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State of the art in biosafety at the European National Reference Laboratories for Transmissible Spongiform Encephalopathies

 State of the art in biosafety at the European National Reference Laboratories for Transmissible Spongiform Encephalopathies


ORIGINAL RESEARCH article Front. Public Health, 09 February 2026 Sec. Occupational Health and Safety Volume 13 - 2025 | https://doi.org/10.3389/fpubh.2025.1733350

State of the art in biosafety at the European National Reference Laboratories for Transmissible Spongiform Encephalopathies

M. Begovoeva .jpeg M. Begovoeva 1 R. Nonno .jpeg R. Nonno 2 M. Mazza .jpeg M. Mazza 1* E. Bozzetta .jpeg E. Bozzetta 1 G. Ru .jpeg G. Ru 1 C Colleagues of the European Network of National Reference Laboratories for Transmissible Spongiform Encephalopathies 1. Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Torino, Italy 2. Istituto Superiore di Sanità, Roma, Italy Article metrics View details 675 Views 107 Downloads 

Abstract

Introduction: Laboratory technicians handling material containing or potentially infected with prions are exposed to the occupational risk of contracting infectious forms of Creutzfeldt–Jakob disease. The occurrence of three fatalities among laboratory personnel with a history of occupational exposure to bovine spongiform encephalopathy has highlighted the need for greater attention to the correct implementation of biosafety procedures in laboratories that handle prions.

Methods: The European Reference Laboratory for Transmissible Spongiform Encephalopathies (EURL-TSE) carried out a survey to review biosafety procedures currently adopted by the 31 National Reference Laboratories for TSEs (NRLs), stimulate discussion among laboratory directors, and define future actions aimed at reducing the risk of occupational exposure among laboratory personnel.

Results: The survey showed that 83.9% of laboratories adhered to TSE-specific biosafety guidelines, and 61.3% had conducted a biosafety risk assessment, while the biosafety level of the facilities had been determined in 93.5% of cases. In addition, 83.9% reported implementing staff biosafety training protocols, and 77.4% had procedures addressing the risk associated with sharp or pointed tools. Most laboratories had established biosafety procedures for ELISA and western blot assays, whereas fewer had protocols for pathology and immunohistochemistry. Among the three facilities performing experimental inoculations, only two indicated having specific biosafety measures in place.

Discussion: Despite the overall positive results, the survey showed that biosafety procedures are applied heterogeneously across the NRLs. The EURL-TSE will further encourage the full implementation of specific biosafety procedures to address the gaps identified by the survey. The EURL-TSE has created a digital repository of guidelines and bibliographical sources on the subject. It also initiated biannual virtual meetings to discuss critical issues emerged from the survey and to encourage the mutual exchange of experiences and good practices between NRLs Directors.

1 Introduction 

Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative disorders affecting humans and several other mammal species. These conditions are caused by the accumulation, mainly in the central nervous system, of misfolded prion proteins. This accumulation generates lesions in the encephalic tissue and thus confers a spongiform appearance (1). Creutzfeldt–Jakob disease (CJD) is the most common human TSE. Based on its aetiology, CJD is classified in sporadic (sCJD), genetic (gCGD) and infectious forms, the latter resulting from transmission between humans or from animals and including iatrogenic CJD (iCJD) and variant CJD (vCJD) (2). Major non-human TSEs are Bovine Spongiform Encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in cervids, and scrapie in sheep and goats. Both human and animal prion diseases are characterized by a long incubation period followed by the onset of rapidly progressive neurological symptoms and an invariably fatal outcome, as there are no treatments available (2). Given the absence of immune system involvement, to date it is not possible to make a reliable intravitam diagnosis of these pathologies. Moreover, due to their lack of nucleic acids and proteinaceous nature, the prions show high resistance to decontamination procedures commonly used against other microorganisms, posing a serious threat to laboratory personnel and requiring the careful implementation of appropriate risk management strategies (3). Health professionals are exposed to the risk of contracting infectious forms of CJD. Humans can in fact contract iCJD when exposed to CJD-infected material, or vCJD if exposed to BSE prions (2). BSE transmission from animals to humans through the food chain has unfortunately long since been confirmed and associated to vCJD cases, while this possibility has not yet been ruled out for CWD (4). Although some experimental studies have suggested that specific strains of scrapie may cause forms of human CJD with clinical and pathological features indistinguishable from sCJD (5, 6), no epidemiological evidence has yet supported this hypothesis. Even though no relative excess of sCJD was found among healthcare professionals, limitations in registries and case-control studies preclude the definitive exclusion of this possibility (7). Furthermore, this finding does not rule out the potential for specific professions to be associated with an occupational risk (7), as in the case of laboratory technicians handling material containing prions or potentially infected with them.

Biosafety is the implementation of principles, technologies and practices designed to prevent unintended exposure to biological agents or their accidental release (3, 8). Despite the existence of laboratory biosafety guidelines, the severe threat posed by prions prompted the development of several studies and the drafting of specific TSE biosafety guidelines by national and international institutions. Nevertheless, three cases of vCJD have been documented in laboratory personnel with occupational exposure to TSE agents. In Italy, a patient affected by variant CJD who died in 2016 had worked with brain tissue infected by BSE, even though no laboratory accidents were reported in this regard (9). Two cases were described in France in 2020 and 2021, both involving laboratory workers who had experienced accidental occupational exposure 7.5 and 15 years before the onset of clinical symptoms, respectively (9, 10).

The notification of these occupational fatalities has been of great concern to the operators working in the field of prion diseases. In response to these concerns, the staff of the European Reference Laboratory for TSEs (EURL-TSE) highlighted the need for a comprehensive review of the biosafety procedures of the European National Reference Laboratories for TSEs (NRLs). To this end, in September 2023, the EURL-TSE carried out a survey to collect detailed information about the procedures adopted by each of the NRLs. The aim of the initiative was to promote the harmonization and potential implementation of biosafety measures through benchmarking activities and discussions at scheduled meetings. This paper presents the findings of the survey and describes follow-up actions undertaken by the EURL-TSE.

snip…

3 Results The survey achieved a 100.0% response rate, with feedback received from all 31 NRL Directors contacted (Figure 1). The countries hosting the 31 surveyed NRLs were Austria, Belgium, Bulgaria, Croatia, Cyprus, Czechia, Denmark, Estonia, Finland, France, Germany, Greece, Hungary, Iceland, Ireland, Italy, Latvia, Lithuania, Malta, The Netherlands, North Macedonia, Norway, Poland, Portugal, Romania, Slovakia, Slovenia, Spain, Sweden, Switzerland.

snip…

Most NRLs (n = 26, 83.9%) adhered to specific guidelines for safely working with TSEs, with three quarters of these relying on national guidelines (n = 19, 61.3%) and the remaining quarter referring to the international sources listed in Table 1 (n = 7, 22.6%) (Figure 2A).

A biosafety risk assessment had been carried out in almost two thirds of laboratories (n = 19, 61.3%), while the BSL had been determined for almost all (n = 29, 93.5%) (Figure 3). BSL-3 was the highest biosafety level applied in 18 NRLs (58.1%), while BSL-2 was the highest in 11 NRLs (35.5%, with 5 of these implementing supplementary measures beyond those mandated for BSL-2). When reporting laboratory BSL, biocontainment facilities, whether dedicated or within mixed-level buildings, were considered equivalent. Only two laboratories (6.5%) had not determined their BSL.

snip…

Most NRLs (n = 26, 83.9%) were exclusively dedicated to TSE agents. Nearly all laboratories (n = 30, 96.8%) performed diagnostic activities, with minimal differences between BSL. Approximately one third (n = 11, 35.5%) were involved in research activities, predominantly BSL-3 laboratories, followed by BSL-2 facilities. Only 3 (9.7%) reported conducting experimental inoculations, all of them classified as BSL-3. Three quarters of the laboratories (n = 24, 77.4%) conducted the different activities (diagnostics, research, and experimental inoculations) concerning TSEs in separate areas according to their level of risk or did not engage in multiple activities.

Staff biosafety training protocols were widely implemented (n = 26, 83.9%), and access to TSE laboratories was restricted to authorised personnel only (n = 31, 100.0%). In case of personnel exposure, most laboratories applied specific procedures to mitigate the risk of transmission (n = 25, 80.6%) and kept record of the events in a register of personnel accidents (n = 26, 83.9%). Over three quarters of NRLs applied specific procedures to prevent the use of sharp or pointed tools or the risks associated with them (n = 24, 77.4%). The vast majority of NRLs employed biosafety cabinets (n = 28, 90.3%). Compliance with training and staff management procedures was consistently higher or equal in BSL-3 laboratories compared with BSL-2 laboratories.

Specific biosafety procedures for ELISA and western blot methods were available in most facilities conducting diagnostic (n = 24; 80.0%) and research (n = 9; 81.8%) activities (Figure 2B). However, biosafety procedures specific for pathology and immunohistochemistry were less commonly available in both diagnostics (n = 17, 56.7%) and research (n = 7, 63.6%) laboratories. Of the three laboratories performing experimental inoculations, two reported employing specific biosafety procedures for this practice (66.7%).

Across all BSL categories, most laboratories implemented measures for the laboratory environment, including surfaces, during cleaning procedures (n = 30; 96.8%), as well as for the laboratory equipment in case of maintenance (n = 27, 87.1%), disposal (n = 29, 93.5%), or accidental contamination (n = 28, 90.3%). The totality of NRLs had procedures for safe waste management, and most of them employed procedures for safe transportation of TSE agents (n = 27, 87.1%).

Major differences in compliance across BSL categories were observed in domains related to risk assessment and the implementation of biosafety frameworks, staff training and management, and laboratory biosafety procedures (Figure 3). In these areas, compliance was heterogeneous, ranging from 45.5 to 100.0%. However, in most cases, BSL-3 laboratories showed slightly higher compliance than BSL-2 laboratories. In contrast, adherence to decontamination and waste management procedures was high across all BSL categories, with only one item at 50.0% and the remainder between 83.3 and 100.0%.

Full survey results are provided in the Supplementary material.

https://www.frontiersin.org/journals/public-health/articles/10.3389/fpubh.2025.1733350/full#sec11

4 Discussion Despite the overall positive results, the survey revealed that biosafety procedures are applied heterogeneously across the NRLs. Each laboratory demonstrated stronger capacity to apply certain measures and varying degrees of adherence to others. Higher BSL did not consistently correspond to greater compliance with biosafety procedures. However, five BSL-2 laboratories implemented measures exceeding their level requirements, and the survey did not distinguish between biocontainment facilities in dedicated versus mixed-level buildings. These factors may have partially masked the influence of BSL on compliance with biosafety procedures. Previous studies have linked variability in biosafety compliance across laboratory settings to multiple factors, including institutional type, the nature of laboratory activities, staffing and resource availability, geographic and administrative contexts, and the professional experience of personnel (12–15). In this study, all participating NRLs were hosted by government agencies or universities but were distributed across 30 countries. The diversity of institutional arrangements, laboratory functions, and local regulatory contexts in which the NRLs operate, coupled with the fact that biosafety procedures are governed at the national level, likely contributes to differences in how biosafety procedures are implemented.

The high resistance of prions, combined with the dramatic consequences of accidental exposure, requires the implementation of tailored biosafety procedures. Five NRLs did not report the use of biosafety guidelines specifically designed for the safe management of TSE agent; in some cases, they adopted procedures intended for the management of generic pathogens. This highlights the need for wider dissemination of such guidelines. Similarly, some of the laboratories were not exclusively dedicated to TSE agents or did not separate the activities based on their level of risk, thus hindering the proper application of safety procedures. The lack of specific procedures for ELISA, western blot methods, pathology, immunohistochemistry, and experimental inoculations reported by certain laboratories involved in such activities further indicates room for improvement. These activities involve direct manipulation of high-risk tissues, generation of aerosols, and the use of sharp instruments, all of which may increase the likelihood of personnel exposure in the absence of standardised procedures. The implementation of activity-specific procedures could therefore play a key role in reducing exposure risks by ensuring consistent application of containment measures, appropriate use of personal protective equipment, and effective decontamination and waste management.

Other critical findings of the survey related to operational aspects of daily laboratory routine that could be improved with a modest investment of time and resources. Almost a quarter of the laboratories did not implement measures to prevent the use or reduce the risk of damage from sharp or pointed tools. At least one of the reported laboratory accidents was caused by stabbing with a sharp tool (9), which likely represents the major source of risk for laboratory personnel. This highlights the need to avoid the use of such tools wherever possible and to regulate their use when it is unavoidable. Furthermore, it is essential that TSE agents are always handled within safety cabinets, and laboratory equipment should be designed to fit within these cabinets. If this is not feasible, closed-system techniques for sample processing should be employed to minimize the risk of exposure. The implementation of decontamination procedures is also crucial to ensure the safety of the laboratory environment and the equipment.

Each stage of the process of sample management should be clearly defined and regulated by specific operating procedures to ensure the adoption of appropriate practices, minimise the risk of exposure, and respond appropriately to accidents. Training schemes should always be available to ensure that the personnel are familiar with these procedures.

The findings of the survey emphasized the importance of conducting a biosafety risk assessment and determining the level of biocontainment through a systematic review of practices, equipment, and facilities. This allows for the methodical identification and resolution of critical points, the implementation of necessary protective measures for the safety of the staff and the public, and a clear delineation of the activities that can be carried out safely within the laboratory. Despite this, the survey revealed that such assessments were incomplete in the NRLs. Risk assessment is a key biosafety tool. It identifies biological hazards, evaluates the likelihood of exposure and potential consequences, and informs the implementation of control measures. In practice, the role of a formal risk assessment in selecting appropriate mitigation measures may nevertheless be minimised or overlooked. This can result in a biological agent being classified within a generic group of pathogens, with the subsequent implementation of unspecific biosecurity measures that have been envisaged for that group. Such an approach fails to consider the risks associated with the specific characteristics of that agent, including the facilities, handling procedures, and protocols required for its safe management. This can lead to an underestimation of the likelihood (probability) of occurrence and the severity of potential harm. With regard to TSEs, the implementation of an appropriate risk assessment scheme leads to the conclusion that the measures to be implemented by TSE diagnostic laboratories are a combination of those required for biosafety level 2 and 3. Particular focus should be on a few crucial operations (puncture/cutting, surface contamination and aerosol production) to prevent environmental contamination and reduce the risk of exposure and infection for operators. The absence of risk assessment is often not only a technical problem, but also an organisational and cultural one. Risk assessment requires structural support, resource allocation, and the definition of responsibilities, all of which depend on management decisions. A lack of structured, periodically updated risk assessments may indicate limited management involvement in biosafety governance, which could affect the effectiveness of the biological risk management system.

Thanks to the 100% response rate, the survey is unlikely to be affected by sampling biases. Nonetheless, several limitations inherent to the survey method should be acknowledged. Self-reported data are vulnerable to response and social desirability biases, which may lead to overestimation of laboratory compliance with biosafety guidelines. No cross-validation methods were used to verify the accuracy of the responses. The reliance on binary and multiple-choice questions constrained the depth of insight into how and why certain practices are implemented. To mitigate this limitation, an open-ended question was included at the end of the questionnaire to allow participants to provide clarifications or add information they considered relevant. The comments collected offered useful details on the application of BSL procedures, and no major concerns regarding the questionnaire design were raised by respondents. Finally, this study was designed to assess biosafety procedures specifically within NRLs; therefore, the findings cannot be generalised to laboratories outside the NRL network.

The EURL-TSE will further encourage the full implementation of biosafety procedures specific for TSEs agents to address identified gaps and minimise risk to laboratory personnel. While the development of biosafety controls and improvement actions remains the responsibility of national authorities, the EURL-TSE promotes awareness and the exchange of best practices across NRLs. Given the abundance of bibliographical resources and guidelines, future actions of the EURL-TSE will focus on securing access to such resources and enhancing their application across all NRLs. To this end, the EURL-TSE has created a digital repository of guidelines and bibliographical sources on the subject (16). It also initiated biannual virtual meetings to elucidate critical issues emerged from the survey and to encourage the mutual exchange of experiences and good practices between NRL Directors. Each meeting will focus on a specific biosafety topic, with the decontamination of the laboratory environment and equipment being the first topic addressed. Moreover, these meetings will serve as a platform to qualitatively investigate the “root causes” of variability in compliance directly with the NRL Directors. Finally, a follow-up survey will be carried out to assess the effectiveness of the awareness and information-sharing efforts and to monitor progress over time; it will also include questions aimed at investigating precisely the institutional or economic barriers that may affect the laboratory biosafety.

The EURL-TSE will continue to promote the critical review and ongoing enhancement of biosafety procedures across European NRLs. As BSE and other transmissible spongiform encephalopathies persist in Europe, ensuring the safety of those working to detect and respond to these diseases remains crucial.

Statements Data availability statement The anonymised raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.

https://www.frontiersin.org/journals/public-health/articles/10.3389/fpubh.2025.1733350/full

that’s a frightening assessment, imho. i remember…

all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, provern, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd. ...terry

least we forget...

*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract

Detection of Human Prion Seeding Activity in Formalin-Fixed Paraffin-Embedded Archival Tissues

Soňa Baranová, Radoslav Matěj, Jiri G. Safar, Karel Holada

First published: 09 July 2025


Funding: This work was supported by the Ministry of Health of the Czech Republic (projects Conceptual Development of Research Organization), General University Hospital, Prague (VFN, 00064165) and Thomayer University Hospital (FTN, 00064190), the Czech Health Research Council (Project No. NU23-04-00173), by the project National Institute for Neurological Research (Program EXCELES, ID Project No. LX22NPO5107) — Funded by the European Union — Next Generation EU and by Charles University (projects Cooperation Medical Diagnostics and Basic Medical Sciences and GAUK No. 362521).

 ABSTRACT

Aims

Formalin-fixed paraffin-embedded (FFPE) samples, routinely used in neuropathology, represent an invaluable resource for studying rare diseases like transmissible spongiform encephalopathies (TSE). Despite fixation-induced protein cross-linking, prion seeding activity can be effectively detected using the seeding amplification assays. In this study, we employed the second-generation real-time quaking-induced conversion (RT-QuIC) assay to analyse and quantify human prion seeding activity in FFPE brain tissues.

Methods

FFPE frontal brain tissues were deparaffinised in xylene, followed by rehydration through descending concentrations of ethanol. The prion seeding activity in tissue homogenates was assessed by RT-QuIC assay utilising short recombinant hamster prion protein (rHaPrP90-231) as a substrate.

Results

A total of 60 samples, including 30 cases of confirmed TSE, comprising both sporadic and genetic forms, as well as 30 non-TSE controls, were analysed. Prion seeding activity has been detected in all TSE samples except one sCJD (VV2) and one GSS (P102L) case, corresponding to an assay sensitivity of 93.3%. Conversely, we did not detect any RT-QuIC positivity in the control group, resulting in 100% specificity. The mean 50% prion seeding dose of FFPE sporadic TSE samples was 107.8/g of brain tissue.

Conclusion

Our study emphasises high sensitivity and specificity of RT-QuIC assay for prion detection in archival human FFPE brain tissues and demonstrates its diagnostic reliability comparable to other tissue types even after years of storage. The applicability of FFPE samples in RT-QuIC assays facilitates retrospective diagnostics and provides logistical advantages for sample preservation and transportation.

SUMMARY

The RT-QuIC assay demonstrated high sensitivity and specificity in detecting prion seeding activity in FFPE brain tissues.

The sensitivity and specificity of the assay were comparable to frozen tissues. Prion seeding activity was detectable in FFPE tissues even after years of storage. Using FFPE samples facilitates retrospective studies and simplifies sample handling and transport.


Research Article

Prion seeding activity in DNA extractions: implications for laboratory biosafety

Sarah C. Gresch , Tamara Morrill , Maddy Ellis-Cramer , Maria Arifin , Lexi E. Frank , Jason C. BartzORCID Icon , Marc D. SchwabenlanderORCID Icon , Tiffany M. WolfORCID Icon , Gordon B. Mitchell , Jiewen GuanORCID Icon & Peter A. Larsen

Pages 1-16 | Received 23 Oct 2025, Accepted 14 Jan 2026, Published online: 29 Jan 2026 Cite this article https://doi.org/10.1080/19336896.2026.2619277 

ABSTRACT

Infectious prions (PrPSc) are largely resistant to proteolytic digestion, including proteinase K (PK) digestion. While nucleic acid extracts are generally considered non-infectious from a classical microbiology context (i.e. free of intact bacteria and viruses), we investigated whether standard DNA purification methods co-purify PrPSc, posing an unrecognized biosafety risk. Commercial DNA extraction kits can eliminate conventional pathogens but are likely ineffective against PrPSc due to resistance to kit reagents and enzymatic degradation. Two laboratories, the University of Minnesota Center for Prion Research and Outreach (MNPRO) and the Canadian Food Inspection Agency (CFIA), independently tested filter-based and magnetic bead-based DNA extraction kits using tissues from chronic wasting disease (CWD)-positive and -negative white-tailed deer (WTD; Odocoileus virginianus), as well as prion-infected and control Syrian hamster (Mesocricetus auratus) brains. CFIA used two filter-based kits (one automated, one manual), while MNPRO tested two manual kits (filter- and magnetic bead-based). PrPSc seeding activity was measured in extracted DNA and source tissues using real-time quaking-induced conversion (RT-QuIC). MNPRO found substantial to almost perfect agreement between RT-QuIC seeding activity of DNA eluates from both extraction methods and that of the source WTD tissue homogenate. CFIA optimized RT-QuIC to a 30-hour runtime, achieving 74% sensitivity and 94% specificity in 88 archived WTD DNA samples. Both laboratories concluded that commercial DNA extraction kits do not eliminate PrPSc, enabling carry-over into DNA eluates. Until infectivity is resolved by animal bioassay, DNA from PrPSc-positive tissues should be handled under biosafety protocols appropriate for the originating prion disease, with decontamination and containment procedures.

snip…

Both participating laboratories independently reached the same fundamental conclusion: standard DNA extraction procedures do not guarantee the removal of PrPSc. Until the infectivity of DNA extracts from TSE-positive tissues is fully characterized, our findings suggest that all nucleic-acid preparations derived from such materials should be handled as potentially infectious. Enhanced biosafety measures, validated workflows, and rigorous decontamination protocols will be essential to protect laboratory personnel, prevent cross-contamination, and ensure the continued safe advancement of genetic and molecular research in prion diseases.


CORRESPONDENCE| VOLUME 20, ISSUE 12, P981, DECEMBER 01, 2021

Safe laboratory management of prions and proteopathic seeds

Simon Mead Thomas Evans

on behalf of theAdvisory Committee for Dangerous Pathogens Transmissible Spongiform Encephalopathy Subgroup

Published: December, 2021 DOI: https://doi.org/10.1016/S1474-4422(21)00379-3

Prions, the infectious agents of fatal and transmissible neurodegenerative disorders in humans and animals, are comprised of assemblies of misfolded forms of prion protein (PrP). The death of a 33-year-old researcher of prion diseases from variant Creutzfeldt-Jakob disease (ie, the strain of disease that is derived from bovine spongiform encephalopathy) 9 years after a percutaneous exposure to prion-contaminated material, and the death from or diagnosis of prion disease in two other people in Europe after working in prion research, emphasises the importance of statutory guidance for laboratory safety when working with dangerous pathogens.1 People in numerous laboratories handling diagnostic blood, CSF, and other low-risk biofluid samples from patients with or suspected to have Creutzfeldt-Jakob disease have contacted us to suggest that the existing guidance was not sufficiently clear or proportionate. Evidence has accrued for the potential for proteins that are linked to neurodegenerative diseases, other than PrP, to adopt abnormal conformations, self-propagate, and cause transmissible pathologies and diseases in humans and laboratory animals.2, 3 These proteins share a range of pathological properties but are also distinct from prions in important ways, including that there are no known animal or human epidemics or established occupational risks. Experiments that involve inoculating, concentrating, or synthesising these so-called proteopathic seeds have become routine in the past decade, but no statutory guidance is available for safety. Human–human transmission of amyloid β proteopathic seeds has been observed in some specific circumstances that were also shown to transmit prion infection (eg, use of cadaver-derived human pituitary hormones or dura mater in neurosurgery) and can cause iatrogenic cerebral amyloid angiopathy and fatal brain haemorrhage after long latencies.4 The popularity of this field of research, and the long latencies that are to be expected for diseases that are caused by these proteopathic seeds, mean that occupational exposures might not yet have resulted in any clinical consequences. It is prudent, therefore, to consider potential risks from laboratory work involving these agents.

The UK's Advisory Committee for Dangerous Pathogens convened a subgroup to revise guidance for safe working with prions and to consider whether any measures were needed for work with proteopathic seeds, involving experts from research laboratories for prion and other neurodegenerative diseases, infectious disease specialists, pathologists, veterinarians, and health and safety experts. In the new guidance, we emphasise a distinction between high-risk CNS tissues and research samples that contain high concentrations of prions, which need to be managed in specialised laboratories with strict policies, and low-risk biofluids, such as blood and CSF, from patients who are suspected to have Creutzfeldt-Jakob disease with no or low concentrations of prions, which can be managed in a high-throughput diagnostic laboratory setting through adherence to appropriate general laboratory practices.

We also concluded that the poorly defined pathogenicity in humans of proteopathic seeds when prepared in concentrated forms for biochemical, structural, or transmission studies means that they should now be considered as hazard group 2 pathogens, necessitating work in a containment level 2 facility. We recommend a range of safety measures,5 including special attention to risk assessment and staff training; recording of accidental exposures; special caution with the use of any sharp tools to avoid percutaneous injury; work inside a microbiological safety cabinet; and the use of spill trays, absorbent material, and defined procedures to decontaminate equipment and spills to avoid contamination of the laboratory environment.

Importantly, we do not recommend any changes to existing procedures for the routine handling of tissues and biofluids from patients with non-prion neurodegenerative conditions for diagnostic or research purposes. We hope that this new guidance will be seen as proportionate and precautionary and help organisations to have increased confidence about the safety of their employees.5

We declare no competing interests. Members of the Advisory Committee for Dangerous Pathogens Transmissible Spongiform Encephalopathy Subgroup are listed in the appendix.

Supplementary Material

Download .pdf (.45 MB)


Supplementary appendix


https://www.thelancet.com/cms/10.1016/S1474-4422(21)00379-3/attachment/8e8e4017-5165-46a4-a60a-b8ae6525418e/mmc1.pdf



References 1.Brandel JP Vlaicu MB Culeux A et al. Variant Creutzfeldt-Jakob disease diagnosed 7·5 years after occupational exposure. N Engl J Med. 2020; 383: 83-85 View in Article Google Scholar

2.Lauwers E Lalli G Brandner S et al.

Potential human transmission of amyloid β pathology: surveillance and risks.

Lancet Neurol. 2020; 19: 872-878

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3.Jaunmuktane Z Brandner S

Invited review: the role of prion-like mechanisms in neurodegenerative diseases.

Neuropathol Appl Neurobiol. 2020; 46: 522-545

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4.Jaunmuktane Z Mead S Ellis M et al.

Evidence for human transmission of amyloid-beta pathology and cerebral amyloid angiopathy.

Nature. 2015; 525: 247-250



5.Department of Health and Social Care

Guidance: minimise transmission risk of CJD and vCJD in healthcare settings.


Date: Nov 27, 2012 Date accessed: November 2, 2021 

Article Info Publication History Published: December 2021 Identification DOI: https://doi.org/10.1016/S1474-4422(21)00379-3

Copyright © 2021 Elsevier Ltd. All rights reserved.

https://www.thelancet.com/article/S1474-4422(21)00379-3/fulltext



FRIDAY, DECEMBER 02, 2022

Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update


The importance of ongoing international surveillance for Creutzfeldt–Jakob disease

Neil Watson1, Jean-Philippe Brandel2, Alison Green1, Peter Hermann3, Anna Ladogana 4, Terri Lindsay1, Janet Mackenzie1, Maurizio Pocchiari 4, Colin Smith1, Inga Zerr3 and Suvankar Pal 1 ✉ 

Abstract | Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. Emerging evidence from peripheral tissue distribution studies employing high-sensitivity assays suggests that all forms of human prion disease carry a theoretical risk of iatrogenic transmission. Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments.


see also;




ACDP TSE subgroup minutes, agendas and papers, history


TUESDAY, NOVEMBER 1, 2022

SEAC Scientific Steering Committee on TSE Prion


iatrogenic transmissible Spongiform encephalopathy 

iatrogenic TSE PrP


also, i submitted several papers to different Government Entities on TSE PrP and its infectious properties, and not to forget, very important as well ENVIRONMENTAL RISK FROM TSE PrP…terry

RE: re-National Bio and Agro-Defense lab's safety procedures and Transmissible Spongiform Encephalopathy TSE Prion Disease

Dear Mr. Singletary,

Thank you for your interest in the NBAF and the public’s safety. Your thoughts have been shared with our program team and will be taken into consideration.

Sincerely,

DHS S&T Office of National Laboratories

Please continue to visit the S&T Office of National Laboratories website: http://www.dhs.gov/science-and-technology/onl

From: Terry Singeltary <flounder9@verizon.net> 

Sent: Tuesday, June 21, 2022 12:46 PM

To: khughes@ansi.org Cc: S&T Nat Labs <sandtnatlabs@HQ.DHS.GOV>; public-affairs@oig.usda.gov; S&T Nat Labs <sandtnatlabs@HQ.DHS.GOV>; ALFONSO.CLAVIJO@UDA.GOV; mmoser@themercury.com; vets <vets@HQ.DHS.GOV>; Media Inquiry <MediaInquiry@HQ.DHS.GOV>; S&T Media <stmedia@hq.dhs.gov>; DHS-Silicon-Valley <dhs-silicon-valley@hq.dhs.gov>; HSIN <hsin@hq.dhs.gov>; Cunningham, Stanley <stanley.cunningham@HQ.DHS.GOV>; letters@themercury.com; info@homelandcouncil.org

Subject: re-National Bio and Agro-Defense lab's safety procedures and Transmissible Spongiform Encephalopathy TSE Prion Disease

CAUTION: This email originated from outside of DHS. DO NOT click links or open attachments unless you recognize and/or trust the sender. Contact your component SOC with questions or concerns.

Greetings Honorable Director Kathryn Coulter Mitchell, Alfonso Clavijo, OIG, Department of Homeland Security et al,

I wish to kindly and urgently comment on the;

National Bio and Agro-Defense lab's safety procedures and Transmissible Spongiform Encephalopathy TSE Prion Disease

National Bio and Agro-Defense Facility Location: Manhattan, KS

The National Bio and Agro-Defense Facility (NBAF) will be a state-of-the-art biocontainment laboratory for the study of diseases that threaten both America’s animal agricultural industry and public health. DHS S&T is building the facility to standards that fulfill the mission needs of the U.S. Department of Agriculture (USDA) which will own, manage and operate (PDF, 16pp, 165 KB) the NBAF once construction and commissioning activities are complete. The NBAF will strengthen our nation’s ability to conduct research, develop vaccines, diagnose emerging diseases, and train veterinarians. The NBAF will be a national security asset and will meet the needs of the homeland security mission.

The United States currently does not have a laboratory facility with maximum biocontainment (BSL-4) space to study high-consequence zoonotic diseases affecting large livestock. The NBAF will be the first laboratory facility in the U.S. to provide BSL-4 laboratories capable of housing cattle and other large livestock. The NBAF will also feature a vaccine development module. For more information about the facility and intended use of its state-of-the-art features, please visit the USDA NBAF Program website.

DHS S&T leads construction activities for NBAF which remain underway. Current operations at the Plum Island Animal Disease Center (PIADC) will continue until the mission is transitioned to the NBAF.

The federal government will execute a plan to provide for seamless transition of the agricultural defense mission from PIADC to the NBAF that includes an overlap of operations to make certain there is no interruption of the critical science mission and operational capabilities.

In January 2020, USDA and DHS S&T signed a Memorandum of Understanding (MOU) to outline their ongoing strategic interagency partnership at NBAF focused on national security. The MOU establishes an initial framework for scientific collaboration and identifies current areas of opportunity for collaboration which include:

Threat Risk Assessment and Research Prioritization: To determine which transboundary, emerging animal diseases and zoonotic pathogens present the greatest risk to animal health, human health and national security. Outputs from this work will inform the process for research prioritization at NBAF. Research and Collaboration: To support the related USDA and DHS S&T food and agriculture missions including threat characterization and classified research; RDT&E involving biological countermeasures (vaccines, biotherapeutics and diagnostics); subject matter expert collaboration and information sharing; and partnerships.

Collaborative program areas may evolve over time. As a result, this MOU establishes a process for periodic review to ensure consistency with current missions and scopes of activities.

Current Project Status

Our Impact

Contact

Last Updated: 03/15/2022

https://www.dhs.gov/science-and-technology/national-bio-and-agro-defense-facility

National Bio and Agro-Defense Facility

https://www.usda.gov/sites/default/files/documents/nbaf-strategic-vision.pdf

https://www.dhs.gov/science-and-technology/national-biodefense-analysis-and-countermeasures-center

https://www.usda.gov/nbaf

NBAF officials detail some of federal disease lab's safety procedures

Megan Moser mmoser@themercury.com

Jun 18, 2022

https://themercury.com/news/nbaf-officials-detail-some-of-federal-disease-labs-safety-procedures/article_8310807b-78ce-5b3d-bf82-b5bb248df465.html

Greetings Honorable Director Kathryn Coulter Mitchell, Alfonso Clavijo, OIG, Department of Homeland Security et al,

I made a comment submission, back of book of the Galveston National Laboratory for Biodefense and Emerging Infectious Diseases Research Facility in Galveston: Environmental Impact Statement February 2005, pages 701 through page 729, of my submission, on my concerns of the TSE Prions, and it was printed in the back of that book. see;

With great urgency, I wish to submit the following on my Concerns with these New National Bio and Agro-Defense lab's, and safety protocols for the Transmissible Spongiform Encephalopathy TSE Prion disease, aka mad cow type disease, that are emerging now around the world in different species, most notable recently a new TSE Prion disease in Camels, presently called Camel Prion Disease, a new TSE prion disease in a new livestock species.


https://catalog.hathitrust.org/Record/100978478

https://play.google.com/books/reader?id=jCs3AQAAMAAJ&pg=GBS.RA10-PA5-IA1&hl=en

snip…end

Managing a Black Swan in health care: a lesson in transparency 

JOSEPH PEPE, MD PHYSICIAN FEBRUARY 2, 2026 

excerpt from On All Sides of the Bed: One physician’s/CEO’s journey.

In the early summer of 2013, our hospital collided with a Black Swan.

Before Europeans discovered Australia, they were familiar only with white swans. The discovery of black swans overturned a long-held assumption. In business and risk theory, a “Black Swan” refers to a rare, unpredictable event with massive consequences, a concept popularized by Nassim Nicholas Taleb. For us, that Black Swan arrived in the form of a patient.

He presented with neurological symptoms requiring brain surgery. Unbeknownst to anyone at the time, he had contracted Creutzfeldt-Jakob disease (CJD), a rare and fatal neurodegenerative disorder caused by an abnormal protein known as a prion. Unlike viruses or bacteria, prions contain no DNA or RNA. They are purely protein-based (and extraordinarily difficult to destroy).

The sterilization challenge

Hospitals rely on autoclaves (machines that use high-pressure steam) to sterilize surgical instruments. This method is highly effective against bacteria and viruses, but it is not completely reliable against prions. If a patient with undiagnosed CJD undergoes brain or spinal surgery, there is a theoretical risk that prions could survive standard decontamination. Instruments reused in subsequent neurosurgical cases could, in rare circumstances, transmit the disease.

Days after the operation, CJD became a possible diagnosis. By then, eight additional patients had undergone neurosurgery using the same instrument set. Definitive diagnosis requires biopsy or autopsy, meaning confirmation could take weeks. With a working diagnosis but no certainty, we had to assume the worst.

I immediately halted all neurosurgical procedures and quarantined the instruments. Typically, such decisions involve multiple committees and consultations. But as a physician-internist serving as hospital CEO, I occupied both roles: administrator and clinician. I made it clear from the outset that patient safety and community trust would take precedence over financial, legal, or reputational concerns.

The decision to disclose

Based on the clinical progression and testing, we were approximately 98 percent certain the patient had CJD. Even without definitive confirmation, I decided we would inform the potentially exposed patients in person and disclose the situation to the community shortly thereafter.

This decision was controversial. Some argued that disclosure created unnecessary fear, especially given the extremely low risk of transmission. Others worried about lawsuits, reputational harm, financial losses, and job security (including my own). After all, only a handful of documented cases worldwide had ever linked CJD transmission to contaminated neurosurgical instruments.

But transparency mattered. Those patients deserved the information so they could make informed decisions about future surgeries, organ donation, and their own medical care. What if they underwent neurological procedures elsewhere, unknowingly placing others at risk? What if they moved away and were lost to follow-up? If I were the patient, I would want to know.

I believed that honest communication, ongoing education, and counseling could reduce anxiety rather than inflame it. If the diagnosis proved correct, patients could make informed end-of-life decisions or choose to participate in CJD-related research. Silence would serve no one.

Destruction and cost

Once the diagnosis was confirmed, we consulted experts and sought guidance from federal health agencies. The recommendations for decontamination were inconsistent and, frankly, unsatisfying. Ultimately, I ordered the destruction of the instrument set, at a cost exceeding $200,000.

I could not guarantee complete decontamination. The potential benefit of saving the instruments did not outweigh even a minuscule chance of exposing another patient. Our chief financial officer initially pushed back, but when I asked whether he would allow those instruments to be used in his own brain surgery, the discussion ended.

The outcome of transparency

After informing staff, the hospital board, local and state officials, neighboring hospitals, and public health authorities, we held a press conference. We explained what had happened and how we were responding. The story spread quickly, appearing in national newspapers, on cable news, and across social media.

We anticipated sensationalism, but by communicating early and clearly, we controlled the narrative. Most coverage was accurate. When one outlet incorrectly labeled the case “mad cow disease,” our team promptly corrected the error.

As the media attention faded, the response surprised us. The community expressed trust rather than fear. Editorials praised the hospital for prioritizing transparency and patient welfare. Multiple regulatory agencies visited our small institution, organizations known more for citations than compliments. Yet we received no deficiencies and considerable praise.

I knew we had done something right when a large Boston marketing firm called, not to offer crisis management, but to congratulate us. The caller told me his firm could not have handled the situation better.

In health care leadership, crises reveal values more clearly than mission statements ever can. In that moment, we chose transparency over protection, ethics over expediency, and trust over fear. It was the right decision, then and now.

Joseph Pepe is a physician executive.

Founded in 2004 by Kevin Pho, MD, KevinMD.com is the web’s leading platform where physicians, advanced practitioners, nurses, medical students, and patients share their insight and tell their stories.

TAGGED AS: HOSPITAL-BASED MEDICINE

https://kevinmd.com/2026/02/managing-a-black-swan-in-health-care-a-lesson-in-transparency.html

Dr. Pepe, Outstanding Sir! 

iatrogenic TSE/CWD, my greatest fear, here’s why. 

all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd, PLUS, SPORADIC CJD HAS NOW BEEN LINKED TO ATYPICAL AND TYPICAL BSE, SCRAPIE, AND NOW CWD? what if? what if cwd to humans has already happened and being misdiagnosed as sporadic cjd? what about every hospital surgical unit exposed to the CWD TSE Prion from deer and elk, friendly fire from humans, iatrogenic friendly fire?

one of the old studies that has always stuck out in my mind, one that the late great Dr. Gibbs, Gajdusek, et al did way back, and to this day is still amazes me...

*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract

TSE Prions and Infection Control

Infection control for CJD. CDC. May 13, 2024. Accessed December 9, 2024.

https://www.cdc.gov/creutzfeldt-jakob/hcp/infection-control/index.html

WHO infection control guidelines for transmissible spongiform encephalopathies: report of a WHO consultation, Geneva, Switzerland, 23-26 March 1999. World Health Organization. Accessed December 9, 2024. 

https://iris.who.int/handle/10665/66707

Clinical overview of Creutzfeldt-Jakob disease (CJD). CDC. May 13, 2024. Accessed December 9, 2024. 

https://www.cdc.gov/creutzfeldt-jakob/hcp/clinical-overview/index.html

Prion diseases are a group of rare, neurodegenerative brain disorders. CJD Foundation. Accessed December 9, 2024. 

https://cjdfoundation.org/types-of-prion-disease/#acquired

Classic Creutzfeldt-Jakob disease. CDC. May 13, 2024. Accessed December 9, 2024. 

https://www.cdc.gov/creutzfeldt-jakob/about/index.html

CJD diagnostic criteria. CDC. May 13, 2024. Accessed December 9, 2024. 

https://www.cdc.gov/creutzfeldt-jakob/hcp/clinical-overview/diagnosis.html

Asked & answered: device disposal. Association for the Advancement of Medical Instrumentation. March 1, 2020. Accessed December 9, 2024. 

https://array.aami.org/content/news/asked-answered-device-disposal

ANSI/AAMI ST79:2017 with amendments A1:2020, A2:2020, A3:2020, A4:2020: comprehensive guide to steam sterilization and sterility assurance in health care facilities. Association for the Advancement of Medical Instrumentation. Accessed December 9, 2024. 

https://aami.org/standards/featured-standards/ansi-aami-st79

Clinical overview of variant Creutzfeldt-Jakob disease. CDC. November 12, 2024. Accessed December 9, 2024. 

https://www.cdc.gov/variant-creutzfeldt-jakob/hcp/clinical-overview/index.html

About prion diseases. CDC. April 22, 2024. Accessed December 9, 2024. 

https://www.cdc.gov/prions/about/index.html

Prion Safety Laboratory Swipe Test

DOI: 10.3791/67889

February 14th, 2025

Sara M. Simmons1, Qi Yuan1, Jason C. Bartz1

1Department of Medical Microbiology and Immunology, School of Medicine, Creighton University

Summary

A method to assess commonly used areas in laboratory settings for prion contamination and effective decontamination is lacking. The protocol described here provides key fundamentals for implementing a laboratory prion safety swipe test that can easily be modified to meet the individual needs of specific laboratories.

Abstract

Transmission of iatrogenic prion disease has occurred from contaminated neurosurgical tools, transplant materials, and occupational exposure to prion-contaminated laboratory tools. Prions cause disease by the templated misfolding of the normal cellular form of the prion protein, PrPC, into the misfolded and pathogenic form PrPSc and are invariably fatal. Reducing iatrogenic and occupational prion transmission is challenging. First, prions can bind to and persist on surfaces for long periods of time. Second, prions are highly resistant to inactivation. Given this, surfaces can retain infectivity for long periods of time following ineffective decontamination. Not only can this pose a potential occupational risk for prion laboratory workers, but it could potentially cross-contaminate laboratory experiments utilizing sensitive prion amplification techniques. The protocol described here for a prion safety laboratory swipe test includes steps for the identification and documentation of high-traffic laboratory areas, recommended swabbing controls to ensure the validity of results, steps to identify proper responses to positive surface swabbing sites, representative results from prion swipe testing, as well as potential artifactual results. Overall, the prion safety laboratory swipe test can be implemented as part of a broader prion safety program to assess decontamination of surfaces, monitor common spaces for prion contamination, and implement the documentation of prion decontamination status.

Introduction

Prion diseases are invariably fatal neurodegenerative diseases with no known treatment or cure. Prion diseases are caused by PrPSc, the misfolded and pathogenic form of the normal cellular form of the prion protein1,2,3,4,5, PrPC. Prion diseases are known to affect humans and several other animal species. One human prion disease, Creutzfeldt-Jakob Disease, CJD, has three known etiologies: sporadic, inherited, and acquired. Acquired CJD can occur as a result of accidental transmission (iatrogenic and occupational) and is thought to be the cause of Kuru in the Fore people of Papua New Guinea6.

Prion transmission has been associated with prion-contaminated medical devices and transplant materials7,8,9,10,11,12,13,14,15,16,17. Iatrogenic transmission of CJD can occur via blood, tissue, or from prion-contaminated surfaces18,19,20. For example, iatrogenic CJD can develop in patients following an electroencephalogram with electrodes previously used on an individual in the preclinical stage of CJD who then later succumbed to CJD21. More recent laboratory-based occupational transmission has also occurred where a laboratory worker contracted prion disease via a skin puncture with forceps used to handle brain slices from an animal infected with sheep-adapted BSE22,23. Such transmission scenarios could occur within clinical, laboratory, and diagnostic laboratory settings where prion samples are handled.

Prions resist common disinfection techniques and can persist and remain infectious on surfaces for extended periods of time24,25,26,27,28,29. Common disinfection techniques such as the use of ethanol, phenolic cleaners, hydrogen peroxide, various forms of radiation, and formaldehyde are inadequate for the inactivation of prions, allowing surfaces to retain infectivity30,31,32,33,34,35,36,37. These characteristics contribute to the transmission of prions during iatrogenic and occupational exposure.

Methods for the detection of environmental prions have only recently been developed. An environmental swabbing method coupled with real-time quaking-induced conversion (RT-QuIC) can assess residual prion infectivity from environmental surfaces as well as common laboratory surfaces following ineffective disinfection38,39,40,41,42. Here, we describe how this technique can be incorporated into a broader prion safety program. Overall, this method can allow for the monitoring of laboratory-dependent disinfection protocols, the investigation and proper documentation of contamination status, which can help ensure the validity of experiments by minimizing cross-contamination, the assessment of shared use spaces for prion contamination and allows for directional retraining of personnel based on commonly contaminated areas.

Protocol

snip…see;

https://app.jove.com/t/67889/prion-safety-laboratory-swipe-test

France issues moratorium on prion research after fatal brain disease strikes two lab workers

By Barbara CasassusJul. 28, 2021 , 4:35 AM

PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.

If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.

The aim of the moratorium, which affects nine labs, is to “study the possibility of a link with the [new patient’s] former professional activity and if necessary to adapt the preventative measures in force in research laboratories,” according to a joint press release issued by the five institutions yesterday.

“This is the right way to go in the circumstances,” says Ronald Melki, a structural biologist at a prion lab jointly operated by the French national research agency CNRS and the French Alternative Energies and Atomic Energy Commission (CEA). “It is always wise to ask questions about the whole working process when something goes wrong.” "The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community, which is a small 'familial' community of less than 1000 people worldwide," Emmanuel Comoy, deputy director of CEA's Unit of Prion Disorders and Related Infectious Agents, writes in an email to Science. Although prion research already has strict safety protocols, "it necessarily reinforces the awareness of the risk linked to these infectious agents," he says.

In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.

A scientist with inside knowledge says the new patient, a woman who worked at INRAE’s Host-Pathogen Interactions and Immunity group in Toulouse, is still alive. French authorities were apparently alerted to her diagnosis late last week. The press release suggests it’s not yet clear whether the new case is vCJD or “classic” CJD, which is not known to be caused by prions from animals. Classic CJD strikes an estimated one person per million. Some 80% of cases are sporadic, meaning they have no known cause, but others are genetic or contracted from infected human tissues during transplantations. The two types of CJD can only be distinguished through a postmortem examination of brain tissue.

Lab infections are known to occur with many pathogens, but exposure to CJD-causing prions is unusually risky because there are no vaccines or treatments and the condition is universally fatal. And whereas most infections reveal themselves within days or weeks, CJD’s average incubation period is about 10 years.

For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.

In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.

“The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community.” Emmanuel Comoy, French Alternative Energies and Atomic Energy Commission

INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)

Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.

Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)

The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.

In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.

After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors' report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.

The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.

The moratorium will "obviously" cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, and will shift some of its efforts to those.

Although Jaumain’s diagnosis upset many in the field, it hasn't led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”

With reporting by Martin Enserink.

Posted in: EuropeHealthScientific Community

doi:10.1126/science.abl6587

https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab

Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure

Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.

Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure

TO THE EDITOR:

We report a case of variant Creutzfeldt–Jakob disease (CJD) that was plausibly related to accidental occupational exposure in a technician who had handled murine samples contaminated with the agent that causes bovine spongiform encephalopathy (BSE) 7.5 years earlier.

In May 2010, when the patient was 24 years of age, she worked in a prion research laboratory, where she handled frozen sections of brain of transgenic mice that overexpressed the human prion protein with methionine at codon 129. The mice had been infected with a sheep-adapted form of BSE. During this process, she stabbed her thumb through a double pair of latex gloves with the sharp ends of a curved forceps used to handle the samples. Bleeding was noted at the puncture site.

In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. In November 2018, an examination of a sample of cerebrospinal fluid (CSF) obtained from the patient was normal. Magnetic resonance imaging (MRI) of the brain showed a slight increase in the fluid-attenuated inversion recovery (FLAIR) signal in the caudates and thalami (Fig. S1A and S1B in the Supplementary Appendix, available with the full text of this letter at NEJM.org). In January 2019, she became depressed and anxious and had memory impairment and visual hallucinations. There was hypertonia on the right side of her body. At that time, an analysis of CSF for 14-3-3 protein was negative. In March 2019, MRI showed an increased FLAIR signal in pulvinar and dorsomedial nuclei of thalami (Fig. S1C through S1E).

Figure 1.

Detection of Abnormal Prion Protein in Biologic Fluid Samples and Postmortem Findings.

The patient was found to be homozygous for methionine at codon 129 of the prion protein gene without mutation. An analysis of a sample of CSF on real-time quaking-induced conversion analysis was negative for a diagnosis of sporadic CJD. However, an analysis of plasma and CSF by means of protein misfolding cyclic amplification was positive for the diagnosis of variant CJD (Figure 1A and 1B). The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD (Figure 1C and 1D). Western blot analysis showed the presence of type 2B protease-resistant prion protein in all sampled brain areas. The clinical characteristics of the patient and the postmortem neuropathological features were similar to those observed in 27 patients with variant CJD who had previously been reported in France.1 (Additional details are provided in the Supplementary Appendix.)

There are two potential explanations for this patient’s condition. Oral transmission from contaminated cattle products cannot be ruled out because the patient was born at the beginning of the French BSE outbreak in cattle. However, the last two patients who had confirmed variant CJD with methionine homozygosity at codon 129 in France and the United Kingdom died in 2014 and 2013, respectively, which makes oral transmission unlikely. In France, the risk of variant CJD in 2019 was negligible or nonexistent in the post-1969 birth cohort.2

Percutaneous exposure to prion-contaminated material is plausible in this patient, since the prion strain that she had handled was consistent with the development of variant CJD.3 The 7.5-year delay between the laboratory accident and her clinical symptoms is congruent with the incubation period in the transfusion-transmitted form of the disease. The ability of this strain to propagate through the peripheral route has been documented, and experimental studies with scrapie strains have shown that scarification and subcutaneous inoculation are effective routes.4,5 The last known Italian patient with variant CJD, who died in 2016, had had occupational contact with BSE-infected brain tissues, although subsequent investigation did not disclose a laboratory accident (Pocchiari M, Italian Registry of CJD: personal communication). Thus, the last two cases of variant CJD outside the United Kingdom have been associated with potential occupational exposure. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings, as outlined in the Supplementary Appendix.

Jean-Philippe Brandel, M.D. Assistance Publique–Hôpitaux de Paris, Paris, France

M. Bustuchina Vlaicu, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France

Audrey Culeux, B.Sc. INSERM Unité 1127, Paris, France

Maxime Belondrade, M.Sc. Daisy Bougard, Ph.D. Etablissement Français du Sang, Montpellier, France

Katarina Grznarova, Ph.D. Angeline Denouel, M.Sc. INSERM Unité 1127, Paris, France

Isabelle Plu, M.D. Elodie Bouaziz-Amar, Pharm.D., Ph.D. Danielle Seilhean, M.D., Ph.D. Assistance Publique–Hôpitaux de Paris, Paris, France

Michèle Levasseur, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France

Stéphane Haïk, M.D., Ph.D. INSERM Unité 1127, Paris, France stephane.haik@upmc.fr

Supported by a grant (ANR-10-IAIHU-06) from Programme d’Investissements d’Avenir and Santé Publique France.

Disclosure forms provided by the authors are available with the full text of this letter at NEJM.org.

5 References

July 2, 2020

N Engl J Med 2020; 383:83-85

DOI: 10.1056/NEJMc2000687

Metrics

https://www.nejm.org/doi/full/10.1056/NEJMc2000687

34 year old Doctor Orthopedic Surgeon dies from CJD

Dr. Adam Thomas Dialectos

1987 - 2021

BORN

April 29, 1987

DIED

June 21, 2021

FUNERAL HOME

Bean Funeral Homes & Crematory Inc

1605 Rockland St

Reading, PA 19604

UPCOMING SERVICE

Visitation

Jun, 24 2021

9:00a.m. - 11:00a.m.

Saints Constantine & Helen Greek Orthodox Church

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On Monday June 21, 2021, Dr. Adam Thomas Dialectos, loving husband, father, son, brother, uncle, Nouno, friend at the age of 34. Adam was born on April 29, 1987 in Reading, PA to Athan and Gretchen Dialectos. Adam was a 2005 graduate of Governor Mifflin High School, before receiving his degree in Health Sciences from James Madison University in 2009. Adam attended Philadelphia College of Osteopathic Medicine for medical school and his subsequent residency in orthopedic surgery. Adam was completing his Spine Surgery Fellowship at New England Baptist Hospital in Boston, Massachusetts. On February 7, 2019 Adam married the love of his life and girlfriend of 12 years, Lindsey (Schuler) Dialectos. They brought a beautiful baby boy into this world on January 6, 2021, Athananosis Adam Dialectos. Adam’s passion in life was unceasingly seeking to help others, emphasized by his desire to be a surgeon— a decision he made in his early elementary years. Adam continued this love of medicine throughout his life, which led to his achieving of the Henrietta and Jack Avart Memorial Award in 2019, awarded to the Orthopedic surgery resident who exhibited unparalleled excellence in their field during the residency program. This passion to learn, teach and support was truly understood through the patients whose lives Adam touched. When it came to his patients and coworkers, there was never a job too small for Adam. Those who knew Adam saw his personality shine through in so many other aspects of his life. Adam loved traveling. Some of his most memorable trips were with his wife, and countless snowboard trips with his brother, family, and friends. Adam loved everyone he was around; he loved and was loved by so many. Adam was truly one in a million. 

Adam is survived by his loving wife, Lindsey, and their son, Athan Adam; His father and mother, Athan and Gretchen; His brother Jordan and sister-in-law Megan, and their daughter Livia, Adam’s Goddaughter. His sister, Rachel, and her significant other, Bo Wagner. Furthermore, Adam is survived by his Yiayia, Joanne Dialectos, wife of the late George Dialectos; his Pop Pop, Donald Harford, husband of the late Nancy Servent; his Aunt Angel and Uncle Scott Helm; his Aunt Kelly and Uncle Darrell Markley. Adam was preceded in death by his Aunt Maria and Uncle Bob Care. Funeral Service will be held at Saints Constantine & Helen Greek Orthodox Church, 1001 East Wyomissing Blvd. Reading on Thursday June 24th. Father Theodore Petrides and Father Thomas L. Pappalas will officiate. Interment will follow at Charles Evans Cemetery. The family will receive relatives and friends at Saints Constantine & Helen Greek Orthodox Church from 9:00am to 11:00amwith services beginning at 11:00. In lieu of flowers, contributions may be made to the CJD Foundation at 3634 West Market Street Suite 110 Akron, Ohio 44333 or cjdfoundation.org in remembrance of Dr. Adam Dialectos. Donations may also be made to Saints Constantine & Helen Greek Orthodox Church. Bean Funeral Home, 1605 Rockland Street, Hampden Heights, is in charge of arrangements and online condolences may be made at www.beanfuneralhomes.com.

To plant trees in memory, please visit our Sympathy Store.

Published by Reading Eagle from Jun. 22 to Jun. 24, 2021.

https://www.legacy.com/us/obituaries/readingeagle/name/adam-dialectos-obituary?pid=199144663

Our sincere condolences to the Family and Friends of Dr. Adam Thomas Dialectos.

I can't help but ponder, as a Orthopedic Surgeon, Spine Surgery Fellowship, and what the good Doctors work curtailed, i can't help but think this is a potential case of iatrogenic CJD. surgery on humans, i would imagine cadavers as well…terry

Volume 26, Number 8—August 2020

Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons

https://creutzfeldt-jakob-disease.blogspot.com/2020/08/sporadic-creutzfeldt-jakob-disease.html

Saturday, January 23, 2021

Improved surveillance of surgical instruments reprocessing following the variant Creutzfeldt-Jakob disease crisis in England: findings from a 3-year survey

https://itseprion.blogspot.com/2021/01/improved-surveillance-of-surgical.html

https://creutzfeldt-jakob-disease.blogspot.com/2019/09/

SUNDAY, JULY 19, 2020

Joseph J. Zubak Orthopaedic surgeon passed away Monday, July 6, 2020, Creutzfeldt-Jakob Disease (CJD)

https://creutzfeldt-jakob-disease.blogspot.com/2020/07/joseph-j-zubak-orthopaedic-surgeon.html

O17 Evaluation of iatrogenic risk of CJD transmission associated with corneal transplantation

Douet JY. (1), Cassard H. (1), Huor A. (1), Lacroux C. (1), Haïk S. (2), Lugan S. (1), Tillier C. (1), Aron N. (1), Ironside J.W. (3), Andreoletti O. (1) (1) UMR INRA-ENVT 1225, Ecole Nationale Vétérinaire de Toulouse, France. (2) Université Pierre et Marie Curie, UMR-S 1127, CNRS UMR 722, Institut du Cerveau et de la Moelle Epinière, G.H. Pitié-Salpêtrière, Paris, France. (3) National CJD Research and Surveillance Unit Centre for Clinical Brain Sciences, University of Edinburgh, UK.

Sporadic Creutzfeldt–Jakob disease (sCJD) has been documented to be accidentally transmitted by contaminated corneal transplants. To date, only one case is considered as definite, while 5 other suspect cases are classified as probable or possible. However, the specific transmission risk associated with this widely-performed transplantation procedure has never been studied.

In this study, bioassays in transgenic mice expressing the human PrP confirmed the presence of infectivity in the cornea of 2 sCJD patients. Infectivity was also detected in other ocular tissues (optic nerve, retina, vitreous body, choroid and lacrymal gland) from one of these patients. Based on these results, we investigated the presence of infectivity in the cornea of different TSE animal models. In conventional mice (RML strain) as well as in sheep (PG127 scrapie), infectivity could only be detected in the corneas collected at the late stage of the disease incubation phase. In parallel to these experiments, corneas collected at different stages of the incubation period in infected mice and sheep were grafted into healthy recipients.

Our results showed that corneas collected during the late asymptomatic phase or in affected animals were able to transmit TSE infectivity. Importantly, after the death of the recipients (up to 2.5 years after surgery) infectivity could still be detected in the grafted cornea.

These data confirm the potential for sCJD transmission by corneal grafts. They also provide crucial data for the assessing the TSE transmission risk associated with various other ophthalmologic procedures.

Prion Conference 2018 Abstract

O17 Evaluation of iatrogenic risk of CJD transmission associated with corneal transplantation


Friday, February 16, 2024 

The Eyes are the windows to Our Souls, and a Potential Pathway for the TSE Prion disease, what if?


Friday, January 29, 2021 

Scientists identify locations of early prion protein deposition in retina, what if? 

https://itseprion.blogspot.com/2021/01/scientists-identify-locations-of-early.html

FRIDAY, SEPTEMBER 06, 2019

Disinfection of Multi-Use Ocular Equipment for Ophthalmological Procedures: A Review of Clinical Effectiveness, Cost-Effectiveness, and Guidelines

https://creutzfeldt-jakob-disease.blogspot.com/2019/09/disinfection-of-multi-use-ocular.html 

some history ;

Thursday, June 24, 2021

34 year old Doctor Orthopedic Surgeon dies from CJD, what about iatrogenic CJD?

https://itseprion.blogspot.com/2021/06/34-year-old-doctor-orthopedic-surgeon.html

Thursday, April 12, 2012 

Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010 Eurosurveillance, Volume 17, Issue 15, 12 April 2012 Research articles 
  1. Terry S. Singeltary Sr. Doctor Antonio Ruiz Villaespesa, pathologist and CJD researcher deceased because of Creutzfeldt-Jakob Disease SPAIN. 21 Apr 2009. [Accessed 11 Apr 2012]. In: Monitoring the occurrence of emerging forms of CJD [blog]. Available from: http://cjdusa.blogspot.com.es/2009/04/doctor-antonio-ruiz-villaespesa.html
https://web.archive.org/web/20121004130010/http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=20144


http://creutzfeldt-jakob-disease.blogspot.com/2012/04/health-professions-and-risk-of-sporadic.html

SATURDAY, MARCH 16, 2019

Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) Guidance for Industry and Food and Drug Administration Staff Document issued on March 15, 2019 Singeltary Submission


TUESDAY, MARCH 12, 2019

Early preclinical detection of prions in the skin of prion-infected animals


SUNDAY, MARCH 10, 2019

National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated Feb 1, 2019 Variably protease-sensitive prionopathy VPSPr


TUESDAY, NOVEMBER 20, 2018

CDC Eyes of CJD patients show evidence of prions concerns for iatrogenic transmission


SUNDAY, JANUARY 17, 2016

Of Grave Concern Heidenhain Variant Creutzfeldt Jakob Disease


FRIDAY, DECEMBER 04, 2009 

New guidance on decontamination of trial contact lenses and other contact devices has been revealed for CJD AND vCJD New DoH guidance on decontaminating lenses

December 4th, 2009

New guidance on decontamination of trial contact lenses and other contact devices has been revealed.

The latest recommendations from the Department of Health’s Advisory Committee on Dangerous Pathogens (ACDP) replace previous guidance issued amid fears that Creutzfeldt-Jakob Disease (CJD) and variant CJD (vCJD), the human form of bovine spongiform encephalopathy (BSE, ‘mad cow disease’), could theoretically be transmitted from person to person by contact lenses and other devices such as tonometer heads and diagnostic lenses.

Details were discussed at the British Contact Lens Association’s Pioneers’ Conference in London late in November.

Professor Roger Buckley (pictured), a member of the ophthalmology subgroup of the ACDP’s Transmissible Spongiform Encephalopathy Working Group established to review this advice, told BCLA members at the Royal Society of Medicine that there had been no known cases of transmission of CJD/vCJD resulting from contact lens wear or diagnostic examination, and there was now thought to be a low level of risk of infectivity of the cornea and ocular surface.

Under the new guidance, six steps are required to minimise the risk of transmission via re-used contact devices. The lens or device should be: decontaminated immediately after contact with the eye surface; rinsed in Water for Irrigation BP (not tap water) for not less than 30 seconds; cleaned on all surfaces with a liquid soap or detergent, then rinsed in Water for Irrigation BP for a further 30 seconds; immersed in a freshly-prepared solution of sodium hypochlorite providing 10,000ppm of available chlorine for ten minutes; rinsed in three changes of Water for Irrigation BP for a total of not less than ten minutes; shaken to remove excess water, dried with a disposable tissue, and stored dry in a suitable container.

Any further measure (such as autoclaving) can then be carried out, if this is necessary and if the device is designed to withstand such a process. Otherwise, it is ready for immediate re-use.


Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex C ANNEX C General principles of decontamination and waste disposal


Alert to Urological Surgeons TRANSRECTAL PROSTATIC BIOPSY IN MEN AT RISK OF VARIANT CJD


Ophthalmology

The Ophthalmology subgroup met twice in 2008 on 7th April and 20th June to discuss issues relating to CJD infection control in ophthalmology. The following topic groups were identified: 17 • Anterior eye • Posterior eye • Assessment tool • Decontamination of ophthalmic surgical instruments • Examination, diagnostic equipment and contact lenses • CJD incident management Members were assigned to relevant topic groups, and discussions and research were coordinated by a topic group lead. The topic groups then produced draft guidance on their particular areas of expertise towards the end of 2008. Pathology (Annex K) The Working Group drafted guidelines for pathologists and pathology laboratories for the handling of tissues from patients with, or at risk of, CJD. This document (Annex K of the Working Group guidance) is aimed at pathologists and individuals working in pathology laboratories who handle tissues from patients. It aims to ensure that laboratory staff are aware of risk factors for CJD prior to carrying out procedures on tissues. The draft annex was sent out for a limited consultation with representatives from the Royal College of Pathologists, the Institute for Biomedical Sciences, the British Neuropathological Society and the Health and Safety Executive. The Annex was approved by the Working Group at their December 2008 meeting. Annex K has since been published at:


Pre-surgery assessment (Annex J) The updates to Annex J were approved for publication by the Working Group at their February 2008 meeting, subject to some minor adjustments. The Annex was then signed off by the ACDP Chairman and published on 1st May 2008 at:


see full text ;


Friday, July 17, 2009

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009


Six steps will minimise risk from trial lenses

Revised guidance on the decontamination of trial contact lenses and other contact devices has been released by the Department of Health's (DoH) Advisory Committee on Dangerous Pathogens (ACDP).

The new guidance replaces previous advice issued amid fears that Creutzfeldt-Jakob disease (CJD) and variant CJD, the human form of bovine spongiform encephalopathy (BSE), could theoretically be transmitted from person to person by contact lenses, tonometer heads and diagnostic lenses.

Under the new guidance, six steps are required to minimise the risk of transmission via re-used contact devices. The lens or device should be:

Decontaminated immediately after contact with the eye surface

Rinsed in Water for Irrigation BP (not tap water) for not less than 30 seconds

Cleaned on all surfaces with a liquid soap or detergent, then rinsed in Water for Irrigation BP for a further 30 seconds

Immersed in a freshly prepared solution of sodium hypochlorite providing 10,000ppm of available chlorine for 10 minutes

Rinsed in three changes of Water for Irrigation BP for a total of not less than 10 minutes

Shaken to remove excess water, dried with a disposable tissue, and stored dry in a suitable container.

Any further measure (such as autoclaving) can then be carried out, if this is necessary and if the device is designed to withstand such a process. Otherwise, it is ready for immediate re-use.

Speaking at the British Contact Lens Association's (BCLA) Pioneers' Conference in London on November 26, Professor Roger Buckley, a member of the ophthalmology subgroup of the ACDP's Transmissible Spongiform Encephalopathy Working Group set up to review the advice, said that there had been no known cases of transmission of CJD resulting from contact lens wear or diagnostic examination and there was now thought to be a low level of risk of infectivity of the cornea and ocular surface.

Professor Buckley told BCLA members that if the recommendations were not followed practitioners could be 'on their own medico-legally'.

The full guidance, entitled Managing CJD/vCJD risk in ophthalmology can be found on the DoH website.


Wednesday, August 20, 2008

Tonometer disinfection practice in the United Kingdom: A national survey


Friday, December 04, 2009

New guidance on decontamination of trial contact lenses and other contact devices has been revealed for CJD AND vCJD



Flexible Bronchoscopes and Updated Recommendations for Reprocessing: FDA Safety Communication


Date Issued: June 25, 2021

The U.S. Food and Drug Administration (FDA) is providing updated information about medical device adverse event reports and recommendations for health care providers on bronchoscopes.

The FDA is providing the following new recommendations:

Consider using a single-use bronchoscope in situations where there is increased risk of spreading infection (for example, multidrug resistant microorganisms, immunocompromised patients, or patients with prion disease) or when there is no support for immediate reprocessing of the bronchoscope.

When treating patients with Coronavirus Disease 2019 (COVID-19), refer to recent recommendationsExternal Link Disclaimer from the American Association for Bronchology & Interventional Pulmonology (AABIP).

https://www.fda.gov/medical-devices/safety-communications/flexible-bronchoscopes-and-updated-recommendations-reprocessing-fda-safety-communication?utm_medium=email&utm_source=govdelivery

i tried to tell GUT journal, and Bramble et al this way back, decades ago...terry

were not all CJDs, even nvCJD, just sporadic, until proven otherwise?

Terry S. Singeltary Sr., P.O. BOX, Bacliff, Texas 77518 USA

########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############

Professor Michael Farthing wrote:

Louise Send this to Bramble (author) for a comment before we post. Michael

-----Original Message-----

From: Terry S. Singeltary Sr. [mailto:flounder@wt.net] ;

Sent: 03 June 2002 17:14

To: lcamp@bmjgroup.com

Subject: gutjnl_el;21 Terry S. Singeltary Sr. (3 Jun 2002) "CJDs (all human TSEs) and Endoscopy Equipment"

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

Date submitted: 3 Jun 2002 eLetter ID: gutjnl_el;21

Gut eLetter for Bramble and Ironside 50 (6): 888

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

Name: Terry S. Singeltary Sr. Email: flounder@wt.netTitle/position: disabled {neck injury} Place of work: CJD WATCH IP address: 216.119.162.85 Hostname: 216-119-162-85.ipset44.wt.net Browser: Mozilla/5.0 (Windows; U; Win98; en-US; rv:0.9.4) Gecko/20011019 Netscape6/6.2

Parent ID: 50/6/888

Citation: Creutzfeldt-Jakob disease: implications for gastroenterology

M G Bramble and J W Ironside Gut 2002; 50: 888-890 (Occasional viewpoint)

http://www.gutjnl.com/cgi/content/abstract/50/6/888

http://www.gutjnl.com/cgi/content/full/50/6/888

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

"CJDs (all human TSEs) and Endoscopy Equipment"

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

regarding your article;

Creutzfeldt-Jakob disease: implications for gastroenterology

i belong to several support groups for victims and relatives of CJDs. several years ago i did a survey regarding endoscopy equipment and how many victims of CJDs have had any type of this procedure done. to my surprise, many victims had some kind of endoscopy work done on them. as this may not be a smoking gun, i think it should warrant a 'red flag' of sorts, especially since data now suggests a substantial TSE infectivity in the gut wall of species infected with TSEs. If such transmissions occur, the ramifications of spreading TSEs from endoscopy equipment to the general public would be horrible, and could potential amplify the transmission of TSEs through other surgical procedures in that persons life, due to long incubation and sub-clinical infection. Science to date, has well established transmission of sporadic CJDs with medical/surgical procedures.

Terry S. Singeltary Sr. CJD WATCH

Subject: Re: gutjnl_el;21 Terry S. Singeltary Sr. (3 Jun 2002) "CJDs (all human TSEs) and Endoscopy Equipment" 

Date: Thu, 20 Jun 2002 16:19:51 -0700 

From: "Terry S. Singeltary Sr." 

To: Professor Michael Farthing 

CC: lcamp@BMJgroup.com References: <001501c21099$5c8bc620$7c58d182@mfacdean1.cent.gla.ac.uk>

Greetings again Professor Farthing and BMJ,

I was curious why my small rebuttal of the article described below was not listed in this month's journal of GUT? I had thought it was going to be published, but I do not have full text access. Will it be published in the future? Regardless, I thought would pass on a more lengthy rebuttal of mine on this topic, vCJD vs sCJDs and endoscopy equipment. I don't expect it to be published, but thought you might find it interesting, i hope you don't mind and hope to hear back from someone on the questions I posed...

Here is my short submission I speak of, lengthy one to follow below that:

Date submitted: 3 Jun 2002

snip...see full text;

Friday, September 27, 2019

Prion disease and recommended procedures for flexible endoscope reprocessing – a review of policies worldwide and proposal for a simplified approach Singeltary, GUT journal and Bramble et al

https://prionprp.blogspot.com/2019/09/prion-disease-and-recommended.html

Saturday, January 16, 2010

*** Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al

Evidence For CJD/TSE Transmission Via Endoscopes

From Terry S. Singletary, Sr flounder@wt.net 1-24-3

Terry S. Singeltary Sr., P.O. , Bacliff, Texas 77518 USA

http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html

Professor Michael Farthing wrote:

*** Louise Send this to Bramble (author) for a comment before we post. Michael

http://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html

http://vcjd.blogspot.com/2014/07/

Review Article Dent Implants Dentures, Vol 6(1) DOI: 10.4173/did.1000174

Prions-Dental Implications

Dr. Mehnu Zain1*, Dr. Prasanth Dhanpal2, Dr. Mohammed Sagir3, Dr. Biju Babu4 and Dr. Kennet J Chirayath5

1Senior Lecturer, Department of Dentistry, Royal dental college, Kerala 679536, India 2Prof. Department of Dentistry, Royal dental college, Kerala 679536, India 3Prof. and Head, Department of Dentistry, Royal dental college, Kerala 679536, India 4Prof. Department of Dentistry, Royal dental college, Kerala 679536, India 5Prof. Department of Dentistry, Royal dental college, Kerala 679536, India

*Corresponding Author: Dr. Mehnu Zain, Senior Lecturer, Department of Dentistry, Royal dental college, Kerala 679536, India, Email: mehnuzain17@gmail.com

Received: 03-Jan-2023 / Manuscript No. did-22-83913 / Editor assigned: 06-Jan-2023 / PreQC No. did-22-83913(PQ) / Reviewed: 20-Jan-2023 / QC No. did-22-83913 / Revised: 23-Jan-2023 / Manuscript No. did-22-83913(R) / Published Date: 30-Jan-2023 DOI: 10.4173/did.1000174

Abstract

Prions are infectious proteinaceous particle that causes fatal neurodegenerative disease. Prions have recently emerged as challenge to health care workers. Resistance to routine sterilization technique makes this infective protein particle unique and fearsome. Although transmission of prions through dental operative procedures is scarce, its risk cannot be avoided. This article reviews existing knowledge on etiology, pathogenesis, clinical features and dental implication on prion infected disease.

Keywords Prions; Infective protein particles; CJD; Infectious disease; vCJD

Introduction

Prions are infective proteins lacking a well-defined genetic constitution (DNA/RNA). The word prion was first coined by Stanley Prusiner in 1982 to describe an infectious agent that causes transmissible spongiform encephalopathy. The term prion was derived from the phrase ‘Proteinaceous infectious particle’ [1]. Initially they were believed to cause fatal zoonotic infections, but later on prions were isolated from brain samples of humans affected by Alzheimer’s disease (AD), Huntington’s disease (HD), and Parkinson’s disease (PD) [2]. Hence prions affect both animals and humans. Prion infected diseases have unique characteristics; they are highly heterogeneous and have varied phenotype [3]. Diagnostic procedures are not sensitive, no therapeutic intervention has shown reliable result, and mode of transmission is not under stood. So prion associated disease is an enigma to the medical field.

History

It was Griffith in 1967 first proposed protein could be infectious, pathogenic and postulated their involvement in scrapie [4]. Scrapie was a zoonotic infection seen among sheep’s and goats in European farms which was first reported in the 18th century, were animals scraped of their coats suffering from pruritus, hence the name scrapie4 .Griffiths hypothesis was later proved by Prusiner and co-worker when infectious proteinaceous particles were isolated from infected hamster brain and called the particle prion.

Cellular Prion Protein

The exact physical nature of prion protein is a controversy. It is believed that prion proteins are of 2 types PrPc and PrPsc. PrPc is associated with cell surface protein, they are present in healthy human cell and are soluble, monomeric and protease sensitive [5]. They play role in oxidative stress reduction, signal transduction, apoptosis regulation, binding of copper ions, adhesion of extracellular matrix, formation and maintenance of synapse [6].

PrPc is usually soluble; however insoluble form of PrPc (IPrPc) has also been identified in the brains of normal healthy human as well as cultured neuronal cells [7]. This isoform IPrPc binds only to the misfolded PrPsc not to the PrPc and is resistant to proteinase K degradation [8]. PrPsc (protein associated with scrapie) is an isomer of PrPc found in infected brain as aggregated material and are associated with pathogenesis; the molecular phenomenon involved is transition of α-helix rich PrPc to β sheets of PrPsc. βsheet rich structure has better stability and can form aggregates that are capable of forming amyloid fibrils [9]. This protein aggregation is major molecular event accompanying the conversion of PrPc to PrPsc.

Pathogenisis

The true mode of transmission of prion disease is not yet proven. Pathogenesis can be explained in following steps

1) Peripheral Replication

2) Neuroinvasion

3) Neuron degeneration [10]

Peripheral Replication

Various strains of prions show varying cell tropism. Hence the site of peripheral replication may vary, but many strains have shown high titres in the lymphoid tissues like tonsils, Peyer’s patches, and spleen before neuroinvasion. Next appears in serous and mucous glands in the oral cavity. It is the heamopiotic cells responsible for the transport of prions from the site of injury to the lyphoreticular system [10].

Neuroinvasion

Mature and follicular dendritic cells located in payer’s patches and B lymphocytes are known to cause neuroinvasion of prions. The exact mechanism of neuroinvasion is not well clear and is beyond the scope of this review .However role of complement system and hyper innervation to the secondary lymphoid organ in neuroinvasion has been documented in few studies [10] [Table 1].

Table 1:Various Human Prion Diseases and clinical manifestation.

Oral Manifestation

Since these are neurodegenerative disorder the oral manifestation reported include- dysphagia, paraesthesia, orofacial dysasthesia, loss of taste more over infectivity to trigeminal ganglion has been documented [14].

Diagnosis

Detection of PrPsc may be advantageous in the diagnosis of prion disease as PrPsc is associated with pathogenesis. Detection of sarogate markers has been documented but these markers are less specific they might show high titre in other neurodegenerative disorder as well. PrPsc proteins are usually identified with Western Blot, ELISA, and Immune Precipitation [15]. Other tests used: EEG, Cranial magnetic resonance imaging, Cerebrospinal fluid test, Tonsilar biopsy, blood test to extract DNA and check for mutation.

Transmission

The risk of transmission of CJD through dental treatment is unclear. A very few cases of disease transmission through blood transfusion have been reported [16] but further studies need to be conducted on it. However, the risk of disease transmission through neuronal tissue following neurological surgery like Dura mater graft, corneal transplant cannot be excluded. Studies also state that long incubation period could be the reason that masks the iatrogenic transmission of the disease [17]. There is no evidence of saliva as an infective agent for prion disease, even though few animal studies have isolated pathogenic prion protein from serous and mucous glands no human studies have been documented [18].

Prions and Dental Implication

Although the occupational risk of transmission of CJD in dentistry is less, on endodontists perspective, pulp tissue which is highly innervated by the nerves the chances of prion protein transmission through endodontic files cannot be completely exempted. One of the most important feature of prions are they are highly resistant to autoclave and other methods of sterilisation and disinfection. Therefor proper infection control has to be followed. All treatment should begin with a detailed case history [19]. Any history of neurological surgery like Dura mater graft, corneal transplant coinciding with neurological symptoms should be subjected to further neurological evaluation prior to the treatment [20].

The CDC guidelines for infection control for treatment of patients with vCJD include

• The dental items that are difficult to clean such as endodontic files, broaches, carbide and diamond burs should be discarded, other dental instrument that are heat resistant should be thoroughly cleaned and steam autoclaved for 134ºC for 18 minutes

• Patients with confirmed disease should be scheduled for end of the day to permit more extensive disinfection cleaning and decontamination

• Activation of waterlines should be avoided as there are chances of retraction of prions from the oral fluid.

• Standalone suction unit with disposable reservoir and disposable bowl instead of spittoon should be used.

• All the dental equipment should be shielded with impermeable sheets [11]. Besides these measures given by CDC operator should use all the PPE including face shield, once used all the all the PPE along with disposable used instruments should be quarantined in a leak proof combustible clinical waste container and should be subjected for incineration as soon as possible [21]. In case of re-usable instruments, it should be kept moist as the resistance of prion tissue to get removed increases as it becomes dry [Table 2].

Table 2: WHO Guidelines.

Post Exposure Prophylaxis • Contamination of unbroken skin with internal body fluids or tissues

Wash with detergent and abundant qualities of warm water, rinse and dry. Exposure to 0.1N NaOH or 1:10dilution of bleach for 1 minute can be considered for maximum safety.

• Needle sticks or lacerations

Gently encourage bleeding. Wash with warm soup water, rinse, dry and cover with a water proof dressing. Further treatment like suturing should be appropriate to the type of injury. Report the injury according to normal procedures of your hospital or health care facility. Records should be kept for no less than 20 years.

• Splashes into eye or mouth

Irrigate with either saline (eye) or tap water (mouth). Report according to normal procedures for your hospital or health care facility [21].

Conclusion Till date no case of transmission of prion associated disease through dental treatment has been reported yet. Still it is our responsibility to raise the standards of sterilisation and disinfection protocol to ensure safe and secure dental practice. This review aims to provide an overview of prion, its structure, pathogenesis diagnosis treatment and its relevance in dentistry. Further research should come up for a proper understanding of its transmission diagnosis and treatment.

References 

Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 9(216):136-144. Indexed at, Google Scholar, Crossref

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Aguzzi A, Heikenwalder M, Miele G (2004) Progress and problems in the biology, diagnostics, and therapeutics of prion diseases. The Journal of clinical investigation 114(2):153-160. Indexed at, Google Scholar, Crossref

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Azarpazhooh A, Fillery ED (2008) Prion disease: The implications for dentistry. J Endod 34:1158-1166. Indexed at, Google Scholar, Crossref

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Walker JT (2013) Evaluation of ninhydrin for monitoring surgical instrument decontamination. Journal of Hospital Infection 84(2): 95-96. Indexed at, Google Scholar, Crossref

Kingsbury L, Canada H (2002) Classic Creutzfeldt-Jakob disease recommendations for the operating room. Canadian operating room nursing journal 20(4): 6-10. Google Scholar, Crossref

Hamilton J (2007) Prions: transmissible spongiform encephalopathies and dental transmission risk assessment. J Calif Dent Assoc 35: 30. Google Scholar, Crossref

World Health Organization (2000) WHO infection control guidelines for transmissible spongiform encephalopathies: report of a WHO consultation, Geneva, Switzerland, 23-26 March 1999. World Health Organization. Google Scholar

Citation: Zain M, Dhanpal P, Sagir M, Babu B, Chirayath KJ (2023) Prions-DentalImplications. Dent Implants Dentures 6: 170. DOI: 10.4173/did.1000174

Copyright: © 2023 Zain M, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


Transmissible Spongiform Encephalopathy Unforeseen Circumstances, Friendly Fire, Pass it Forward 

Friday, November 11,2016

Human prion diseases:surgical lessons learned from iatrogenic prion transmission


Saturday, November 12, 2016

 Maine Medical Center received confirmation patient treated at the hospital has Creutzfeldt-Jakob disease 


Wednesday, November 09,2016

Maine Medical Centerpostpones elective surgeries over suspected case of prion disease


-----Original Message----- 

From: Terry Singeltary <flounder9@verizon.net> 

To: flounder9 <flounder9@verizon.net> 

Sent: Wed, Nov 13, 2019 9:52 am 

Subject: Moncton Hospital A third person who had cataract surgery has been diagnosed with the fatal Creuztfeld-Jakob disease

Woman is 3rd person this year diagnosed with Creutzfeldt-Jakob disease at the Moncton Hospital

Shepody woman is 3rd person diagnosed with fatal disease at the hospital this year

Tori Weldon · CBC News · Posted: Nov 12, 2019 5:00 AM AT | Last Updated: 7 hours ago

Diana Paterson first showed signs of Creutzfeldt-Jakob disease in April. Since her diagnosis in June, her husband Art Paterson has become her full-time caregiver. (Tori Weldon/CBC)

Diana Paterson is not sure how much longer she has to live.

In June, she was diagnosed with Creutzfeldt-Jakob disease, known as CJD, after showing signs of the fatal illness for several months.

Art Paterson, speaking with his wife's permission, said Diana now suffers symptoms of dementia, which came on quickly.

"It's like all of a sudden you walked into a nursing home and you don't know the person you're with," Art said.

When the couple went to Toronto to see a baseball game in April, it was the first time he noticed something was off.

Art and Diana Paterson have been married for 29 years. Only a few months after her diagnosis of CJD, Diana uses a walker and can't be left alone. (Pierre Fournier/CBC) Diana became agitated and couldn't sit still, and they ended up leaving part way through the game, despite Diana's love of the Blue Jays.

Thirteen falls, a six-week hospital stay and numerous tests later, the couple received the diagnosis. Art can't pronounce Creutzfeldt-Jakob, but he knows its prognosis well.

"It's downhill and you're never going back up," he said.

In the seven months since she first started showing symptoms, Diana has lost her short-term memory and can no longer walk unassisted.

"I do everything for her that I can, get her mad, make her laugh, do all the laundry, cook all our meals, whatever," Art said. "That's what husbands and wives are about."

Information Morning - Moncton Another Creuztfeld-Jakob case diagnosed at Moncton Hospital 25:19

A third person who had cataract surgery has been diagnosed with the fatal Creuztfeld-Jakob disease. 25:19 'What the heck is going on?'

While CJD is said to occur at a rate of only one in a million people, Diana is the third person to be diagnosed with the deadly degenerative brain disease at the Moncton Hospital in less than a year.

"You just kind of throw your hands up in the air and say, 'What the heck is going on?'" said Art.

Dr. Gordon Dow is the division head of infectious diseases at the Moncton Hospital. (Pierre Fournier/CBC) After his wife's diagnosis, Art went online looking for more information. He found two other CJD cases had made the news earlier in the year.

Like Diana, both underwent cataract surgery at the Moncton Hospital before their diagnoses. She had her surgery in December 2016. The other two patients had their surgeries in December 2018 and January 2019.

2 cases of Creutzfeldt-Jakob disease at Moncton Hospital not cause for concern, officials say Frustration mounts over Horizon's response to deadly disease But unlike Diana, both were already showing signs of the disease at the time of surgery.

Dr. Gordon Dow, division head of infectious diseases at the Moncton Hospital, said even he was shocked when another case of CJD was discovered.

'Flabbergasted'

"I was flabbergasted initially when I saw that we had three cases of this rare disease in a small city the size of Moncton," Dow said.

He immediately contacted the Public Health Agency of Canada, which runs a national surveillance system for the disease. He said the agency scrutinized each case, and he believes there is no need for people to panic.

Three years before showing signs of CJD, Diana Paterson went on a trip to Italy with her husband.(Submitted/Art Paterson) "I think even though there's the overwhelming weight of evidence suggesting that we've had a cluster of sporadic cases, there is no need for public alarm," Dow said.

"This is not an indication that there's been an outbreak of CJD."

Dow said he found several factors that could explain why Moncton is seeing an increase in CJD diagnoses.

Testing has improved

"Up until now, the only definitive diagnosis was [done through an] autopsy or brain biopsy," said Dow.

He said within the last two years a new method of testing for CJD has been making it much easier to diagnose living patients.

"It has literally revolutionized our ability to diagnose this disease, which is probably more common than we realized," said Dow.

He points to national numbers that show an increase in CJD cases in the last 21 years in Canada, with the mortality rate rising steadily from 0.79 in 1998 to 2.09 in 2018.

As well, Dow said, the city has the largest cohort of neurologists in the province at its two hospitals. With neurologists and geriatricians best equipped to diagnose the disease, a diagnosis that might be missed elsewhere would get picked up in Moncton, he said.

With all three CJD patients having had cataract surgery at Moncton Hospital, Dow said the first concern he had was whether there was any chance that could have caused CJD. He turned to Public Health for information and looked at studies done around the world. He found there is no evidence of a link.

"There's no scientific evidence that cataract surgery can cause CJD," he said.

CJD is most often found in older people, and the elderly receive most cataract surgeries, Dow said. A symptom of CJD is blurred vision and blindness, issues that lead to people receiving cataract surgery.

Types of CJD

According to Dow there are four kinds of CJD:

Iatrogenic (through hospital or medical procedures). Familial (through hereditary link). Sporadic (spontaneously occurring for no apparent reason). Variant CJD (BSE or "mad cow" disease). He said the two earlier cases of CJD were determined to be sporadic, and for confidentiality reasons he would not discuss Paterson's results.

Dow said he doesn't see a reason for the public to be concerned but, "if we keep seeing further rates of CJD rising in the Moncton area we're going to treat every case as a unique and important case," he said.

"There's going to be a lot of scrutiny."

CBC's Journalistic Standards and Practices|About CBC News Report Typo or Error|Send Feedback


See history;


Statement on low risk of transmitting CJD through cataract surgery

(Moncton) April 8, 2019 - We would like to confirm Horizon's The Moncton Hospital has identified two separate cases where a patient with probable Creutzfeldt-Jakob disease (CJD) had cataract surgery in our facility. After careful review it was determined that these two cases are totally unrelated. CJD is a rare degenerative brain disorder that leads to dementia.

Patients who received the same procedure in the following weeks have no significant risk of contracting the protein from the same medical instruments being used.

Horizon is confident the risk of transmitting CJD from using the same instruments is not significant. Horizon uses modern cleaning and sterilization processes that make the transmission nearly impossible.

Horizon notified 103 patients of the risk by letter upon discovering the first case of CJD on January 15, 2019. We notified an additional 601 patients on February 14,2019 following the discovery of the second probable case.

Patients were encouraged to call Horizon to speak with either their ophthalmologist or a member of Horizon's team. We can confirm that 43 patients contacted Horizon to learn more about their potential risk.

The transmission of CJD by surgical instruments has only been documented on seven occasions worldwide, occurring more than 20-40 years ago, and none of the CJD cases have been linked to cataract surgery.

Even though the risk of one of our patients contracting CJD is extremely low, Horizon is committed to being transparent and wanted to share this information with patients that received cataract surgery at Horizon's The Moncton Hospital.

We also shared this information with the family physicians for each patient.

Due to the rarity of having two separate CJD cases identified, we have disclosed this information to the Public Health Agency of Canada.


April 8, 2019 7:10 pm Updated: April 9, 2019 8:57 am

N.B. health authority contacts over 700 patients after detecting rare degenerative brain disease

By Alexander Quon

File - The Horizon Health Network has notified more than 700 patients after two cases of Creutzfeldt-Jakob disease were diagnosed. Both patients had undergone cataracts surgery before being diagnosed.

File - The Horizon Health Network has notified more than 700 patients after two cases of Creutzfeldt-Jakob disease were diagnosed. Both patients had undergone cataracts surgery before being diagnosed.

New Brunswick’s Horizon Health Network has identified two separate cases in which a patient with a probable case of a degenerative brain disease had cataract surgery at the Moncton Hospital — spurring them to contact 700 patients who underwent similar procedures at the facility.

The health authority confirmed to Global News on Monday evening that two cases of Creutzfeldt-Jakob disease (CJD) were detected at the facility but are unrelated.

Horizon directed Global News to a video that they published on their YouTube channel earlier on Monday about CJD when asked for information on incidents. The video is narrated by Dr. Gordon Dow, the chief of infectious diseases at Moncton Hospital.

READ MORE: Young Edmonton mother with rare form of dementia passes away

In the video, Dow says that the first case of CJD was diagnosed after a patient was admitted to hospital in December. Six weeks later another man was diagnosed with the same degenerative brain disease.

Both of the patients had undergone surgery before being diagnosed with CJD.

“We could not find any reason for this but statistical probability. It just so happens that two rare events happened at once,” said Dow.

Dow stresses in the video that the risk of transmission of CJD is “very low.”

What is CJD

According to the Alzheimer Society of Canada, CJD is a rare and fatal brain disease that is caused by a protein in the brain called prion.

In its natural form, prion is harmless. But when it is abnormal it becomes toxic to brain cells.

The disease is difficult to diagnose and it can be several years before a person who is exposed to CJD has the abnormal prions form.

The society says CJD can affect everyone differently but that the disease progresses quickly once symptoms appear. People with CJD rarely live beyond a year.

CJD can be “accidentally transmitted during a medical procedure involving human tissues” but can also be transmitted from exposure to a cattle infected with a variant of CJD known as mad cow disease.

CJD can also happen sporadically, often in elderly people without warning, or be the result of a genetic mutation.

READ MORE: What is mad cow disease? Quick facts about BSE

Informing patients

Dow stresses in the video that the risk of transmission of CJD is “very low” during cataract surgeries because the protein that results in the disease is not significantly present in the area of the where the surgery is carried out.

The chief of infectious diseases also says that modern sterilization methods used on surgical tools reduce the likelihood of transmission.

In the video — which, as of 7:30 p.m. Monday, has not been shared on Horizon’s official social media accounts — Dow says that transparency is important in medicine, even when there is low to no risk.

As a result, Dow says after detecting the first case, the cataract surgeon notified “each and every one” of 103 patients by phone who had “potentially been exposed” to the medical instruments used during the surgery.

WATCH: Families continue to come forward after Moncton nurse fired for administering labour-inducing drugs

The patients also received a letter informing them of the low risk of transmission.

The second incident prompted Horizon to notify 601 patients who had been potentially exposed. Dow says they couldn’t call all of the patients but they were informed through a letter.

A note has also been placed on all of the patient’s charts in order to flag that they warrant extra attention.

“The risk looks like zero and if its not zero it’s too low to measure,” Dow said in the video.

“So wouldn’t it be appropriate to be vigilant with the patients who had been exposed to the instruments.”

Dow says that the incident has made the hospital stress the importance of keeping track of tools used during surgeries.

Any patient who has questions or concerns are urged to contact 1-844-225-0220

If you’ve received a letter as a result of the incident and are interested in talking to Global News please email us at newbrunswick@globalnews.ca.


TUESDAY, APRIL 09, 2019

Horizon Health Network Moncton Hospital notified more than 700 patients after two cases of CJD were diagnosed both patients had undergone cataracts surgery before being diagnosed


O17 Evaluation of iatrogenic risk of CJD transmission associated with corneal transplantation

Douet JY. (1), Cassard H. (1), Huor A. (1), Lacroux C. (1), Haïk S. (2), Lugan S. (1), Tillier C. (1), Aron N. (1), Ironside J.W. (3), Andreoletti O. (1)

(1) UMR INRA-ENVT 1225, Ecole Nationale Vétérinaire de Toulouse, France.(2) Université Pierre et Marie Curie, UMR-S 1127, CNRS UMR 722, Institut du Cerveau et de la Moelle Epinière, G.H. PitiéSalpêtrière, Paris, France.(3) National CJD Research and Surveillance Unit Centre for Clinical Brain Sciences, University of Edinburgh, UK.

Sporadic Creutzfeldt–Jakob disease (sCJD) has been documented to be accidentally transmitted by contaminated corneal transplants. To date, only one case is considered as definite, while 5 other suspect cases are classified as probable or possible. However, the specific transmission risk associated with this widely-performed transplantation procedure has never been studied.

In this study, bioassays in transgenic mice expressing the human PrP confirmed the presence of infectivity in the cornea of 2 sCJD patients. Infectivity was also detected in other ocular tissues (optic nerve, retina, vitreous body, choroid and lacrymal gland) from one of these patients.

Based on these results, we investigated the presence of infectivity in the cornea of different TSE animal models. In conventional mice (RML strain) as well as in sheep (PG127 scrapie), infectivity could only be detected in the corneas collected at the late stage of the disease incubation phase.

In parallel to these experiments, corneas collected at different stages of the incubation period in infected mice and sheep were grafted into healthy recipients.

Our results showed that corneas collected during the late asymptomatic phase or in affected animals were able to transmit TSE infectivity. Importantly, after the death of the recipients (up to 2.5 years after surgery) infectivity could still be detected in the grafted cornea.

These data confirm the potential for sCJD transmission by corneal grafts. They also provide crucial data for the assessing the TSE transmission risk associated with various other ophthalmologic procedures.

PRION2018CONFERENCE ABSTRACT



JUST OUT CDC;

Tuesday, November 20, 2018

Eyes of CJD patients show evidence of prions Finding could help early diagnosis, raise concern for eye exams and transplants.


Singeltary 1999

***> THE EYES HAVE IT, CJD, AND THEY COULD BE STEALING THEM FROM YOUR LOVED ONE!...year 1999

i said that 20 years ago about this very thing. but did anyone listen...no!

prepare for the storm...terry

year 1999 to 2000

Subject: RE-The Eyes Have It (cjd) and they could be stealing them from your loved one... "pay back time"

Date: Sat, 16 Sep 2000 10:04:26 -0700

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

To: BSE-L@uni-karlsruhe.de

######### Bovine Spongiform Encephalopathy #########

Greetings List Members,

I hate to keep kicking a madcow, but this still is very disturbing to me. Not only for the recipient of the cornea's, but as well, for the people whom would be operated on, using the same tools that were used to put those stolen cornea's in the recipient with. No history of this donor or his family (re-ffi), or anything would be known, using stolen organs and or tissue's. I just think this is not only wrong, but very dangerous to a great many other people, as this is one of the most infectious tissues of TSE's. It seems that this practice of stealing organ/tissue happens more than we think. Anyway, the family of the victim which had their cornea's stolen, are now suing. In the example I used with my Mother, if 3 months before, she would have been in a catastrophic accident (car wreck, whatever), no autopsy (for whatever reason), no family (for whatever reason), she lay in the morgue, and after 4 hours, they come steal the cornea's, lot of people could have been infected, just because of lack of medical history of donor/family. It may be hypothetical, but very real. We need to stop the spread of this disease.

kind regards, Terry S. Singeltary Sr., Bacliff, Texas USA

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

Previous story--

Cadaver corneal transplants -- without family permission...

Cadaver corneal transplants -- without family permission Houston, Texas channel 11 news 28 Nov 99

Reported by Terry S. Singeltary Sr.son of CJD victim

"It was a story about how the Lions eye bank were harvesting corneas from victims in the Morgue, without their consent. Under Texas law, this appears to be legal (remember Texas has the Veggie liable law). Even if Family says no, this appears to happen, from what the news story said.

They said the only way to prevent this, is to fill out a form, stating not to have this done. So if you don't fill out the form, they can do this. How many people don't know about the form?

This is not only disgusting and appalling, it could be highly infectious. Without proper background checking of the donors, on their physical history, checking on past dementia, and/or family history, some of these unfortunate victims, could be passing a human TSE.

Response Jill Spitler Clevelland Eye Bank:

"No, we are not stealing.........Yes, you do have such a law in the state of Texas, but not all your state Eye Banks utilize the law. The Eye Bank that you're speaking of is only one of 43 certified Eye Bank throughout the USA.

And there are measure taken per the Medical Standards of the Eye Bank Association of America, the certifying body for eye banks and per FDA regulations to address those concerns that you speak of.

I would suggest that those interested/concern with transplant contact their local agencies. The Eye Bank Association of America has a web. site . Further if anyone has problems contacting or finding out about their local organization(s), call me or e-mail me I would be glad to help. My e-mail address is jill@clevelandeyebank.org"

Terry Singeltary responds:

"Explain this to the family in Houston who went to their loved ones funeral, only to find out that the loved one that was in the casket, had their corneas removed without their permission, without the consent of the victim or it's family. They would not have known it, only for the funny look the victim had. So, they questioned, only to find out, the corneas, had in fact, been removed without consent.

I call that stealing, regardless what the law states. This type of legal grave robbing is not a logical thing to do without knowing any type of background of the victims medical past, which really will not prove anything due to the incubation period. Eye tissue being potentially a highly infective source, there are risks here.

Should they not at least know of the potential ramifications of TSE's (the person receiving the corneas)?

Should there not be some sort of screening?

Should there be some sort of moral issue here?

If this is the case, and in fact, they can come take your corneas, without your consent, then what will they start taking next, without your consent?

Lets look at a hypothetical situation:

What would happen if my Mom (DOD 12-14-97 hvCJD) would have gotten into a car wreck and died, before the symptoms of CJD appeared. Not much money, so there was no autopsy. What would have happened to that recipient of those infecting corneas?"

Comment (webmaster): Actual transmission of CJD by means of corneal transplant may or may not be rare. The incidence of infectivity in older people could be fairly high; this is not to be confused with the lower incidence of symptomatic (clinical) CJD. It is very unlikely that familial CJD would have been diagnosed in earlier generations; however, without interviewing the family even known kindreds would not be excluded.

In blood donation, a much stricter policy is followed, even though corneal transplant may be far more dangerous (being a direct link to the brain and not going through purification steps).

Since highly sensitive tests for pre-clinical CJD are now available, it would make sense to screen corneas for CJD, just as they are screened for AIDS, hepatitus, and a host of other conditions.

http://www.mad-cow.org/dec99_news.html#bbb

archived url;


http://www.braintalkcommunities.org/archives/06_11/showthread.php?t=76877

archived url;


Eye procedure raises CJD concerns

2004

BySTEVE MITCHELL, Medical Correspondent

WASHINGTON, Nov. 18 (UPI) -- A New York man who died from a rare brain disorder similar to mad cow disease in May underwent an eye procedure prior to his death that raises concerns about the possibility of transmitting the fatal disease to others, United Press International has learned.

The development comes on the heels of the announcement Thursday by U.S. Department of Agriculture officials of a possible second case of mad cow disease in U.S. herds.

Richard Da Silva, 58, of Orange County, N.Y., died from Creutzfeldt Jakob disease, an incurable brain-wasting illness that strikes about one person per million.

Richard's wife Ann Marie Da Silva told UPI he underwent a check for the eye disease glaucoma in 2003, approximately a year before his death. The procedure involves the use of a tonometer, which contacts the cornea -- an eye tissue that can contain prions, the infectious agent thought to cause CJD.

Ann Marie's concern is that others who had the tonometer used on them could have gotten infected.

A 2003 study by British researchers suggests her concerns may be justified. A team led by J.W. Ironside from the National Creutzfeldt-Jakob Disease Surveillance Unit at the University of Edinburgh examined tonometer heads and found they can retain cornea tissue that could infect other people -- even after cleaning and decontaminating the instrument.

"Retained corneal epithelial cells, following the standard decontamination routine of tonometer prisms, may represent potential prion infectivity," the researchers wrote in the British Journal of Ophthalmology last year. "Once the infectious agent is on the cornea, it could theoretically infect the brain."

Prions, misfolded proteins thought to be the cause of mad cow, CJD and similar diseases, are notoriously difficult to destroy and are capable of withstanding most sterilization procedures.

Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University, agreed with the British researchers that tonometers represent a potential risk of passing CJD to other people.

Manuelidis told UPI she has been voicing her concern about the risks of corneas since 1977 when her own study, published in the New England Journal of Medicine, showed the eye tissue, if infected, could transmit CJD.

At the time the procedure was done on Richard Da Silva, about a year before he died, she said it was "absolutely" possible he was infectious.

The CJD Incidents Panel, a body of experts set up by the U.K. Department of Health, noted in a 2001 report that procedures involving the cornea are considered medium risk for transmitting CJD. The first two patients who have a contaminated eye instrument used on them have the highest risk of contracting the disease, the panel said.

In 1999, the U.K. Department of Health banned opticians from reusing equipment that came in contact with patients' eyes out of concern it could result in the transmission of variant CJD, the form of the disease humans can contract from consuming infected beef products.

Richard Da Silva was associated with a cluster of five other cases of CJD in southern New York that raised concerns about vCJD.

None of the cases have been determined to stem from mad cow disease, but concerns about the cattle illness in the United States could increase in light of the USDA announcement Thursday that a cow tested positive on initial tests for the disease. If confirmed, this would be the second U.S. case of the illness; the first was detected in a Washington cow last December. The USDA said the suspect animal disclosed Thursday did not enter the food chain. The USDA did not release further details about the cow, but said results from further lab tests to confirm the initial tests were expected within seven days.

Ann Marie Da Silva said she informed the New York Health Department and later the eye doctor who performed the procedure about her husband's illness and her concerns about the risk of transmitting CJD via the tonometer.

The optometrist -- whom she declined to name because she did not want to jeopardize his career -- "didn't even know what this disease was," she said.

"He said the health department never called him and I called them (the health department) back and they didn't seem concerned about it," she added. "I just kept getting angrier and angrier when I felt I was being dismissed."

She said the state health department "seems to have an attitude of don't ask, don't tell" about CJD.

"There's a stigma attached to it," she said. "Is it because they're so afraid the public will panic? I don't know, but I don't think that the answer is to push things under the rug."

New York State Department of Health spokeswoman Claire Pospisil told UPI she would look into whether the agency was concerned about the possibility of transmitting CJD via tonometers, but she had not called back prior to story publication.

Disposable tonometers are readily available and could avoid the risk of transmitting the disease, Ironside and colleagues noted in their study. Ann Marie Da Silva said she asked the optometrist whether he used disposable tonometers and "he said 'No, it's a reusable one.'"

Ironside's team also noted other ophthalmic instruments come into contact with the cornea and could represent a source of infection as they are either difficult to decontaminate or cannot withstand the harsh procedures necessary to inactivate prions. These include corneal burrs, diagnostic and therapeutic contact lenses and other coated lenses.

Terry Singletary, whose mother died from a type of CJD called Heidenhain Variant, told UPI health officials were not doing enough to prevent people from being infected by contaminated medical equipment.

"They've got to start taking this disease seriously and they simply aren't doing it," said Singletary, who is a member of CJD Watch and CJD Voice -- advocacy groups for CJD patients and their families.

U.S. Centers for Disease Control and Prevention spokeswoman Christine Pearson did not return a phone call from UPI seeking comment. The agency's Web site states the eye is one of three tissues, along with the brain and spinal cord, that are considered to have "high infectivity."

The Web site said more than 250 people worldwide have contracted CJD through contaminated surgical instruments and tissue transplants. This includes as many as four who were infected by corneal grafts. The agency noted no such cases have been reported since 1976, when sterilization procedures were instituted in healthcare facilities.

Ironside and colleagues noted in their study, however, many disinfection procedures used on optical instruments, such as tonometers, fail. They wrote their finding of cornea tissue on tonometers indicates that "no current cleaning and disinfection strategy is fully effective."

Singletary said CDC's assertion that no CJD cases from infected equipment or tissues have been detected since 1976 is misleading.

"They have absolutely no idea" whether any cases have occurred in this manner, he said, because CJD cases often aren't investigated and the agency has not required physicians nationwide report all cases of CJD.

"There's no national surveillance unit for CJD in the United States; people are dying who aren't autopsied, the CDC has no way of knowing" whether people have been infected via infected equipment or tissues, he said.

Ann Marie Da Silva said she has contacted several members of her state's congressional delegation about her concerns, including Rep. Sue Kelly, R-N.Y., and Sen. Charles Schumer, D-N.Y.

"Basically, what I want is to be a positive force in this, but I also want more of a dialogue going on with the public and the health department," she said.


***> CWD, Scrapie, BSE, TSE PrP Environmental Factors <***

Chronic wasting disease (CWD) prion detection in environmental and biological samples from a taxidermy site and nursing facility, and instruments used in surveillance activities

Available online 9 April 2025

Highlights

• CWD prions were identified in a taxidermy and deer nursing facility.

• Contaminated samples included waters, soils, dermestid beetles, domestic flies and a dumpster.

• Surgical instruments used to collect deer samples can get contaminated with CWD prions.

• Some of the infectious particles are readily released from surgical instruments when washed.

• Our results suggest that taxidermy practices actively contribute in the spreading of CWD.

Snip…

In summary, the information provided in this report demonstrate how anthropogenic activities, specifically taxidermy practices, animal processing, and rehabilitation of CWD susceptible species, may facilitate CWD transmission through the environmental dissemination of CWD prions. This study, along with future research efforts characterizing the overall level of infectivity, provides relevant information on managing CWD and to control its rapid geographic expansion. …

https://www.sciencedirect.com/science/article/abs/pii/S0048969725009544

Chronic wasting disease detection in environmental and biological samples from a taxidermy site

Results: The PMCA analysis demonstrated CWD seeding activity in some of the components of this facility, including insects involved in head processing, soils, and a trash dumpster.

Conclusions: Different areas of this property were used for various taxidermy procedures. We were able to detect the presence of prions in

i) soils that were in contact with the heads of dead animals, ii) insects involved in the cleaning of skulls, and iii) an empty dumpster where animal carcasses were previously placed.

This is the first report demonstrating that swabbing is a helpful method to screen for prion infectivity on surfaces potentially contaminated with CWD. These findings are relevant as this swabbing and amplification strategy may be used to evaluate the disease status of other free-ranging and captive settings where there is a concern for CWD transmissions, such as at feeders and water troughs with CWD-exposed properties. This approach could have substantial implications for free-ranging cervid surveillance as well as in epidemiological investigations of CWD.

Prion 2022 Conference abstracts: pushing the boundaries

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

Artificial mineral sites that pre-date endemic chronic wasting disease become prion hotspots

The Ames Research and Educational Center property, centrally located within the CWD zone of southwest Tennessee, contains 49 historical mineral supplementation sites that were decommissioned in 2012. Here, we demonstrate that 32 of the 49 (65%) mineral sites within Ames established prior to the regional CWD outbreak, serve as foci of environmental PrPCWD contamination. Detection of PrPCWD in soils from these artificial mineral sites was dependent on site-specific management efforts. Soil physical properties were very similar across sites and no correlation between PrPCWD detection and soil physical properties was found. The detection of PrPCWD in soils at attractant sites within an endemic CWD zone significantly advances our understanding of environmental PrPCWD accumulation dynamics, providing valuable information for advancing adaptive CWD management approaches.

https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf

Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer

Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.

Prion 2022 Conference abstracts: pushing the boundaries

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

"Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation."

15 YEARS!

Detection of prions in soils contaminated by multiple routes

Results: We are able to detect prion seeding activity at multiple types of environmental hotspots, including carcass sites, contaminated captive facilities, and scrapes (i.e. urine and saliva). Differences in relative prion concentration vary depending on the nature and source of the contamination. Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation.

Conclusions: Detection of prions in the environment is of the utmost importance for controlling chronic wasting disease spread. Here, we have demonstrated a viable method for detection of prions in complex environmental matrices. However, it is quite likely that this method underestimates the total infectious prion load in a contaminated sample, due to incomplete recovery of infectious prions. Further refinements are necessary for accurate quantification of prions in such samples, and to account for the intrinsic heterogeneities found in the broader environment.

Funded by: Wisconsin Department of Natural Resources

Meeting-book-final-version prion 2023 Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years

***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded.

JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12

Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free

https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0

Rapid recontamination of a farm building occurs after attempted prion removal

First published: 19 January 2019 https://doi.org/10.1136/vr.105054

The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease. snip...

This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapie positive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.

https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054

***>This is very likely to have parallels with control efforts for CWD in cervids.

https://pubmed.ncbi.nlm.nih.gov/30602491/

I remember what “deep throat” told me about Scrapie back around 2001, during early days of my BSE investigation, after my Mom died from hvCJD, I never forgot, and it seems it’s come to pass;

***> Confidential!!!!

***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!

---end personal email---end...tss

and so it seems…

Chronic Wasting Disease CWD TSE Prion

THE CWD TSE Prion aka mad cow type disease is not your normal pathogen.

The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.

You cannot cook the TSE prion disease out of meat. In fact new data now shows that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification.

you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well.

the TSE prion agent also survives Simulated Wastewater Treatment Processes.

IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.

you can bury it and it will not go away.

The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.

it’s not your ordinary pathogen you can just cook it out and be done

New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

http://www.pnas.org/content/97/7/3418.full

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/

Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals

https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf

THURSDAY, FEBRUARY 28, 2019

BSE infectivity survives burial for five years with only limited spread

https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf

Chronic wasting disease prions on deer feeders and wildlife visitation to deer feeding areas

First published: 10 February 2025

Snip…

Finally, we swabbed 19 feeders in 2 areas where CWD was newly detected, finding prion contamination on swabs from 4 feeders. We show that deer feeders in free-ranging populations with high CWD prevalence become contaminated with CWD prions quickly, becoming a potential site of exposure of deer to CWD prions. Our results also demonstrate the ability to find evidence of prion contamination on deer feeders, even in areas where CWD is newly detected.

Snip…

We found that supplemental feeding increased the risk of exposure to CWD prions due to contamination of feeders, increased deer visitation, and increased deer-to-deer contact.

The 12-fold increase in deer visitation to feeders compared to mast trees and 2-fold increase compared to food plots demonstrates increased risk for direct disease spread.

https://wildlife.onlinelibrary.wiley.com/doi/10.1002/jwmg.70000

Chronic Wasting Disease in Texas A Real Disease with Proven Impacts

Produced by a coalition of concerned hunters, landowners, & conservationists (last update 1/2025)

storymaps.arcgis.com/stories/b93f528938ac48e9b56dcc79953cbec0

Aug 18, 2021

Oh, Deer

Heading Off a Wildlife Epidemic

CWD poses a significant threat to the future of hunting in Texas. Deer population declines of 45 and 50 percent have been documented in Colorado and Wyoming. A broad infection of Texas deer populations resulting in similar population impacts would inflict severe economic damage to rural communities and could negatively impact land markets. Specifically, those landowners seeking to establish a thriving herd of deer could avoid buying in areas with confirmed CWD infections. As they do with anthrax-susceptible properties, land brokers may find it advisable to inquire about the status of CWD infections on properties that they present for sale. Prospective buyers should also investigate the status of the wildlife on prospective properties. In addition, existing landowners should monitor developments as TPWD crafts management strategies to identify and contain this deadly disease.

Dr. Gilliland (c-gilliland@tamu.edu) is a research economist with the Texas Real Estate Research Center at Texas A&M University.

www.recenter.tamu.edu/articles/tierra-grande/oh-d

2025

***> Cwd, cattle, pigs, sheep, raccoons, oh my <***

Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry

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

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.

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

Prion Conference 2023

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

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

Strain characterization of chronic wasting disease in bovine-PrP transgenic mice

Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

***> CWD PIGS, OH MY! <***

WEDNESDAY, JANUARY 28, 2026

Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak

https://assets.publishing.service.gov.uk/media/697a3b013c71d838df6bd413/CWD_Prions_in_Cervids_and_Wild_Pigs_in_North_America.pdf

https://transmissiblespongiformencephalopathy.blogspot.com/2026/01/chronic-wasting-disease-prions-in.html

Volume 31, Number 1—January 2025

Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United States

Abstract

Using a prion amplification assay, we identified prions in tissues from wild pigs (Sus scrofa) living in areas of the United States with variable chronic wasting disease (CWD) epidemiology. Our findings indicate that scavenging swine could play a role in disseminating CWD and could therefore influence its epidemiology, geographic distribution, and interspecies spread.

Conclusions

In summary, results from this study showed that wild pigs are exposed to cervid prions, although the pigs seem to display some resistance to infection via natural exposure. Future studies should address the susceptibility of this invasive animal species to the multiple prion strains circulating in the environment. Nonetheless, identification of CWD prions in wild pig tissues indicated the potential for pigs to move prions across the landscape, which may, in turn, influence the epidemiology and geographic spread of CWD.

https://wwwnc.cdc.gov/eid/article/31/1/24-0401_article

Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.

The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans. Interestingly, bioassay of material from the longest surviving >6 month orally challenged pig (72 mpc), which was negative for PrPcwd by all other tests, produced a positive bioassay result. Bioassay of material from additional animals is currently underway. This study demonstrates that pigs can serve as potential hosts for CWD, although with low attack rates and scant PrPcwd accumulation. Detection of infectivity in orally challenged pigs using mouse bioassay raises the possibility that naturally exposed pigs act as a reservoir of CWD infectivity, even though affected pigs do not develop overt clinical signs or readily detectable PrPcwd.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166

cwd scrapie pigs oral routes

***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***

*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <***

***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%).

***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091

https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017

https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105

Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105

Component 6: Transmissible Spongiform Encephalopathies

Sheep scrapie agent can infect white-tailed deer after oronasal exposure.

The origin of chronic wasting disease (CWD) is not known, but it has many similarities to the sheep prion disease called scrapie. It has long been hypothesized that CWD arose through transmission of sheep scrapie to deer. ARS researchers in Ames, Iowa, conducted research to determine if scrapie derived from sheep could be transmitted to white-tailed deer. The deer inoculated with sheep scrapie developed clinical signs and the abnormal prion protein could be detected in a wide range of tissues. These results indicate that deer may be susceptible to sheep scrapie if exposed to the disease in natural or agricultural settings. In addition, several strong similarities between CWD in white-tailed deer and the experimental cases of scrapie in white-tailed deer suggests that it would be difficult to distinguish scrapie from CWD in deer or identify scrapie if a case occurs. This information should be considered by deer farmers for keeping their herds free from prion diseases.

https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/NP103%20FY2023%20Annual%20Report_Final.pdf

The chronic wasting disease agent from white-tailed deer is highly infectious to humanized mice after passage through raccoons

https://www.ars.usda.gov/research/publications/publication/?seqNo115=400777

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed

PUBLIC SUBMISSION

Comment from Terry Singeltary Sr.

Posted by the Food and Drug Administration on May 17, 2016 Comment

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

https://www.regulations.gov/comment/FDA-2003-D-0432-0011

https://www.regulations.gov/docket/FDA-2003-D-0432


USDA Statement BSE Surveillance Information Center Update 2026 

https://oieusdabseprp.blogspot.com/2026/04/usda-statement-bse-surveillance.html

Terry S. Singeltary Sr.

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