/web/20230408043406/https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf

UK CJD Questionnaire

http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf

cjd questionnaire 1979

https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf

RE: re-Human Prion Diseases in the United States part 2 flounder replied to flounder on 02 Jan 2010 at 21:26 GMT

I would kindly like to add to my initial concerns, something I brought up years ago, and I believe that still hold true today, more so even than when I first stated these concerns in 2003 ;

***> routine passive mortality CJD surveillance USA ?

***> THIS has been proven not to be very useful in the U.K.;

THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)

snip...

One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys by 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...

snip...

http://web.archive.org/web/20040521215716/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf

Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will

snip...

IDENTIFICATION OF CASES

Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...

full text;

http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf

CJD Questionnaire

F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)

https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf

THE MAKING OF THE USA CJD QUESTIONNAIRE

https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html

https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html

https://cjdquestionnaire.blogspot.com/2009/

http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html

https://cjdquestionnaire.blogspot.com/2007/

http://cjdquestionnaire.blogspot.com/

MONDAY, SEPTEMBER 11, 2023

Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide

“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”

https://www.ucl.ac.uk/brain-sciences/research/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases

Singeltary sCJD

https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html

TUESDAY, APRIL 15, 2025

Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology

https://creutzfeldt-jakob-disease.blogspot.com/2025/04/cases-of-creutzfeldt-jakob-disease-in.html

SUNDAY, MARCH 23, 2025

Creutzfeldt Jakob Disease TSE Prion Increasing 2025 Update

https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html

Creutzfeldt Jakob Disease

https://creutzfeldt-jakob-disease.blogspot.com/

Iatrogenic Transmissible Spongiform Encephalopathy

https://itseprion.blogspot.com/

Terry S. Singeltary Sr.