The surveillance programme for Chronic Wasting Disease (CWD) in free-ranging and captive cervids in Norway 2025 to 2016 Singeltary Review

The surveillance programme for Chronic Wasting Disease (CWD) in free-ranging and captive cervids in Norway 2025 to 2016 Singeltary Review

Norwegian Veterinary Institute 3/17

Summary

A total of 11,612 samples of wild, semi-domesticated and captive Norwegian cervids were analysed in 2025. Of these, three moose (Alces alces) tested positive for CWD. All were old females culled in Folldal (20 years old), Tvedestrand (17 years old) and Selbu (16 years old) municipalities. The later case was a traffic injury. The cases detected showed disease characteristics previously described for the species in previous reports, i.e. PrPSc was only detected in brain tissue, and no prions were detected in lymphoid tissues with diagnostic tests (ELISA and western blot). In the Nordic countries, CWD has shown sporadic appearance in moose and red deer, whilst in reindeer the disease has been contagious and with lymphoid detections in routine diagnostics.

Introduction

Chronic Wasting Disease (CWD) was for the first time detected in Europe in 2016, in Norway [1]. This was also the first detection of a natural CWD infection in reindeer (Rangifer tarandus) worldwide. The index case in reindeer was in Nordfjella management area in Southern Norway. All subsequent positive reindeer until 2020 were found in this area (Figure 1). The Nordfjella sub-population was eradicated in 2018 [2]. In 2020, one reindeer CWD case was discovered at Hardangervidda, which is the neighboring area/population to Nordfjella. Another case in reindeer was detected at Hardangervidda in 2022. CWD is a transmissible spongiform encephalopathy (TSE) or prion disease of cervids [3]. It is an invariably fatal neurodegenerative disease. Well-known in North America, CWD has since the 1960’s gradually spread to an increasing number of states and provinces (July 2025: 36 states in USA and five provinces in Canada), both in captive and free-ranging cervids [4]. The republic of Korea has also diagnosed the disease after importation of infected elk (Cervus elaphus nelsoni) from Canada [5]. Following the emergence of CWD in Norway, reindeer were identified as a naturally susceptible species. Norway has by now three affected species; reindeer, moose (Alces alces) and red deer (Cervus elaphus). CWD in the Nordic cervids has shown different epidemiological paterns according to species. In routine diagnostics (ELISA and western blot) reindeer have detectable prions both in brain and lymphoid tissues, whilst moose and red deer cases only in the brain. The tissue- and geographic distribution suggests a contagious character of the disease in reindeer. The disease in moose and red deer occurs sporadically, has only been found in older animals, and there is litle evidence for contagiousness and horizontal spread between live animals. Since 2018, old female moose has also been diagnosed with sporadic CWD in Finland and Sweden [6,7].

Four cervid species are prevalent in natural free-ranging populations in Norway: moose, red deer, roe deer (Capreolus capreolus) and reindeer. Red deer predominates along the west coast, whereas moose and roe deer mainly inhabit other areas of the country. The wild reindeer is found in fragmented sub-populations in the remote alpine regions of Southern Norway [8]. The official numbers (February 2026) of hunted cervids were in the 2025 season: 25,951 moose, 49,449 red deer, 27,173 roe deer and 3,851 wild reindeer [9] In addition, Norway has populations of semi-domesticated reindeer that live in a herded condition, though free ranging and migrating between summer- and winter-feeding areas. Most semi-domesticated reindeer are found in the Northern part of Norway as part of the Sami culture, particularly in the county Finnmark, and the total population counts about 250,000 animals [10]. There are approximately 100 deer farms in Norway; most of them keep red deer, but some farms have fallow deer (Dama dama) and occasionally both species.

Testing wild cervids for CWD was initiated in 2002 through the National Health Surveillance Program for Cervids and muskox (Ovibos moschatus), operated by the Norwegian Veterinary Institute (NVI) on behalf of the The surveillance programme for Chronic Wasting Disease (CWD) in free-ranging and captive cervids in Norway 2025 Norwegian Veterinary Institute 4/17 Norwegian Environmental Agency. From 2003, a passive surveillance programme for CWD has been in place in Norwegian wild and captive cervids. The programme is coordinated by NVI and financed by the Norwegian Food Safety Authority (NFSA). In addition, the programme includes testing samples from slaughtered semi-domesticated reindeer from several regions of the country. In 2006 and 2007 the European Commission (decision 2007/182/EC) initiated a survey for CWD where Norway took part, like the EU member-states, testng 700 cervids. A similar testing was performed in EU in the years 2018-2020 (reg. 2017/1972), covering six member states in addition to Norway. Shown in Table 1 is the total number of cervids tested for CWD in Norway from 2002-2025.

Since there is no separate program to report surveillance of TSE in Muskox, such testing is included in this publication. TSE has never been reported in the species.

Aim

The aim of the program is to document the occurrence of CWD in the Norwegian cervid populations.

Materials and methods…

snip…

Discussion

In 2016, CWD was for the first time detected in Europe, in six free-ranging cervids in Norway. In total, the CWD testing revealed 21 reindeer, 17 moose, and three red deer with the disease in the period 2016-2025. The first 19 reindeer cases were detected within the outbreak zone 1 of Nordfjella wild reindeer management area. The affected population was culled in 2018. Two additional cases have been diagnosed in reindeer hunted at Hardangervidda in September 2020 and September 2022. This last discovery of CWD in a second reindeer area challenges the prospect of eradicating the contagious disease in the reindeer populations. While the eradication of the Nordfjella sub-population was aimed to eliminate the local outbreak, subsequent findings have highlighted new epidemiological challenges. Hardangervidda holds the largest sub-population of wild reindeer in Europe. Compared with Nordfjella, the area is far less suitable for containment of the disease.

Nevertheless, so far, no additional reindeer cases have been detected outside Nordfjella and Hardangervidda despite the testing of more than 98,000 reindeer. In the absence of additional detected cases, probabilistic modelling has therefore been applied to assess the likelihood of disease freedom across different areas. This indicates a limited infection in this species. Nevertheless, the probability of freedom as estimated using scenario tree modelling varies a lot between different wild reindeer management areas or semi-domesticated reindeer districts depending on population size and number of tested animals per area or district. From the extended surveillance of cervids, there is a confidence that CWD is not present at high prevalence (5%) in cervids in Norway [16]. For a lower prevalence (below 1%), there is less confidence. Only a few wild reindeer management areas have obtained a 95% probability of freedom for a design prevalence of 0.5% [17]. The Norwegian Veterinary Institute provide central management authorities with modelling of freedom probability in areas within restriction zones of CWD. Under the modelling assumptions of a design prevalence of 4 (i.e. that the probability of absence of CWD is calculated as the probability of finding at least one CWD-infected animal if there are 4 CWD-infected animals in the population), Hardangervidda and Nordfjella management areas have obtained 41 % and 99 % respectively, post hunting season in 2025. The reindeer district of Filefjell, with around 3000 semi- domesticated reindeer during the winter (post slaughter) and sharing (a fenced) border at summer pasture to the Nordfjella area, has obtained a high probability of freedom for a design prevalence less than 0.5 % [18].

Analysis of surveillance data from Norwegian and Swedish semi-domesticated reindeer districts used a scenario tree model, and at the national level the mean probability of disease freedom was 87.0 % in Norway by 2021 [19].

Beyond prevalence estimates, insight into disease characteristics across species is further informed by prion strain characterisation. Further research studies have shown that the strain found in reindeer is different from those of the two other cervid species. The reindeer cases were indistinguishable, in results of diagnostic test methods, from cases of CWD from North America. The positive European moose and red deer have shown characteristics of sporadic distribution [6,7,14], with no detectable prions in the lymphoid tissues using diagnostic tests, in contrast with the reindeer and cervids from North America. Although routine diagnostics primarily define these species differences, complementary research methods (PMCA) showed low detectable levels of PrPSc also in peripheral tissues [20] from moose and red deer. Inoculation studies in rodents demonstrated multiple strains among the Norwegian cases, across three species, all different from North American cases. Nevertheless, the reindeer strain has many similarities with strains isolated from cases from North America [21,22] and is expected to harbor similar epidemiological properties.

https://www.vetinst.no/overvaking/chronic-wasting-disease-vilt-cwd/_/attachment/inline/701c0cc5-1708-484a-8b89-779661e7080e:0b97daa380fbd88031c6d2fe58726cacd3e00dcf/2026_17_OK_CWD%20in%20Norway%202025%20KOMPLETT.pdf

please note;

We call scrapie for «skrapesyke».

We call CWD for «skrantesyke».

«Skrante» have a meaning similar to «wasting».

Norway CWD 2025 Update

2025

A total of 11,612 samples of wild, semi-domesticated and captive Norwegian cervids were analyzed in 2025. Of these, three moose (Alces alces) tested positive for CWD. All were old females culled in Folldal (20 years old), Tvedestrand (17 years old) and Selbu (16 years old) municipalities. The later case was a traffic injury.

The cases detected showed disease characteristics previously described for the species in previous reports, ie PrPSc was only detected in brain tissue, and no prions were detected in lymphoid tissues with diagnostic tests (ELISA and western blot). In the Nordic countries, CWD has shown sporadic appearance in moose and red deer, whilst in reindeer the disease has been contagious and with lymphoid detections in routine diagnostics. Download the report

Chronic wasting disease 2025 (pdf 2mb)

https://www.vetinst.no/en/surveillance-programmes/chronic-wasting-disease

Download report

Chronic wasting disease 2025 (pdf 2mb) Previous reports

Chronic wasting disease 2024 (pdf 3mb) Chronic wasting disease 2023 (pdf 2mb) Chronic wasting disease 2022 (pdf 4mb) Chronic wasting disease 2021 (pdf 5mb) Chronic wasting disease 2020 (pdf 5mb) Chronic wasting disease 2019 (pdf 5mb) Chronic wasting disease 2018 (pdf 3mb) Chronic wasting disease 2017 (pdf 2mb) Chronic wasting disease 2016 (pdf 4mb) Chronic wasting disease 2015 (pdf 1mb) Chronic wasting disease 2014 (pdf 539kb) Chronic wasting disease 2013 (pdf 1mb) Chronic wasting disease 2012 (pdf 1mb) Chronic wasting disease 2011 (pdf 904kb) Chronic wasting disease 2010 (pdf 616kb) Chronic wasting disease 2009 (pdf 530kb) Chronic wasting disease 2008 (pdf 497kb) Chronic wasting disease 2007 (p. 103) (pdf 6mb) Chronic wasting disease 2006 (p. 109) (pdf 3mb) Chronic wasting disease 2005 (p. 105) (pdf 4mb)

https://www.vetinst.no/en/surveillance-programmes/chronic-wasting-disease

Norway cwd skrantesjuke

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Norway Changes to the TSE and CWD regulations, and repeal of regulations on the import of snails

Regulatory process

Changes to the TSE and CWD regulations, and repeal of regulations on the import of snails

Published 13/02/2023 Last changed 13/02/2023

The Norwegian Food Safety Authority implements a regulation which entails changes to the provisions on prion diseases (TSE regulations). The changes mean that the list of tissue types (SRM) that must be removed from sheep and goats to avoid infection with mad cow disease (BSE) is reduced to only include the skull and spinal cord.

The protective measures against scrapie disease (CWD) given in EEA regulations are moved from the CWD regulations to the general TSE regulations. The measures are continued and adjusted so that they also apply to other EEA countries with scrapie. They concern a ban on the export of live deer from countries with scrapie and a ban on natural odorants (lure urine) from deer.

At the same time, the CWD regulations are amended by repealing the EEA provisions on protective measures against CWD described above.

Finally, a regulation on the import of certain foodstuffs which is redundant is repealed, as the provisions are now found in other regulations.

The progress of the work is described in the timeline below, and the black box shows where we are in the process.

Timeline for this work:

Hearing

The Norwegian Food Safety Authority sends draft regulations on changes for consultation after it has been sent to the Ministry of Agriculture and Food for clearance before consultation.

The regulatory change will be determined by the Norwegian Food Safety Authority, but not before the legal acts have entered into force in the EEA agreement. There are limited opportunities to influence the content of the Norwegian implementation of EU legal acts. The hearing of such cases is therefore mainly carried out for informational reasons.

Hearing deadline 27/03/2023

Download case documentation

https://www.mattilsynet.no/om_mattilsynet/endringer_i_tse_og_cwdforskriften_og_oppheving_av_forskrift_om_import_av_snegler.49399

Norway Mapping and monitoring of chronic wasting disease (CWD) 2021

Kartlegging og overvåking av skrantesjuke (chronic wasting disease - CWD) 2021

Christer M. Rolandsen, Jørn Våge, Petter Hopp, Sylvie L. Benestad, Hildegunn Viljugrein, Erling J. Solberg, Erlend B. Nilsen, Roy Andersen, Olav Strand, Turid Vikøren, Knut Madslien, Attila Tarpai, Vebjørn Veiberg, Morten Heim, Frode Holmstrøm, Atle Mysterud

Abstract

Rolandsen, C.M., Våge, J., Hopp, P., Benestad, S.L., Viljugrein, H., Solberg, E.J., Nilsen, E.B., Andersen, R., Strand, O., Vikøren, T., Madslien, K., Tarpai, A., Fremstad, J., Veiberg, V., Heim, M., Holmstrøm, F., Mysterud, A. 2022. Surveillance of chronic wasting disease (CWD) in Norway 2021. NINA Report 2158 / Norwegian Veterinary Institute Report 22, 2022. 52 pp.

This report summarizes the results of the Norwegian surveillance program for chronic wasting disease (CWD) in 2021, and the total number of cervids tested in the period 2016-2021.

In 2021, 21,661 cervids were tested for CWD, fulfilling the authorities' goal of about 22,000. Three cases of atypical CWD were detected in 2021. Two moose, a 17 year old female from Vinje and a 13 year old male from Bamble. Furthermore, one case was detected in red deer, an adult female of unknown age. In total, classical CWD has been detected in 20 wild reindeer and atypical CWD has been detected in nine moose and two red deer, in the period 2016-2021.

Samples from both the brain and lymph nodes were analyzed from 73% of tested cervids. This is approximately similar to the previous year, with 72%, 78% and 80% in 2020, 2019 and 2018, respectively.

There are still some challenges with poor sample quality and with data registered for some sam- ples. Accordingly, 1% of the samples were not marked with municipality or area and 3% lacked information about species. For 21% of the samples from wild reindeer, we lack information on whether the sample came from hunting or from animals found dead for other reasons. Therefore, samples received during the hunting season were automatically registered as taken from hunted animals unless otherwise stated.

In 2021, the percentage of cervids found dead for other reasons than hunting and that were tested for CWD, was higher compared to 2020. For moose the percentage increased from 18% to 27%, and for red deer from 21% to 30%. There was also an increase for wild reindeer from 8% to 25%, but the number of registered wild reindeer found dead for other reasons than hunting is low. For roe deer, there was no change in this percentage. The goal of increasing the test percentage of cervids found dead for other reasons than hunting was therefore partially reached. However, we believe this percentage can still be increased significantly.

From the population model for Hardangervidda, we have estimated the population size of wild reindeer before hunting in 2022 to be around 6 863. Of this, the model estimates that around 1 113 are two year and older males. After hunting in 2021, the model estimated that there were about 655 two year and older males left in a population of about 5 760. The population structure survey after hunting 2021 indicated that the younger males (two-year-olds) accounted for about 55 percent of the male segment (2 years and older)) after hunting. Based on this, and the model estimates there were about 295 three year and older males left, which corresponds to approx. five percent of the post-harvest population. This is somewhat higher than the government's target of keeping this percentage at 0-3.

Judging from the age composition of reindeer aged by counting cementum annuli in teeth of jaws collected from hunting, there appears to be a significant decrease in the proportion of males that are 5 years and older in the last two to three years. At the same time, the proportion of three- and four-year old males is increasing. This is probably due to a combination of several factors, including increased recruitment of young males because of less harvesting of calves, adult fe- males and young males, in an attempt to decrease the proportion of older males. It is therefore to be expected that a relatively (and unusually) large proportion of the population before hunting in the last two years has consisted of younger males.

Atypical CWD in Norway seems to mainly affect animals that are 12 years or older. For that reason, it is of interest to know if we can estimate the proportion of old individuals, based on variation in harvesting strategies, population sex ratio and recruitment rates. We have started to work on a theoretical model and compare model outputs with empirical data from populations (municipalities) where adult moose are aged. The empirical analyzes show that the age structure in the empirical data corresponds with the expectations from the model populations. On average, the proportion of old (12 years and older) female moose of all adults (2 years and older) female moose was higher in populations with a low than high proportion of adult animals in the harvest, and higher in populations with a skewed sex ratio. In addition, there were more old females in populations with low productivity (low calf per cow) compared to high productivity. In the empirical analysis, the effect of the proportion of adult animals was statistically significant, while the effects of sex ratio and productivity were more uncertain. In practice, this suggests that with knowledge of harvest strategy, population sex ratio and recruitment rate (calf pr. female), we can predict the relative proportion of old individuals in different populations before and after hunting, and the relative probability that a shot, adult female moose is 12 years or older.

Christer M. Rolandsen, Erling J. Solberg, Erlend B. Nilsen, Roy Andersen, Olav Strand, Jørn Fremstad, Vebjørn Veiberg, Morten Heim, Frode Holmstrøm, Norsk institutt for naturforskning (NINA), Postboks 5685 Torgarden, 7485 Trondheim. christer.rolandsen@nina.no Jørn Våge, Petter Hopp, Sylvie L. Benestad, Hildegunn Viljugrein, Turid Vikøren, Knut Madslien, Attila Tarpai, Veterinærinstituttet, Postboks 750 Sentrum, 0106 Oslo. jorn.vage@vetinst.no Atle Mysterud, UiO, Postboks 1066 Blindern, 0316 OSLO. atle.mysterud@ibv.uio.no

https://brage.nina.no/nina-xmlui/bitstream/handle/11250/3000616/ninarapport2158.pdf

Norway More of the NON-CONTAGIOUS Scrapie (CWD) TSE Prion Disease Keeps Mounting

NEW CASE OF SCRAP DISEASE IN DEER 3.03.2022

BY: THE VETERINARY INSTITUTE The Veterinary Institute has detected scrapie in a deer from Bremanger municipality.

New case of scrapie in deer Deer colt. Photo: Svein-Håkon Lorentsen, NINA

The deer that was diagnosed with CWD was killed after observations of signs of disease, and is considered a "fallen game". Testing of fallen game is important with regard to diseases such as CWD. It is important to shoot game carcasses until there is a response to submitted samples.

The deer that were killed had detectable prions only in brain tissue, and not in the lymph node.

The detection in the deer from Bremanger is compatible with previous findings of scrapie (CWD) in this species in Norway. Observations of scrapie in Norwegian deer (and elk) indicate that the disease occurs sporadically. This is in contrast to wild reindeer, where it has been contagious between live animals.

Important to test fallen game - The killed deer is considered a "fallen game". That is, animals that are killed after observing signs of illness, traffic injury or have been found dead. Fallen game is statistically more likely to be sick and contagious than other game. For this reason, these animals are important to test, especially for CWD, says CWD coordinator and senior researcher Jørn Våge at the Veterinary Institute.

Analyzes provide more knowledge The Veterinary Institute analyzes samples of brain tissue and lymph nodes to gain important knowledge about the spread of the disease, and to be able to remove positive carcasses from nature, as they represent a potential source of infection.

Våge therefore emphasizes the importance of a thorough job that is done in the field to secure fallen game carcasses until there is a response to submitted samples. When CWD is detected, all residues from such an animal must be transported to the Veterinary Institute for further sampling and destruction.

Background Chronic Wasting Disease (CWD) is a prion disease that has been known in deer in North America for decades. In 2016, this serious animal disease was first detected in wild reindeer and elk in Norway, and in 2017 it was also detected in deer.

Read about mapping scrapie on the deer game portal Facts about scrapie Research on scrapie CWD at the Veterinary Institute Monitoring of scrapie

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Norway No extraordinary removal of wild reindeer on the Hardangervidda this winter

No extraordinary removal of wild reindeer on the Hardangervidda this winter

News | Date: 11.02.2022

The Ministry of Agriculture and Food and the Ministry of Climate and the Environment have concluded that no extraordinary sampling of wild reindeer will be carried out on the Hardangervidda in the winter of 2022.

In 2016, classic scrapie was detected in wild reindeer in Nordfjella. To combat the disease, the entire population was shot out. On 3 September 2020, the disease was detected in a wild reindeer buck field while hunting on the Hardangervidda.

To combat the disease outbreak on the Hardangervidda, a goal was set, among other things, to take out almost all bucks over 3.5 years and 1000 - 1500 adult sims during the hunt in 2021. Despite good efforts, the goal was not reached. Updated estimates from the Veterinary Institute show that there are probably still a small number of infected wild reindeer on the Hardangervidda. This gives cause for concern.

However, the condition and behavior of the population on the Hardangervidda, as well as the distribution and topography of the grazing areas, make it very difficult to carry out extraordinary extraction this winter. The cost in time and money will be high, and the probability of achieving a successful killing of many animals will be low. In addition, there has been strong local opposition to extraordinary withdrawals on the Hardangervidda.

The goal is still to limit infection, and if possible eradicate classic scrapie. To achieve this goal, many measures must be implemented at the same time, and good cooperation with local authorities and hunters is crucial. Although there is a risk in postponing further withdrawals until the autumn of 2022, the possible positive effect of a withdrawal in the winter of 2022 is too uncertain in relation to the negative consequences.

The Minister of Agriculture and Food and the Minister of Climate and the Environment have therefore concluded that it is not advisable to carry out extraordinary sampling of wild reindeer in the winter of 2022. This is in line with the professional recommendations given by the Norwegian Food Safety Authority and the Norwegian Environment Agency.

- It is demanding to deal with the serious animal disease scrapie on the Hardangervidda, says Minister of Agriculture and Food Sandra Borch (SP).

- Adult bucks represent a particular risk of further spread within the population, but it will be very demanding to succeed with a targeted withdrawal. In addition, there has been strong local opposition to the extraordinary removal of wild reindeer on the Hardangervidda. I have placed great emphasis on the consideration of good future management and local support for the further work, when I now follow the recommendation not to carry out extraordinary removal of wild reindeer, says Borch.

The Norwegian Food Safety Authority and the Norwegian Environment Agency will establish a broad-based working group to bring about a good dialogue on further management of wild reindeer on the Hardangervidda. They have ordered a professional assessment of genetics and population reduction, which is expected to be available in March 2022. Recommendation with final assessment of further management of the wild reindeer population on the Hardangervidda is expected to be completed in the summer of 2022.

- We still have a goal of limiting infection and, if possible, eradicating classical scrapie in Norway. To achieve this goal, it is important to keep ordinary hunting as the most important form of withdrawal. Fighting scrapie requires a great deal of effort over a long period of time. I look forward to further cooperation with local actors in the long-term work of dealing with this serious disease, says Minister of Climate and Environment Espen Barth Eide (AP).

Enlarge image

https://www.regjeringen.no/no/aktuelt/ikke-ekstraordinart-uttak-av-villrein-pa-hardangervidda-i-vinter/id2900756/

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Norway Infamous, Mysterious, Spontaneous, Sporadic, atypical, BSE, CWD, Scrapie, TSE, Prion, Outbreak Update

Sporadic Spontaneous CWD TSE Prion Outbreak Norway

Detection of scrapie in moose

Published 17.01.2022

The Veterinary Institute has diagnosed scrapie in a moose from Nord-Odal.

Scrapie has been detected in a moose in Nord-Odal. Prions have been detected in brain tissue. The Veterinary Institute has not received samples from the lymph node, so tissue from the lymph node has not been examined. Illustration photo: Knut Madslien / Veterinary Institute. Scrapie has been detected in a moose in Nord-Odal. Prions have been detected in brain tissue. The Veterinary Institute has not received samples from the lymph node, so the lymph node has not been examined for prions.

Prions have been detected in brain tissue from moose. No lymph node analyzes have been performed because the Veterinary Institute has only received a sample from the brain.

In Norway, various strains of prions that cause scrapie (CWD) have been detected. In wild reindeer, the prions are found in everyone in lymph nodes. For the animals that have progressed further in the disease, also in the brain.

The fact that contagious behavior of CWD has been observed in wild reindeer is believed to be related to the fact that prions are detected more widely in the body, such as in lymph nodes.

In moose with CWD in the Nordic countries, prions have so far been diagnosed only in brain tissue, and the disease probably occurs sporadically.

- In order to be able to carry out the safest possible diagnosis, it is therefore important that both brain tissue and lymph nodes can be examined, says CWD coordinator and senior researcher Jørn Våge at the Veterinary Institute.

Chronic Wasting Disease (CWD) is a prion disease that has been known in deer in North America for decades. In 2016, this serious animal disease was first detected in wild reindeer and elk in Norway, and in 2017 it was also detected in deer.

Read about mapping scrapie on the deer game portal

Facts about scrapie

Research on scrapie CWD at the Veterinary Institute

Monitoring of scrapie

Jørn Våge Senior researcher wildlife health, scrapie / CWD coordinator Mobile no: +47 93413615 E-mail: Jorn.Vage@vetinst.no

https://www.vetinst.no/nyheter/pavisning-av-skrantesjuke-hos-elg

https://www.vetinst.no/overvaking/chronic-wasting-disease-vilt-cwd

New case of scrapie in moose

Published 11.01.2022 Modified 12.01.2022

The Veterinary Institute has diagnosed scrapie in a moose from Tynset municipality.

The moose cow that was diagnosed with CWD is considered a "fall game". Testing of fallen game is important with regard to diseases such as CWD. It is important to shoot game carcasses until there is a response to submitted samples. Illustration photo: Colourbox. The moose cow that was killed had detectable prions only in brain tissue, and not in the lymph node.

The detection in the elk from Tynset is compatible with previous findings of scrapie (CWD) in this species in the Nordic countries. Observations of scrapie in Nordic moose indicate that the disease occurs sporadically. This is in contrast to wild reindeer, where it has been contagious between live animals.

Important to test fallen game - The elk cow is considered a "wild game". That is, animals that are killed after observing signs of illness, traffic injury or have been found dead. Fallen game is statistically more likely to be sick and contagious than other game. For this reason, these animals are important to test, especially for CWD, says CWD coordinator and senior researcher Jørn Våge at the Veterinary Institute.

Analyzes provide more knowledge The Veterinary Institute analyzes samples of brain tissue and lymph nodes to gain important knowledge about the spread of the disease, and to be able to remove positive carcasses from nature, as they represent a potential source of infection.

Våge therefore emphasizes the importance of the thorough work that is done in the field when securing fallen game carcasses until there is a response to submitted samples. When CWD is detected, all residues from such an animal must be transported to the Veterinary Institute for further sampling and destruction.

Background Chronic Wasting Disease (CWD) is a prion disease that has been known in deer in North America for decades. In 2016, this serious animal disease was first detected in wild reindeer and elk in Norway, and in 2017 it was also detected in deer.

Read about mapping scrapie on the deer game portal

Facts about scrapie

Research on scrapie CWD at the Veterinary Institute

Monitoring of scrapie

Jørn Våge Senior wildlife researcher, scrapie / CWD coordinator Mobile number: +47 93413615Email: Jorn.Vage@vetinst.no

https://www.vetinst.no/nyheter/nytt-tilfelle-av-skrantesjuke-hos-elg

The Veterinary Institute has confirmed CWD in the red deer from Etne in Norway

Publisert 24.09.2021

The institute notified on 17th of September of suspected infection with Chronic Wasting Disease (CWD) in a female red deer from Etne in the Western part of Norway. Examinations of additional material submitted to the Institute, could not detect infection in the lymph nodes.

The finding is compatible with «atypical» CWD, as previously described in Nordic moose and one red deer from Gjemnes in Nordmøre in 2017. «Atypical» CWD is defined on the basis of lack of detection of prions in lymphatic tissue, with the diagnostic methods used. The occurrence of CWD in Nordic moose and red deer has been sporadic, and not contagious as in wild reindeer.

Jørn Våge Seniorforsker vilthelse, skrantesyke/CWD-koordinator Mobilnr: +47 93413615 E-post: Jorn.Vage@vetinst.no

https://wwweng.vetinst.no/news/the-veterinary-institute-has-confirmed-cwd-in-the-red-deer-from-etne-in-norway

***> In moose with CWD in the Nordic countries, prions have so far been diagnosed only in brain tissue, and the disease probably occurs sporadically.

***> The occurrence of CWD in Nordic moose and red deer has been sporadic, and not contagious as in wild reindeer.

***> In recent years an atypical (spontaneous) variant of BSE has been discovered, which occurs spontaneously in older cows and not due to infection through feed.

***> Atypical BSE has a global and even prevalence, and occurs in older cattle. This supports the assumption that this extremely rare disease occurs spontaneously, similar to Creutzfeldt-Jacob's disease (CJD) in humans and atypical scrapie (scrapie Nor98) in sheep. We must assume that atypical BSE has existed for a long time before the classical form was discovered. Atypical BSE is not contagious to humans.

https://www.mattilsynet.no/language/english/animals/norway_has_the_best_status_for_bse.18263#:~:text=Norway%20confirmed%20its%20first%20case,identified%20in%20a%20culled%20cow.

https://www.vetinst.no/overvaking/skrapesjuke-tse-sau-og-geit/_/attachment/download/6e51a18d-f0bd-4c7d-af12-90c518ea1461:a66b6df7cc41fc3bec4ea87d8e82d54657f11019/2020_OK_Scrapie_Report%202019.pdf

https://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.562.7780&rep=rep1&type=pdf

Atypical BSE has a global and even prevalence, and occurs in older cattle. This supports the assumption that this extremely rare disease occurs spontaneously, similar to Creutzfeldt-Jacob's disease (CJD) in humans and atypical scrapie (scrapie Nor98) in sheep. We must assume that atypical BSE has existed for a long time before the classical form was discovered. Atypical BSE is not contagious to humans.

https://www.vetinst.no/sykdom-og-agens/kugalskap-bse

https://www.mattilsynet.no/dyr_og_dyrehold/dyrehelse/dyresykdommer/bse/bekreftet_atypisk_bse_hos_norsk_ku/

https://www.vetinst.no/overvaking/bse-storfe/_/attachment/download/eabf1f1c-2bb2-4d75-8fe3-9bb97b7f3bb8:0be64dc8c4585ac5c60a6c0b7a4de48a58e829fd/2020_OK_%20BSE_Report_%202019.pdf

Like atypical Nor-98 atypical Scrapie in Norway, it's incredibly stupid thing to assume that atypical scrapie, or atypical cwd is an old age disease that is not transmissible, and this assumption will only help spread the TSE Prion agent, just like the atypical BSE in Norway, just like the atypical Nor-98 Scrapie in Norway, and now with the atypical CWD TSE Prion in Norway, you can't wish these TSE prion diseases away with fake science i.e. spontaneous sporadic old age disease not transmissible. ...terry

FRIDAY, SEPTEMBER 24, 2021

NORWAY CONFIRMS ATYPICAL CWD TSE Prion DEER IN ETNE

https://nor-98.blogspot.com/2021/09/norway-confirms-atypical-cwd-tse-prion.html

EFSA ***> AS is considered more likely (subjective probability range 50–66%) that AS is a non-contagious, rather than a contagious, disease.

https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2021.668

when the flip, flops, then twists and turns into something different altogether, another worst case scenario with the TSE Prions…terry

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

Sara Canoyra, Alba Marín-Moreno, Juan Carlos Espinosa, +5 , and Juan María Torres jmtorres@inia.csic.es

Authors Info & Affiliations Edited by Byron Caughey, National Institute of Allergy and Infectious Diseases (National Institutes of Health), Hamilton, MT; received January 17, 2025; accepted June 7, 2025 by Editorial Board Member Lila M. Gierasch July 15, 2025 122 (29) e2501104122 https://doi.org/10.1073/pnas.2501104122

Our results suggest that the conformational shift could be the principal mechanism responsible for the c-BSE emergence. Furthermore, the selection model was dismissed as the key mechanism based on the analysis of an artificial c-BSE and AS mixture. The ability of the AS conformers to shift conformation to a c-BSE one supports the hypothesis that the epidemic c-BSE prion may have originated from the transmission of AS in cattle.

https://www.pnas.org/doi/10.1073/pnas.2501104122

Assessment of the Zoonotic Potential of Atypical Scrapie Prions in Humanized Mice Reveals Rare Phenotypic Convergence but Not Identity With Sporadic Creutzfeldt-Jakob Disease Prions

Alba Marín-Moreno , Fabienne Reine , Laetitia Herzog , Naima Aron , Florence Jaffrézic , Jean-Luc Vilotte , Human Rezaei , Olivier Andréoletti , Davy Martin , Vincent Béringue Author Notes

The Journal of Infectious Diseases, Volume 230, Issue 1, 15 July 2024, Pages 161–171, https://doi.org/10.1093/infdis/jiae093

Published: 24 February 2024

Conclusions The results obtained suggest a low zoonotic potential for AS. Rare adaptation may allow the emergence of prions phenotypically resembling those spontaneously forming in humans.

https://academic.oup.com/jid/article/230/1/161/7613915

MONDAY, OCTOBER 12, 2026

Chronic Wasting Disease CWD TSE Prion Strains and Potential Zoonotic Ramifications

https://chronic-wasting-disease.blogspot.com/2026/10/chronic-wasting-disease-cwd-tse-prion.html

TUESDAY, OCTOBER 13, 2026

Evidence for multiple prion conformers in natural scrapie isolates

https://scrapie-usa.blogspot.com/2026/10/evidence-for-multiple-prion-conformers.html

https://prpsc.proboards.com/thread/256/evidence-multiple-conformers-natural-scrapie

Friday, December 14, 2012

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

snip...

In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.

Animals considered at high risk for CWD include:

1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.

Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.

There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

snip...

http://webarchive.nationalarchives.gov.uk/20130908115835/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf

OIE atypical BSE

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

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

OIE Conclusions on transmissibility of atypical BSE among cattle

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

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

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

34 Scientific Commission/September 2019

3. Atypical BSE

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

The Group acknowledged the challenges in demonstrating the zoonotic transmission of atypical strains of BSE in natural exposure scenarios. Overall, the Group was of the opinion that, at this stage, it would be premature to reach a conclusion other than that atypical BSE poses a potential zoonotic risk that may be different between atypical strains.

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

snip...

REFERENCES

SNIP...END SEE FULL TEXT;

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

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

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

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

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

We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), is the third potentially zoonotic PD (with BSE and L-type BSE), thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.

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

2.3.2. New evidence on the zoonotic potential of atypical BSE and atypical scrapie prion strains

Olivier Andreoletti, INRA Research Director, Institut National de la Recherche Agronomique (INRA) – École Nationale Vétérinaire de Toulouse (ENVT), invited speaker, presented the results of two recently published scientific articles of interest, of which he is co-author:

‘Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice’ (MarinMoreno et al., 2020) and ‘The emergence of classical BSE from atypical/Nor98 scrapie’ (Huor et al., 2019).

In the first experimental study, H-type and L-type BSE were inoculated into transgenic mice expressing all three genotypes of the human PRNP at codon 129 and into adapted into ARQ and VRQ transgenic sheep mice. The results showed the alterations of the capacities to cross the human barrier species (mouse model) and emergence of sporadic CJD agents in Hu PrP expressing mice: type 2 sCJD in homozygous TgVal129 VRQ-passaged L-BSE, and type 1 sCJD in homozygous TgVal 129 and TgMet129 VRQ-passaged H-BSE.

https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2020.EN-1946

This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.

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

VKM Report 2017:9

CWD in Norway – a state of emergency for the future of cervids (Phase II)

Opinion of the Panel on Biological Hazards of the Norwegian Scientific Committee for Food Safety

https://bds.org.uk/wp-content/uploads/pdfs/VKM%202017%20report%202%20-%20CWD%20in%20Norway%202.pdf

From: Terry S. Singeltary Sr.

Sent: Saturday, April 09, 2016 8:59 PM

To: BSE-L@LISTS.AEGEE.ORG

Cc: cjdvoice@yahoogroups.com ; bloodcjd@yahoogroups.com ; CJD-L@LISTS.AEGEE.ORG

Subject: The Norwegian Veterinary Institute (NVI, 2016) has reported a case of prion disease Cervid Spongiform Encephalopathy detected in free ranging wild reindeer (Rangifer tarandus tarandus)

Department for Environment, Food and Rural Affairs

Animal and Plant Health Agency

Veterinary & Science Policy Advice Team - International Disease Monitoring

1

Preliminary Outbreak Assessment

Cervid Spongiform Encephalopathy in Norway

7th April 2016 Ref: VITT/1200 Cervid disease in Norway

Disease Report

The Norwegian Veterinary Institute (NVI, 2016) has reported a case of prion disease detected in free ranging wild reindeer (Rangifer tarandus tarandus), one of a southern population of wild reindeer in the Nordfjella region of Norway; see map; http://www.vetinst.no/eng/Highlights/The-first-detection-of-Chronic-Wasting-Disease-CWD-in-Europe). The (found dead) adult doe showed signs of below average body condition and was tested as part of the national surveillance programme for CWD in wild ungulates. Prion disease was confirmed in mid-March by both biochemical and immunohistochemical tests. Samples are being sent to the OIE reference laboratory (Canada) before confirmation as Chronic Wasting Disease can be made. If confirmed, this would be the first case of Chronic Wasting Disease detected in Europe. Initial experimental research, suggests that human susceptibility to CWD is low and there may be a robust species barrier preventing CWD transmission to humans (Sigurdson, 2008). Norway has put in place additional surveillance.

Situation Assessment

CWD is found in 21 States in the USA as well as the provinces of Alberta and Saskatchewan in Canada (UGS, 2016). It is a slow moving, contagious prion disease which only affects several species of captive and wild cervid, commonly the White tailed deer, Odocoileus virginianus), elk (Cervus elaphus nelsoni) and moose (Alces alces shirasi). This would be the first detection in a naturally-infected reindeer or the related (American) subspecies, caribou.

Current range of CWD in North America (USGS, 2016)

Department for Environment, Food and Rural Affairs Animal and Plant Health Agency Veterinary & Science Policy Advice Team - International Disease Monitoring

2

There are four native cervid species in Norway: moose (Alces alces), red deer (Cervus elaphus), roe deer (Capreolus capreolus) and reindeer (Rangifer tarandus). A large number of free ranging populations graze in areas where another, TSE, scrapie, has been detected (Sviland et al. 2015). Hunting is a popular sport, with over 5% of Norwegians holding hunting licenses to hunt all four native species and around 100,000 animals are hunted a year (Norwegian Scientific Committee for Food Safety, 2013).

Reindeer, where farmed or herded in Norway, come under the jurisdiction of the Norwegian Reindeer Herding Act, 2007. Almost 40% of the Nordic Countries is a reindeer herding area with between 240,000 and 250,000 animals, located mostly in the North, however herding in Norway is restricted to those with the right to have a reindeer earmark (Sámi people or first / second generation descendants of Sámi), except in the Norwegian Concession Area, where both Sámi and non-Sámi people are engaged in herding (Nordregio, 2016). Wild reindeer live in dispersed populations in separate high mountain areas in southern Norway. Official figures for hunted animals in 2013 put the number at 7,900, more than in previous years (Sviland et al., 2015; Norwegian Scientific Committee for Food Safety, 2013). Reindeer are an important species for this region, with special protected status.

Map of reindeer herding areas in Nordic Countries (Nordregio, 2016)

Norway carries out annual surveillance of reindeer for CWD (Sviland et al., 2015). Fallen stock of wild cervids over the age of 18 months and captive deer are tested. In 2014, very few animals (10) were tested as part of the surveillance and all tested negative; none of these were reindeer. In 2013, 10 animals also tested negative and in 2012, 21 animals tested negative.

Reindeer are not native to the UK and there are only a limited number of sites where reindeer are kept. According to the British Deer Society (2016), there are four sites in GB where reindeer may be found: one in Gloucestershire; one in Fife and two in Inverness-shire. There is understood to be a semi-wild reindeer herd in the Cairngorms, Scotland. According to TRACES, the EU electronic trade notification system, since 2013 there have been 27 consignments of 595 reindeer from the Nordic Countries, but only 2 from Norway,

Department for Environment, Food and Rural Affairs Animal and Plant Health Agency Veterinary & Science Policy Advice Team - International Disease Monitoring

3

in 2013 and 2015, totalling 28 reindeer. The UK has several species of native deer which may be susceptible (eg red deer).

This would be the first natural case of CWD in a reindeer. Experimental evidence from oral transmission experiments of CWD recovered from brain tissue of white tailed deer or elk demonstrated clinical disease in two of three reindeer which received inoculum from white tailed deer but in none of the three animals receiving inoculum from elk (Mitchell et al, 2012). Tissue distribution of prion protein (PrPCWD) was similar to that observed in other infected cervids, with multiple organ depositions. Therefore the presence of PrPCWD in saliva, faeces and urine, as seen with other cervids, would also be expected in the case of infected reindeer. In this small experiment, the three reindeer tested appeared not to be infected by CWD isolated from infected elk brain tissue, but there were no definitive conclusions as to whether this was due to a resistant genotype or low titre inoculum.

As seen with the disease status of the USA, once disease becomes established in wildlife, it is very difficult to control. Preventing contact with captive livestock or their pasture will be paramount.

Conclusion

The presence of CWD in a European population of cervids changes the risk assessment to the UK. The risk of incursion was previously considered to be very low, and when more information is available, we will re-assess. What is unknown at present is what the distribution of CWD is in deer in Scandinavia or other parts of Europe: whether it represents a wider problem in farmed reindeer, in other cervid species and other regions or if this is restricted to the wild reindeer in this region and how the disease has arisen: through contact with contaminated equipment, animal by-products from infected animals, feed or as a spontaneous mutation.

We have recently published an updated qualitative risk assessment on the entry pathways for CWD to GB, focussing on North America. This follows reports of imported white tailed deer urine which may be used by hunters as lures in Europe and the assessment considers the potential for such products, whether processed or not, for carrying CWD infectious agents. Other potential pathways include contaminated footwear, hunting equipment or pet food made from venison. For more information please see our qualitative risk assessments available on the gov.uk website, or see www.bds.org.uk.

We will report again when more information is available.

Authors Dr Helen Roberts Dr Paul Gale Dr Jim Hope Dr Marion Simmons Department for Environment, Food and Rural Affairs Animal and Plant Health Agency Veterinary & Science Policy Advice Team - International Disease Monitoring 4

References British Deer Society (2016) Where to see deer http://www.bds.org.uk/index.php/advice-education/watching-deer/where-to-see-deerAccessed 06/04/2016. Mitchell GB, Sigurdson CJ, O’Rourke KI, Algire J, Harrington NP, et al. (2012)

Experimental Oral Transmission of Chronic Wasting Disease to Reindeer (Rangifer tarandus tarandus). PLoS ONE 7(6): e39055. doi:10.1371/journal.pone.0039055. Nordregio (2016) Reindeer herding area in the Nordic Countries

http://www.nordregio.se/Templates/NordRegio/Pages/MapPage.aspx?id=3619&epslanguage=enAccessed 06/04/2016.

Norwegian Scientific Committee for Food Safety (2013) Risk assessment of lead exposure from cervid meat in Norwegian consumers and in hunting dogs. http://www.english.vkm.no/dav/bfbc0b16e9.pdf Accessed 06/04/2016.

NVI (2016) The first detection of Chronic Wasting Disease (CWD) in Europe.

http://www.vetinst.no/eng/Highlights/The-first-detection-of-Chronic-Wasting-Disease-CWD-in-Europe Accessed 06/04/2016. Sigurdson, C.J. (2008)

A prion disease of cervids: Chronic wasting disease. Veterinary Research, 39, 41. Svilan, S., Vikoren, T., Tarpai, A. & Benestad, S.L. (2015) The surveillance programme for Chronic Wasting Disease (CWD) in Norway, 2014. Surveillance programmes for terrestrial and aquatic animals in Norway. Annual report 2014. Oslo: Norwegian Veterinary Institute 2015. © Crown copyright 2016

You may re-use this information (excluding logos) free of charge in any format or medium, under the terms of the Open Government Licence v.2. To view this licence visit www.nationalarchives.gov.uk/doc/open-government-licence/version/2/ or email PSI@nationalarchives.gsi.gov.uk

This publication is available at https://www.gov.uk/government/collections/animal-diseases-international-monitoring Any enquiries regarding this publication should be sent to us at helen.roberts@apha.gsi.gov.uk

https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/515154/poa-tse-norway.pdf

Thursday, April 07, 2016

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

Sheep and cattle may be exposed to CWD via common grazing areas with affected deer but so far, appear to be poorly susceptible to mule deer CWD (Sigurdson, 2008).

***In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006). It is not known how susceptible humans are to CWD but given that the prion can be present in muscle, it is likely that humans have been exposed to the agent via consumption of venison (Sigurdson, 2008). Initial experimental research, however, suggests that human susceptibility to CWD is low and there may be a robust species barrier for CWD transmission to humans (Sigurdson, 2008). It is apparent, though, that CWD is affecting wild and farmed cervid populations in endemic areas with some deer populations decreasing as a result.

snip...

For the purpose of the qualitative risk assessment developed here it is necessary to estimate the probability that a 30-ml bottle of lure contains urine from an infected deer. This requires an estimate of the proportion of deer herds in the USA which are infected with CWD together with the within herd prevalence.

The distribution map of CWD in US shows it is present mainly in central states (Figure 1). However, Virginia in the east of the country has recorded seven recent cases of CWD (Anon 2015a). Some US manufacturers claim to take steps to prevent urine being taken from infected animals eg by sourcing from farms where the deer are randomly tested for CWD (Anon 2015a). However, if disease is already present and testing is not carried out regularly, captive populations are not necessarily disease free (Strausser 2014). Urine-based deer lures have been known to be collected from domestic white-tailed deer herds and therefore there is a recognised risk. This is reflected by 6 US States which have

14

banned the use of natural deer urine for lures, as the deer urine may be sourced from CWD-endemic areas in the USA as well as from areas free of CWD. For example, the US State of Virginia is banning the use of urine-based deer lures on July 2015 and Vermont from 2016 due to the risk of spread of CWD. Alaska banned their use in 2012 (Anon 2015a). Pennsylvania Game Commission has banned urine-based deer lures and acknowledged that there is no way to detect their use (Strausser 2014). On the basis of unpublished data (J. Manson, Pers. Comm.) it appears that up to 50% of deer herds can be infected with 80-90% of animals infected within some herds.

*** It is therefore assumed that probability that a 30-ml bottle of deer urine lure imported from the USA is sources from an infected deer is medium.

SNIP...

In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients.

***For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system.

***However, this recommendation is guidance and not a requirement by law.

***Animals considered at high risk for CWD include:

***1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

***2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

***Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants. The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB cannot be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the very low tonnage of non-fish origin processed animal proteins that were imported from US into GB.

*** Overall, therefore, it is considered there is a greater than negligible risk that (non-ruminant) animal feed and pet food containing deer and/or elk protein is imported into GB. There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

SNIP...

https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/514401/qra-chronic-wasting-disease.pdf

https://web.archive.org/web/20170126200814/https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/514401/qra-chronic-wasting-disease.pdf

Summary and MORE HERE ;

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

http://chronic-wasting-disease.blogspot.com/2016/04/what-is-risk-of-chronic-wasting-disease.html

I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids, as well as non-ruminants such as cats and dogs as well, as soon as possible for the following reasons...

31 Jan 2015 at 20:14 GMT

*** Ruminant feed ban for cervids in the United States? ***

31 Jan 2015 at 20:14 GMT

see Singeltary comment ;

http://www.plosone.org/annotation/listThread.action?root=85351

https://web.archive.org/web/20160128180140/http://www.plosone.org/annotation/listThread.action?root=85351

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

Greetings again FDA and Mr. Pritchett et al,

I would kindly like to comment on ;

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

#158

Guidance for Industry

Use of Material from Deer and Elk in Animal Feed

This version of the guidance replaces the version made available September15, 2003…

FDA recommends that materials from deer and elk considered at high risk for CWD no longer be entered into the animal feed system. Under present circumstances, FDA is not recommending that feed made from deer and elk from a non-endemic area be recalled if a State later declares the area endemic for CWD or a CWD eradication zone. In addition, at this time, FDA is not recommending that feed made from deer and elk believed to be from a captive herd that contained no CWD-positive animals be recalled if that herd is subsequently found to contain a CWD-positive animal. V. Use in animal feed of material from deer and elk NOT considered at high risk for CWD FDA continues to consider materials from deer and elk NOT considered at high risk for CWD to be acceptable for use in NON-RUMINANT animal feeds in accordance with current agency regulations, 21 CFR 589.2000. Deer and elk not considered at high risk include: (1) deer and elk from areas not declared by State officials to be endemic for CWD and/or to be CWD eradication zones; and (2) deer and elk that were not at some time during the 60-month period immediately before the time of slaughter in a captive herd that contained a CWD-positive animal. 3

http://www.fda.gov/downloads/AnimalVeterinary/GuidanceComplianceEnforcement/GuidanceforIndustry/UCM052506.pdf?source=govdelivery&utm_medium=email&utm_source=govdelivery

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

Greetings again FDA and Mr. Pritchett et al, MY comments and source reference of sound science on this very important issue are as follows ;

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

I kindly wish to once again submit to Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed.

Thank you kindly for allowing me to comment again, ...and again...and again, on a topic so important, why it is ‘NON-BINDING’ is beyond me. this should have been finalized and made ‘BINDING’ or MANDATORY OVER A DECADE AGO. but here lay the problem, once made ‘BINDING’ or ‘MANDATORY’, it is still nothing but ink on paper. we have had a mad cow feed ban in place since August 1997, and since then, literally 100s of millions of pounds BANNED MAD COW FEED has been sent out to commerce and fed out (see reference materials). ENFORCEMENT OF SAID BINDING REGULATIONS HAS FAILED US TOO MANY TIMES. so, in my opinion, any non-binding or voluntary regulations will not work, and to state further, ‘BINDING’ or MANDATORY regulations will not work unless enforced. with that said, we know that Chronic Wasting Disease CWD TSE Prion easily transmits to other cervid through the oral route. the old transmission studies of BSE TSE floored scientist once they figured out what they had, and please don’t forget about those mink that were fed 95%+ dead stock downer cow, that all came down with TME.

please see ;

*** PLEASE SEE THIS URGENT UPDATE ON CWD AND FEED ANIMAL PROTEIN ***

Sunday, March 20, 2016

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed ***UPDATED MARCH 2016*** Singeltary Submission

http://chronic-wasting-disease.blogspot.com/2016/03/docket-no-fda-2003-d-0432-formerly-03d.html

see what the UK GOV warns about this;

http://webarchive.nationalarchives.gov.uk/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf

CWD Risk Assessments

https://www.gov.uk/government/publications/chronic-wasting-disease-risk-assessments

Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment for chronic wasting disease prions in cervids and wild pigs in North America. Updated: 28 January 2026

https://www.gov.uk/government/publications/chronic-wasting-disease-prions-in-cervids-and-wild-pigs-in-north-america

Import of hay and straw - Import Information Note (IIN) AF/1 Updated: 7 January 2026

https://www.gov.uk/government/publications/hay-and-straw-import-information-notes/import-of-hay-and-straw-import-information-note-iin-af1

Risk assessment: chronic wasting disease to Great Britain from Scandinavia and North America A qualitative risk assessment on the likelihood of chronic wasting disease incursion into Great Britain from Scandinavia and North America, and the potential for subsequent transmission among susceptible animal populations. Updated: 18 June 2025

https://www.gov.uk/government/publications/risk-assessment-chronic-wasting-disease-to-great-britain-from-scandinavia-and-north-america

Prohibitions on the import of deer urine or faeces, and lures - Import Information Note (IIN) ABP/44 Updated: 2 December 2024

https://www.gov.uk/government/publications/deer-urine-or-faeces-and-lures-import-information-notes/prohibitions-on-the-import-of-deer-urine-or-faeces-and-lures-import-information-note-iin-abp44

Monday, April 17, 2023

EFSA Monitoring of chronic wasting disease (CWD) (IV)

https://efsaopinioncwd.blogspot.com/2023/04/efsa-monitoring-of-chronic-wasting.html

Saturday, April 9, 2022

EFSA EU Request for a scientific opinion on the monitoring of Chronic Wasting Disease (CWD) EFSA-Q-2022-00114 M-2022-00040 Singeltary Submission

https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html

DEER BRAIN SURVEY RED DEER ATAXIA OR CWD???

https://web.archive.org/web/20090506025229/http://www.bseinquiry.gov.uk/files/yb/1991/11/20004001.pdf

https://webarchive.nationalarchives.gov.uk/ukgwa/20080102232755/http://www.bseinquiry.gov.uk/files/yb/1992/11/04002001.pdf

https://web.archive.org/web/20161027181651/http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1982/07/05001001.pdf

https://web.archive.org/web/20161027182246/http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1982/07/28001001.pdf

https://web.archive.org/web/20161027181712/http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1983/03/01001001.pdf

https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html

Norway Chronic Wasting Disease CWD TSE Prion Update September 2026

Chronic Wasting Disease (CWD)

Updated 09.02.2026

https://chronic-wasting-disease.blogspot.com/2026/09/norway-chronic-wasting-disease-cwd-tse.html

TUESDAY, JANUARY 23, 2024

Lessons learned and lingering uncertainties after seven years of chronic wasting disease management in Norway

https://chronic-wasting-disease.blogspot.com/2024/01/lessons-learned-and-lingering.html

Risk assessment on the likelihood of spread of chronic wasting disease to Great Britain from Scandinavia and North America in November 2023 (executive summary) Updated: 18 June 2025

https://www.gov.uk/government/publications/risk-assessment-chronic-wasting-disease-to-great-britain-from-scandinavia-and-north-america/risk-assessment-on-the-likelihood-of-spread-of-chronic-wasting-disease-to-great-britain-from-scandinavia-and-north-america-in-november-2023-executive

Monday, February 13, 2023

Norway Changes to the TSE and CWD regulations

https://chronic-wasting-disease.blogspot.com/2023/02/norway-changes-to-tse-and-cwd.html

FRIDAY, FEBRUARY 11, 2022

Norway No extraordinary removal of wild reindeer on the Hardangervidda this winter

https://chronic-wasting-disease.blogspot.com/2022/02/norway-no-extraordinary-removal-of-wild.html

Friday, February 10, 2023

OIE WAHIS SPAIN BOVINE SPONGIFORM ENCEPHALOPATHY BSE Atypical H-Type

https://woahoie.blogspot.com/2023/02/oie-wahis-spain-bovine-spongiform.html

FRIDAY, FEBRUARY 03, 2023

OIE Netherlands Bovine Spongiform Encephalopathy BSE, atypical strain, L-type

https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html

Thursday, February 2, 2023

EFSA OIE WOHA UPDATES ON CHRONIC WASTING DISEASE CWD TSE PrP ZOONOSIS

https://woahoie.blogspot.com/2023/02/efsa-oie-woha-updates-on-chronic.html

MONDAY, JUNE 27, 2022

Norway Mapping and monitoring of chronic wasting disease (CWD) 2021

https://chronic-wasting-disease.blogspot.com/2022/06/norway-mapping-and-monitoring-of.html

MONDAY, JANUARY 17, 2022

Norway Infamous, Mysterious, Spontaneous, Sporadic, atypical, BSE, CWD, Scrapie, TSE, Prion, Outbreak Update

https://chronic-wasting-disease.blogspot.com/2022/01/norway-infamous-mysterious-spontaneous.html

WEDNESDAY, MARCH 06, 2019

Norway The Madness Continues in Nordfjella Chronic Wasting Disease CWD TSE Prion

https://chronic-wasting-disease.blogspot.com/2019/03/norway-madness-continues-in-nordfjella.html

hay, straw, grains...and cwd tse prion

***> NORWAY CWD UPDATE December 2018 Report from the Norwegian Scientific Committee for Food and Environment (VKM) 2018: 16

Factors that can contribute to spread of CWD – an update on the situation in Nordfjella, Norway

Opinion of Panel on biological hazards of the Norwegian Scientific Committee for Food and Environment 13.12.2018 ISBN: 978-82-8259-316-8 ISSN: 2535-4019 Norwegian Scientific Committee for Food and Environment (VKM) Po 222 Skøyen 0213 Oslo Norway FRIDAY, DECEMBER 14, 2018 Norway, Nordfjella VKM 2018 16

Factors that can contribute to spread of CWD TSE Prion UPDATE December 14, 2018

https://vkm.no/download/18.696229a71677d983532c0c11/1544792739325/CWD%20factors%20for%20spread%202018.pdf

https://chronic-wasting-disease.blogspot.com/2018/12/norway-nordfjella-vkm-2018-16-factors.html

THURSDAY, OCTOBER 25, 2018

***> Norway New additional requirements for imports of hay and straw for animal feed from countries outside the EEA due to CWD TSE Prion

https://www.mattilsynet.no/dyr_og_dyrehold/for/nye_regler_om_tilleggskrav_ved_import_av_hoy_og_halm_til_dyrefor.32299

https://chronic-wasting-disease.blogspot.com/2018/10/norway-new-additional-requirements-for.html

THURSDAY, OCTOBER 25, 2018

Norway New additional requirements for imports of hay and straw for animal feed from countries outside the EEA due to CWD TSE Prion

http://chronic-wasting-disease.blogspot.com/2018/10/norway-new-additional-requirements-for.html

Chronic wasting disease: how to spot and report the disease How to spot chronic wasting disease (CWD), what to do if you suspect it and measures to prevent its spread. Updated: 18 October 2018

https://www.gov.uk/guidance/chronic-wasting-disease

Chronic wasting disease: risk assessments Qualitative assessments of the risk of chronic wasting disease being introduced in the UK, and the risk of exposures. Updated: 13 August 2018

https://www.gov.uk/government/publications/chronic-wasting-disease-risk-assessments

TUESDAY, JULY 03, 2018

Chronic Wasting Disease CWD TSE Prion Global Report Update, USA, CANADA, KOREA, NORWAY, FINLAND, Game Farm

http://chronic-wasting-disease.blogspot.com/2018/07/chronic-wasting-disease-cwd-tse-prion.html

MONDAY, AUGUST 14, 2017

NORWAY CWD, SHEEP GRAZING, and Scrapie, What If? Scrapie/reindeer investigation

http://chronic-wasting-disease.blogspot.com/2017/08/norway-cwd-sheep-grazing-and-scrapie.html018/10/norway-new-additional-requirements-for.html

Accessed 06/04/2016

Mitchell GB, Sigurdson CJ, O’Rourke KI, Algire J, Harrington NP, et al. (2012)

Experimental Oral Transmission of Chronic Wasting Disease to Reindeer (Rangifer tarandus tarandus).

PLoS ONE 7(6): e39055. doi:10.1371/journal.pone.0039055.

Nordregio (2016) Reindeer herding area in the Nordic Countries

http://www.nordregio.se/Templates/NordRegio/Pages/MapPage.aspx?id=3619&epslanguage=en

Accessed 06/04/2016.

NVI (2016) The first detection of Chronic Wasting Disease (CWD) in Europe.

http://www.vetinst.no/eng/Highlights/The-first-detection-of-Chronic-Wasting-Disease-CWD-in-Europe

Saturday, April 9, 2016

The Norwegian Veterinary Institute (NVI, 2016) has reported a case of prion disease Cervid Spongiform Encephalopathy detected in free ranging wild reindeer (Rangifer tarandus tarandus)

Department for Environment, Food and Rural Affairs

http://transmissiblespongiformencephalopathy.blogspot.com/2016/04/the-norwegian-veterinary-institute-nvi.html

Thursday, April 07, 2016

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

Sheep and cattle may be exposed to CWD via common grazing areas with affected deer but so far, appear to be poorly susceptible to mule deer CWD (Sigurdson, 2008).

***In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006). It is not known how susceptible humans are to CWD but given that the prion can be present in muscle, it is likely that humans have been exposed to the agent via consumption of venison (Sigurdson, 2008). Initial experimental research, however, suggests that human susceptibility to CWD is low and there may be a robust species barrier for CWD transmission to humans (Sigurdson, 2008). It is apparent, though, that CWD is affecting wild and farmed cervid populations in endemic areas with some deer populations decreasing as a result.

snip...

For the purpose of the qualitative risk assessment developed here it is necessary to estimate the probability that a 30-ml bottle of lure contains urine from an infected deer. This requires an estimate of the proportion of deer herds in the USA which are infected with CWD together with the within herd prevalence.

The distribution map of CWD in US shows it is present mainly in central states (Figure 1). However, Virginia in the east of the country has recorded seven recent cases of CWD (Anon 2015a). Some US manufacturers claim to take steps to prevent urine being taken from infected animals eg by sourcing from farms where the deer are randomly tested for CWD (Anon 2015a). However, if disease is already present and testing is not carried out regularly, captive populations are not necessarily disease free (Strausser 2014). Urine-based deer lures have been known to be collected from domestic white-tailed deer herds and therefore there is a recognised risk. This is reflected by 6 US States which have

14

banned the use of natural deer urine for lures, as the deer urine may be sourced from CWD-endemic areas in the USA as well as from areas free of CWD. For example, the US State of Virginia is banning the use of urine-based deer lures on July 2015 and Vermont from 2016 due to the risk of spread of CWD. Alaska banned their use in 2012 (Anon 2015a). Pennsylvania Game Commission has banned urine-based deer lures and acknowledged that there is no way to detect their use (Strausser 2014). On the basis of unpublished data (J. Manson, Pers. Comm.) it appears that up to 50% of deer herds can be infected with 80-90% of animals infected within some herds.

*** It is therefore assumed that probability that a 30-ml bottle of deer urine lure imported from the USA is sources from an infected deer is medium.

SNIP...

In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients.

***For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system.

***However, this recommendation is guidance and not a requirement by law.

***Animals considered at high risk for CWD include:

***1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

***2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

***Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

***The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB cannot be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the very low tonnage of non-fish origin processed animal proteins that were imported from US into GB.

*** Overall, therefore, it is considered there is a greater than negligible risk that (non-ruminant) animal feed and pet food containing deer and/or elk protein is imported into GB. There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

SNIP...

https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/514401/qra-chronic-wasting-disease.pdf

Summary and MORE HERE ;

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

http://chronic-wasting-disease.blogspot.com/2016/04/what-is-risk-of-chronic-wasting-disease.html

THURSDAY, APRIL 07, 2016

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

https://chronic-wasting-disease.blogspot.com/2016/04/what-is-risk-of-chronic-wasting-disease.html

Monday, May 05, 2014

Member Country details for listing OIE CWD 2013 against the criteria of Article 1.2.2., the Code Commission recommends consideration for listing

http://chronic-wasting-disease.blogspot.com/2014/05/member-country-details-for-listing-oie.html

This assessment has been provided as an update to a previous version carried out in 2012, due to new evidence about the import of lures for deer hunting which contain deer urine. The previous assessment is available at:

http://webarchive.nationalarchives.gov.uk/20140507133914/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf

Friday, November 22, 2013

*** Wasting disease is threat to the entire UK deer population CWD TSE PRION DISEASE Singeltary submission to Scottish Parliament

http://chronic-wasting-disease.blogspot.com/2013/11/wasting-disease-is-threat-to-entire-uk.html

Sunday, July 21, 2013

Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

*** Singeltary Submission WG18417

http://transmissiblespongiformencephalopathy.blogspot.com/2013/07/welsh-government-and-food-standards.html

Friday, December 14, 2012

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

https://web.archive.org/web/20160128180140/http://chronic-wasting-disease.blogspot.com/2012/12/defra-uk-what-is-risk-of-chronic.html

https://chronic-wasting-disease.blogspot.com/2012/12/defra-uk-what-is-risk-of-chronic.html

Tuesday, July 17, 2012

O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012

http://transmissiblespongiformencephalopathy.blogspot.com/2012/07/oie-bse-cwd-scrapie-tse-prion-disease.html

CWD Risk Assessments

https://www.gov.uk/government/publications/chronic-wasting-disease-risk-assessments

What is the risk of introducing Chronic Wasting Disease being introduced into Great Britain ? a qualitative risk assessment October 2012

https://webarchive.nationalarchives.gov.uk/ukgwa/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf

https://assets.publishing.service.gov.uk/media/5b71567ae5274a1d14d1bf7e/Cover_paper_for_CWD_RA_26062018.pdf

https://assets.publishing.service.gov.uk/media/5b715955e5274a1d16fa0494/DEFRA_QRA_TSE_in_cervids_June2018_v1.pdf

https://assets.publishing.service.gov.uk/media/5b715793e5274a1d053d0eaa/FSA_CWD_RA_for_publication_with_logo_July_2018.pdf

https://assets.publishing.service.gov.uk/media/5b6d71e3ed915d30f76f8a82/PHE_CWD__risk_assessment_July_2018.pdf

***> CHRONIC WASTING DISEASE CWD TSE PRION ZOONOTIC ZOONOSIS UPDATE <***

CDC CWD TSE Prion Update April 17, 2024

KEY POINTS

Chronic wasting disease affects deer, elk and similar animals in the United States and a few other countries.

The disease hasn't been shown to infect people.

However, it might be a risk to people if they have contact with or eat meat from animals infected with CWD.

https://web.archive.org/web/20240814194015/https://www.cdc.gov/chronic-wasting/about/index.html

CWD TSE Prion, Meats

Prions in Muscles of Cervids with Chronic Wasting Disease, Norway

Volume 31, Number 2—February 2025

Research

Prions in Muscles of Cervids with Chronic Wasting Disease, Norway

Snip…

In summary, the results of our study indicate that prions are widely distributed in peripheral and edible tissues of cervids in Norway, including muscles. This finding highlights the risk of human exposure to small amounts of prions through handling and consuming infected cervids.

Appendix

https://wwwnc.cdc.gov/eid/article/31/2/24-0903-app1.pdf

https://wwwnc.cdc.gov/eid/article/31/2/24-0903_article

Volume 31, Number 2—February 2025

Dispatch

Detection of Chronic Wasting Disease Prions in Raw, Processed, and Cooked Elk Meat, Texas, USA

Snip…

Of note, our data show that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification. Considering the potential implications in food safety and public health, we believe that the findings described in this study warrant further research. Our results suggest that although the elk meat used in this study resisted different manipulations involved in subsequent consumption by humans, their zoonotic potential was limited. Nevertheless, even though no cases of CWD transmission to human have been reported, the potential for human infection is still unclear and continued monitoring for zoonotic potential is warranted.

https://wwwnc.cdc.gov/eid/article/31/2/24-0906_article

Detection of chronic wasting disease prions in processed meats

Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked.

Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated.

"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."

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

DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.

In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.

Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.

CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.

Our results show positive prion detection in all products.

Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.

Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.

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

Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.

Further passage to cervidized mice revealed transmission with a 100% attack rate.

Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.

The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.

Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease

=====

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

https://defrauktseprp.blogspot.com/2026/06/hazard-identification-for-risks-to.html 

USA Report, Scrapie, CWD, BSE, TSE, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026, oh my!

“We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease.”

***> Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease

Nathaniel D. Denkers , Clare E. Hoover , Kristen A. Davenport, Davin M. Henderson, Erin E. McNulty, Amy V. Nalls, Candace K. Mathiason, Edward A. Hoover

Published: August 20, 2020

https://doi.org/10.1371/journal.pone.0237410

Abstract

The minimum infectious dose required to induce CWD infection in cervids remains unknown, as does whether peripherally shed prions and/or multiple low dose exposures are important factors in CWD transmission. With the goal of better understand CWD infection in nature, we studied oral exposures of deer to very low doses of CWD prions and also examined whether the frequency of exposure or prion source may influence infection and pathogenesis. We orally inoculated white-tailed deer with either single or multiple divided doses of prions of brain or saliva origin and monitored infection by serial longitudinal tissue biopsies spanning over two years. We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease. This was true whether the inoculum was administered as a single bolus or divided as three weekly 100 ng exposures. However, when the 300 ng total dose was apportioned as 10, 30 ng doses delivered over 12 weeks, no infection occurred. While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.

https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0237410

April 2026

https://fdabse589.blogspot.com/2026/04/usa-report-scrapie-cwd-bse-tse-cattle.html

https://www.researchgate.net/publication/403956772_USA_Report_Scrapie_CWD_BSE_TSE_Cattle_Sheep_Pigs_Cervid_Humans_Zoonotic_2026

***> 1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure.

https://www.ars.usda.gov/research/project/?accnNo=440677&fy=202

https://www.ars.usda.gov/research/publications/publication/?seqNo115=336834

https://wwwnc.cdc.gov/eid/article/30/8/24-0007_article

cwd tse prion, cattle, pigs, sheep, primates, oh my!  Prion Conference 2023

“Conclusions: 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.”

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.

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

***> don’t forget the pigs, outbreak of cwd in pigs in Texas and Arkansas;

Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment DEFRA 26 January 2026 Department for Environment, Food and Rural Affairs

Preliminary Outbreak Assessment

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

26 January 2026

Disease report

“They analysed over 300 brain and lymph node samples from 178 wild pigs living across Arkansas and Texas, USA…the researchers identified CWD prions in up to 37% of the lymph node samples and 15% of brain samples. The lowest detection rates were in the Texas samples (below 16%), matching the lower CWD prevalence in the state’s cervid population.”

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

Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United State Volume 31, Number 1—January 2025

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

Department for Environment, Food and Rural Affairs

Preliminary Outbreak Assessment

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

26 January 2026

Disease report

Chronic wasting disease (CWD) is a fatal neurodegenerative disease of cervids, such as deer, elk, moose and reindeer. It is caused by prions – infectious proteins that cause normal cellular prion proteins to misfold (CIDRAP, 2025). The disease is widespread in captive and free-ranging cervids in North America (Figure 1). For the first time, CWD prions have also been detected in the tissues of wild pigs (Sus scrofa) caught in CWD-affected areas of the USA (Soto et al. 2025). This discovery emerged from a study designed to investigate potential interactions between wild pigs and CWD prions, as wild pigs often coexist with cervids, which can shed prions into the environment. The following assessment discusses the epidemiology of CWD in North America and the detection of CWD prions in wild pigs. It also considers the potential implications for Great Britain.

Conclusion

CWD has continued to spread among captive and free-ranging cervids in North America since it was first detected in the 1960s. The finding of CWD prions in wild pigs in the USA suggests they could contribute towards transmission of the disease, influencing its epidemiology, geographic distribution and interspecies spread. However, further research is needed to confirm this. CWD has never been reported in Great Britain and the current risk of CWD prions being introduced into Great Britain’s wild pig or cervid population ranges from very low to negligible.

Readers are reminded to be vigilant for signs of CWD. Information on how to spot the disease can be found here. Suspected cases must be reported immediately to the Defra Rural Services Helpline on 03000 200 301. In Wales, call 0300 303 8268. In Scotland, contact your local Field Services Office. Failure to do so is an offence. We will continue to monitor the situation.

Authors • Lawrence Finn • Dr. Lauren Perrin • John Spiropoulos • Dr. Helen RobertsDepartment for Environment, Food and Rural Affairs

References

snip…see full report;

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

WEDNESDAY, JANUARY 28, 2026

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

https://journals.asm.org/doi/10.1128/mbio.01800-25

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

https://media.ark.org/agri/Moore_2017_Swine_CWD.pdf

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

cwd to sheep

Chronic Wasting Disease CWD vs Scrapie TSE Prion

https://www.ars.usda.gov/research/publications/publication/?seqNo115=410511

Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP

Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin

Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44. Interpretive Summary:

Technical Abstract: We have previously shown that the chronic wasting disease (CWD) agent from white-tailed deer can be transmitted to domestic pigs via intracranial or oral inoculation although with low attack rates and restricted PrPSc accumulation. The objective of this study was to assess the potential for cross-species transmission of pig-passaged CWD using bioassay in transgenic mice. Transgenic mice expressing human (Tg40), bovine (TgBovXV) or porcine (Tg002) PRNP were inoculated intracranially with 1% brain homogenate from a pig that had been intracranially inoculated with a pool of CWD from white-tailed deer. This pig developed neurological clinical signs, was euthanized at 64 months post-inoculation, and PrPSc was detected in the brain. Mice were monitored daily for clinical signs of disease until the end of the study. Mice were considered positive if PrPSc was detected in the brain using an enzyme immunoassay (EIA). In transgenic mice expressing porcine prion protein the average incubation period was 167 days post-inoculation (dpi) and 3/27 mice were EIA positive (attack rate = 11%). All 3 mice were found dead and clinical signs were not noted prior to death. One transgenic mouse expressing bovine prion protein was euthanized due to excessive scratching at 617 dpi and 2 mice culled at the end of the study at 700 dpi were EIA positive resulting in an overall attack rate of 3/16 (19%). None of the transgenic mice expressing human prion protein that died or were euthanized up to 769 dpi were EIA positive and at study end point at 800 dpi 2 mice had positive EIA results (overall attack rate = 2/20 = 10%). The EIA optical density (OD) readings for all positive mice were at the lower end of the reference range (positive mice range, OD = 0.266-0.438; test positive reference range, OD = 0.250-4.000). To the authors’ knowledge, cervid-derived CWD isolates have not been successfully transmitted to transgenic mice expressing human prion protein.

The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent.

If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.

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

Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies

Location: Virus and Prion Research

2017 Annual Report

4. Accomplishments

1. Showed that swine are potential hosts for the scrapie agent. A naturally occurring prion disease has not been recognized in swine, but the agent of bovine spongiform encephalopathy does transmit to swine by experimental routes. Swine are thought to have a robust species barrier when exposed to the naturally occurring prion diseases of other species, but the susceptibility of swine to the agent of sheep scrapie has not been thoroughly tested. ARS researchers at Ames, Iowa conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Necropsies were done on a subset of animals at approximately 6 months post inoculation (PI): the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of transmissible spongiform encephalopathies (TSE) until study termination at 80 months PI or when removed due to intercurrent disease. Brain samples were examined by multiple diagnostic approaches, and for a subset of pigs in each inoculation group, bioassay in mice expressing porcine prion protein. At 6 months PI, no evidence of scrapie infection was noted by any diagnostic method. 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.

2. Determined that pigs naturally exposed to chronic wasting disease (CWD) may act as a reservoir of CWD infectivity. Chronic wasting disease is a naturally occurring, fatal, neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of CWD disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation. Pigs were assigned to 1 of 3 groups: intracranially inoculated; orally inoculated; or non-inoculated. At market weight age, half of the pigs in each group were tested ('market weight' groups). The remaining pigs ('aged' groups) were allowed to incubate for up to 73 months post inoculation (MPI). Tissues collected at necropsy were examined for disease-associated prion protein (PrPSc) by multiple diagnostic methods. Brain samples from selected pigs were bioassayed in mice expressing porcine prion protein. Some pigs from each inoculated group were positive by one or more tests. Bioassay was positive in 4 out of 5 pigs assayed. Although only small amounts of PrPSc were detected using sensitive methods, this study demonstrates that pigs can serve as hosts for CWD. Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. 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.

3. Developed a method for amplification and discrimination of the 3 forms of BSE in cattle. The prion protein (PrP) is a protein that is the causative agent of transmissible spongiform encephalopathies (TSEs). The disease process involves conversion of the normal cellular PrP to a pathogenic misfolded conformation. This conversion process can be recreated in the lab using a misfolding amplification process known as real-time quaking induced conversion (RT-QuIC). RT-QuIC allows the detection of minute amounts of the abnormal infectious form of the prion protein by inducing misfolding in a supplied substrate. Although RT-QuIC has been successfully used to detect pathogenic PrP with substrates from a variety of host species, prior to this work bovine prion protein had not been proven for its practical uses for RT-QuIC. We demonstrated that prions from transmissible mink encephalopathy (TME) and BSE-infected cattle can be detected with using bovine prion proteins with RT-QuIC, and developed an RT-QuIC based approach to discriminate different forms of BSE. This rapid and robust method, both to detect and discriminate BSE types, is of importance as the economic implications for different types of BSE vary greatly.

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

https://web.archive.org/web/20181013061739/https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017

“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.”

Title: Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease

Author item MOORE, SARAH - Orise Fellow item Kunkle, Robert item KONDRU, NAVEEN - Iowa State University item MANNE, SIREESHA - Iowa State University item SMITH, JODI - Iowa State University item KANTHASAMY, ANUMANTHA - Iowa State University item WEST GREENLEE, M - Iowa State University item Greenlee, Justin

Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 3/15/2017 Publication Date: N/A Citation: N/A Interpretive Summary:

Technical Abstract:

Aims: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of cervids. We previously demonstrated that disease-associated prion protein (PrPSc) can be detected in the brain and retina from pigs challenged intracranially or orally with the CWD agent. In that study, neurological signs consistent with prion disease were observed only in one pig: an intracranially challenged pig that was euthanized at 64 months post-challenge. The purpose of this study was to use an antigen-capture immunoassay (EIA) and real-time quaking-induced conversion (QuIC) to determine whether PrPSc is present in lymphoid tissues from pigs challenged with the CWD agent.

Methods: At two months of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 month challenge groups). The remaining pigs (>6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). The retropharyngeal lymph node (RPLN) was screened for the presence of PrPSc by EIA and immunohistochemistry (IHC). The RPLN, palatine tonsil, and mesenteric lymph node (MLN) from 6-7 pigs per challenge group were also tested using EIA and QuIC.

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

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

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

“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.”

Title: Prion infectivity detected in swine challenged with chronic wasting disease via the intracerebral or oral route

Author item MOORE, S - Orise Fellow item Kunkle, Robert item SMITH, JODI - Iowa State University item WEST-GREENLEE, M - Iowa State University item Greenlee, Justin

Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 4/4/2016 Publication Date: N/A Citation: N/A Interpretive Summary:

Technical Abstract: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of North American cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. In the US, feeding of ruminant by-products to ruminants is prohibited, but feeding of ruminant materials to swine, mink, and poultry still occurs. In addition, scavenging of CWD-affected cervid carcasses by feral pigs presents a potential risk for CWD exposure. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following oral or intracranial experimental challenge. At 8 weeks of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 month challenge groups). The remaining pigs (>6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). At death a complete necropsy examination was performed, including testing of tissues for misfolded prion protein (PrPcwd) by western blotting (WB), enzyme-linked immunosorbent assay (ELISA), and immunohistochemistry (IHC). None of the pigs developed clinical signs consistent with prion disease. Four >6 month intracranially challenged pigs (survival times 45-73 mpc) were positive by ELISA, two were also positive by WB, and one was positive by IHC. One >6 month orally challenged pig (64 mpc) was positive by ELISA. To further investigate the potential for infectivity, brain tissue from selected pigs was bioassayed in mice expressing porcine PRNP. Tissue from the two WB-positive >6 month intracranially challenged pigs produced positive bioassay results, albeit with low attack rates and variable incubation periods. 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

“1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure.”

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

Location: Virus and Prion Research

Title: Differentiation of scrapie from chronic wasting disease in white-tailed deer

Accomplishments

1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in WTD, has only been recognized since the 1960s. ARS researchers in Ames, Iowa, showed that white-tailed deer sick with scrapie from sheep can infect other deer under conditions mimicking natural exposure. Furthermore, this work shows that CWD is difficult to differentiate from WTD infected with scrapie. WTD scrapie prions accumulate in the lymphoreticular system in a manner similar to CWD, meaning that environmental contamination may occur through feces, saliva, and other body fluids of scrapie affected WTD as has been shown for CWD. The presence of WTD infected with scrapie could confound mitigation efforts for chronic wasting disease. This information informs regulatory officials, the farmed cervid industry, and officials tasked with protecting animal health such as state Departments of Agriculture, Natural Resources, or Parks and Wildlife with regard to a disease similar to CWD but arising from sheep scrapie that could be present in WTD that have contact with scrapie affected sheep and/or goats.

https://www.ars.usda.gov/research/project/?accnNo=440677&fy=202

“1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure.”

TUESDAY, OCTOBER 13, 2026

***> Evidence for multiple prion conformers in natural scrapie isolates

https://scrapie-usa.blogspot.com/2026/10/evidence-for-multiple-prion-conformers.html

https://prpsc.proboards.com/thread/256/evidence-multiple-conformers-natural-scrapie

I am reminded of the ch1641 strain that is like BSE ;

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

Title: ASSESSMENT OF THE GENETIC RISK AND IMPACT OF LATERAL TRANSMISSION IN A VALINE-ASSOCIATED SCRAPIE OUTBREAK IN SHEEP

Author item.gif EVONIUK, J - NORTH DAKOTA STATE UNIV item.gif STOLTENOW, C - NORTH DAKOTA STATE UNIV item.gif O'Rourke, Katherine item.gif MOORE, B - NORTH DAKOTA STATE UNIV item.gif REDMER, D - NORTH DAKOTA STATE UNIV

Submitted to: American Journal of Veterinary Research Publication Type: Peer Reviewed Journal Publication Acceptance Date: 6/8/2005 Publication Date: 8/1/2005 Citation: Evoniuk, J.M., Stoltenow, C.L., O'Rourke, K.I., Moore, B.L., Redmer, D.A. 2005.

Assessment of the genetic risk and impact of lateral transmission in a valine-associated scrapie outbreak in sheep.

American Journal of Veterinary Research. 66(8):1-6. Interpretive

Summary: Scrapie is a fatal neurologic disease of sheep, endemic in the US but the subject of a vigorous eradication program. Scrapie is associated with accumulation of a misfolded form of the sheep prion protein. Control measures in an infected flock include removal of all genetically susceptible sheep. Susceptibility is controlled by naturally occurring differences in the prion protein, designated 171Q (susceptible) or 171R (generally resistant). The resistance of sheep with the 171R form of the gene occurs with one scrapie strain, the most US strain, but these sheep are not protected from the alternative strain, identified as valine associated scrapie. Anecdotal reports have shown that the strain probably occurs in the US but is relatively rare. In this publication, the presence of the valine associated strain was demonstrated through epidemiology and genetic analysis of a large flock of sheep. The study demonstrated that this scrapie strain can infect sheep as adults and is spread efficiently among related and unrelated sheep housed together during lambing seasons. The results suggest that the outbreak was due to the relatively high number of sheep with the susceptible form of the gene, probably introduced through the use of highly prolific rams carrying the gene. Further, transmission of the disease among adults, the prolonged incubation time, the unsuitability of some susceptible sheep to live animal testing, and susceptibility of sheep with the 171R gene suggest that removal of sheep with the genotype AVQR from infected flocks would be prudent.

Technical Abstract: Scrapie is a member of the heterogenous family of transmissible spongiform encephalopathies. Although these disorders appear to be related to misfolding of a host protein rather than an exogenous organism, two distinct disease phenotypes or strains are noted in sheep. Characterization of the scrapie strains in experimental and field settings in the United Kingdom and Europe have included differences in incubation time, survival rates, lateral transmission efficiency and most importantly, genetic susceptibility. Sheep of the prototype strain SSPB/1 are susceptible only if the PRNP (prion protein precursor gene) encodes valine (V) at codon 136; a polymorphism (encoding glutamine to arginine) at codon 171 apparently reduces disease prevalence but increases incubation time and limits tissue distribution to the brain. Sheep with the prototype valine-independent strain CH1641 are susceptible only if homozygous for glutamine (Q) at codon 171. In the US, scrapie is reported primarily in sheep homozygous for 136A/171Q (AAQQ) and the disease phenotype is similar to that seen with experimental strain CH1641. The only genotype useful for strain discrimination is the relatively rare 136AV/171QR. In this study, a flock of sheep with a large number of infected sheep was analyzed. The presence of at least one V136 allele was identified in all but 2 sheep in the study, incubation times were very short in V136 homozygous sheep, probable lateral transmission was demonstrated in 15 sheep, and at least one AVQR sheep was identified. Therefore, this outbreak was probably due to a valine-associated (SSBP/1-like) strain and this report is the first full description of the epidemiology of this strain in US sheep. The high rate of lateral transmission and the susceptibility of 136AV/171QR sheep to scrapie suggest that genotyping for codon 136 may assist management decisions following a scrapie diagnosis and that it may be prudent to remove sheep of the 136AV/171QR genotype from infected flocks.

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

Scrapie TSE Prion, Singeltary review, October 2026

https://scrapie-usa.blogspot.com/2026/10/scrapie-tse-prion-singeltary-review.html

https://prpsc.proboards.com/thread/250/scrapie-prion-singeltary-review-2026

***> Chronic Wasting Disease CWD TSE Prion, Economical, Environmental, Zoonotic, Risk Factors 2026

https://transmissiblespongiformencephalopathy.blogspot.com/2026/02/chronic-wasting-disease-cwd-tse-prion.html

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

***> 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.

Chronic Wasting Disease CWD TSE Prion, Economical, Environmental, Zoonotic, Risk Factors 2026

https://transmissiblespongiformencephalopathy.blogspot.com/2026/02/chronic-wasting-disease-cwd-tse-prion.html

MONDAY, SEPTEMBER 15, 2025

Chronic Wasting Disease CWD TSE Prion Environmental Factors Update

https://prpsc.proboards.com/thread/180/chronic-wasting-disease-environmental-factors

https://chronic-wasting-disease.blogspot.com/2025/09/chronic-wasting-disease-cwd-tse-prion.html

CWD TSE PRION CERVID ENVIRONMENTAL RISK FACTORS 2023

https://www.researchgate.net/publication/375182191_CWD_TSE_PRION_CERVID_ENVIRONMENTAL_RISK_FACTORS_2023

***> i remember what Deep Throat said, now this was around 2001;

“Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at…Okay, you need to know. You don't need to”…snip…see;

https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html

and so it seems…

so, this is what we leave our children and grandchildren?

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/

Front. Vet. Sci., 14 September 2015 | https://doi.org/10.3389/fvets.2015.00032

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination.

http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full

"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

Prion 2023 Abstracts

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

SUNDAY, APRIL 06, 2025

Failure to prevent classical scrapie after repeated decontamination of a barn

https://scrapie-usa.blogspot.com/2025/04/failure-to-prevent-classical-scrapie.html

https://prpsc.proboards.com/thread/165/failure-prevent-scrapie-repeated-decontamination

***> what about long term investment and property real estate evaluations?

CWD, So, this is what we leave our children and grandchildren?

THURSDAY, SEPTEMBER 24, 2026

Looming Crisis U. S. Transmissible Spongiform Encephalopathy TSE Prion, in Cattle, Cervid, Sheep, Pigs, and Humans

https://transmissiblespongiformencephalopathy.blogspot.com/2026/09/looming-crisis-u-s-transmissible.html

https://prpsc.proboards.com/thread/246/looming-crisis-prion-cattle-cervid

SUNDAY, AUGUST 09, 2026

Chronic Wasting Disease CWD and Population Declines of Cervid

https://chronic-wasting-disease.blogspot.com/2026/08/chronic-wasting-disease-cwd-and.html

https://prpsc.proboards.com/thread/229/cwd-prion-population-declines-cervid

***> JUST TO BE PERFECTLY CLEAR, me, myself, and i, don’t believe, that 85%+ of all Human TSE Prion disease ie sporadic/spontaneous Creutzfeldt Jakob Disease sCJD, is a happenstance of bad luck, from a bad fold of the PrP ie PrPC to PrPSc, that dog don’t hunt for me, never did, ie the UKBSEnvCJD only theory/conspiracy/lie, take your pick, that 300k or so, BSE TSE Mad Cows, ONLY IN YOUNG ONLY TEENAGERS, ONLY IN THE UK, and that everything else was sporadic/spontaneous, no mad cows in the US, CWD TSE PrP never transmitted to nothing, it’s only in the brain, yada yada yada, rubber stamped BS. hell, all iatrogenic CJD has been, is Sporadic CJD, until the iatrogenic event is discovered, traced back, investigated, confirmed, studied, run through some mice or primates, and studied and studied again, peer reviewed, placed into the academic journals, and then maybe, finally, put into the public mainstream media. nobody talks about or seems to be concerned with VPSPr sCJD, and sCJD cases keep increasing, year after year after year, decade after decade, just better surveillance, lol, or the infamous new ‘prion like mechanism neurological diseases’ popping up everywhere, what the hell is that, new ‘prion like mechanism neurological diseases’, can they transmit or not? oh, i could go on…im just thinking out loud…terry

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

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

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

WEDNESDAY, MAY 29, 2019

***> Incomplete inactivation of atypical scrapie following recommended autoclave decontamination procedures

https://nor-98.blogspot.com/2019/05/incomplete-inactivation-of-atypical.html

THURSDAY, DECEMBER 31, 2020

Autoclave treatment of the classical scrapie agent US No. 13-7 and experimental inoculation to susceptible VRQ/ARQ sheep via the oral route results in decreased transmission efficiency

https://scrapie-usa.blogspot.com/2020/12/autoclave-treatment-of-classical.html

Snip…see full text;

https://nor-98.blogspot.com/2021/01/atypical-nor-98-scrapie-tse-prion-usa.html

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

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

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

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

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

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

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

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

https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032

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

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

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

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

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

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

PRION 2015 CONFERENCE

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

PRION 2016 TOKYO

Saturday, April 23, 2016

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

Prion. 10:S15-S21. 2016 ISSN: 1933-68961933-690X

WS-01: Prion diseases in animals and zoonotic potential

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

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

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

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

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

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

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

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

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

Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014)

Ru G1 ., Pocchiari M2 ., Bertolini S. 1, Pite L.1 , Puopolo M.2 , Ladogana A.2 , Perrotta M.G.3 , Meloni D 1 . (1) National reference center for the study and research on animal encephalopathies and comparative neuropathologies (CEA). Experimental Zooprophylactic Institute of Piemonte, Liguria and Valle d'Aosta, Torino, Italy.

(2) Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanità, Roma, Italy. (3) Office 3 National center for the fight and emergency against animal diseases. Ministry of Health, Roma, Italy.

Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure.

Materials and Methods: National data from prion disease surveillance in humans (sporadic CJD) and small ruminants (CS) in Italy were utilized. A descriptive geographic analysis was conducted for each disease individually. Subsequently, an ecological study was performed to compare the occurrence of both diseases at the district and regional levels. Standardized incidence ratios (SIR), adjusted for confounders, were calculated for CJD and CS by district and region, respectively, representing the outcome and proxy of exposure. Considering a possible long incubation period of CJD, two study periods were analysed: 2010-2014 for CJD and 2002-2006 for CS. Eight alternative linear regression models were developed using SIR in humans as the dependent variable and SIR in sheep as the independent variable. These models varied in the scale of SIR data (continuous vs. categorical), geographical level (district vs. region), and the potential past exposure of sheep in specific areas to a known source of infection (via a contaminated vaccine).

Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased.

Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.

Funded by: Italian Ministry of Health Grant number: Realizzazione del programma epidemiologico finalizzato a dare evidenza del potenziale zoonotico delle TSE animali diverse dalla BSE. Prot. N. 0018730-17/07/2015-DGSAFCOD_UO-P

''Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.''

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

=====

Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (Tg338 and Tg501).

Enric Vidal1,2, Samanta Giler1,2, Montse Ordóñez1,2, Hasier Eraña3,4, Jorge M. Charco3,4, Guillermo Cantero1,2, Juan C. Espinosa5 , Juan M. Torres5 , Vincent Béringue6 , Martí Pumarola7 and Joaquín Castilla3,8,9

Aims: About 90% of Creutzfeldt-Jakob disease cases are classified as sporadic (sCJD), that is, occur infrequently, randomly and without a known cause. It is a fatal neurodegenerative disease with an incidence of 1-1.5 cases per million per year. Epidemiological studies have been so far unable to establish a causal relationship between sCJD and prion diseases in animals.

The zoonotic potential of sheep scrapie was demonstrated in 2014 (Cassard et al., Nature Communications) through inoculation of transgenic mice overexpressing the human prion protein with scrapie isolates. The resulting prion disease was indistinguishable from that occurring after sCJD inoculation in the same model and, while these results do not demonstrate that sCJD is caused by scrapie prions, they do show that the transmission barrier between ovine and human prions is not absolute. Our aim is to further assess this zoonotic risk.

Materials and methods: we have prepared inocula from 3 sCJD cases (MM1, MV2 and VV2) and 2 VPSPr cases (MM and MV) to verify if it is possible to recover the scrapie phenotype upon inoculation in Tg338 and Tg501 ovinized mouse models. Additionally, two different inocula gCJD (E200K) and GSS (A117V) have been also included in the bioassays as controls for classical and atypical genetic human prions, respectively.

Results: No evidence of transmission was found on a first passage in Tg338 nor Tg501 ovinized mice, but on second passage, 4/10 Tg338 mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and 1/12Tg501 mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed.

Funded by: MINECO grant number AGL2017-88535-P and PID2021-1222010B-C22 and by Interreg POCTEFA grant number EFA148/16 (RedPRION)

''but on second passage, 4/10 Tg338 mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and 1/12 Tg501 mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed.''

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

THURSDAY, JUNE 12, 2025

***> Redefining the zoonotic potential of chronic wasting disease Singeltary Review

https://chronic-wasting-disease.blogspot.com/2025/06/redefining-zoonotic-potential-of.html

WEDNESDAY, MAY 27, 2026

***> Limited transmission of cervid prions to nonhuman primates provides insights into the zoonotic potential of chronic wasting disease

https://www.science.org/doi/10.1126/sciadv.aeb7613

https://chronic-wasting-disease.blogspot.com/2026/05/limited-transmission-of-cervid-prions.html

https://prpsc.proboards.com/thread/220/limited-transmission-cervid-nonhuman-primates

***> Cervid CWD TSE PrP Transmission to Humans, has it already happened and being masked as sporadic CJD, What if? July 2026 Review <***

https://chronic-wasting-disease.blogspot.com/2026/07/cervid-cwd-tse-prp-transmission-to.html

https://prpsc.proboards.com/thread/224/cervid-transmission-humans-july-review

MONDAY, JUNE 1, 2026

NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD SURVEILLANCE TABLES OF CASES EXAMINED NPDPSC Last updated March 31st, 2026

https://prionunitusaupdate.blogspot.com/2026/06/national-prion-disease-pathology.html

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025

From the first full year of reporting CJD TSE in the US in 2000, where 90 cases of CJD was reported that year, to today, where in September 2025, the number of CJD cases reported in the last full year reporting, which would have been 2024, the number of CJD cases for 2024 was 249 cases. So, from the first full year 2000 CJD cases were 90 cases confirmed in that year, to 2024, where 2024 CJD statistics rose to 249 confirmed CJD cases in a single year. A dramatic increase in deaths, from figures that don’t seem to be dramatic. But thes figures today, they are not from “better surveillance”, that dog don’t hunt no more. They have been saying this for over 25 years, year after year, well it’s time to call it for what it is, Human Transmissible Spongiform Encephalopathy TSE Prion cases are rising, and it’s NOT because of better surveillance, or just a “happenstance of bad luck, that 85%+ of all human cases, sporadic CJD, including VPSPr, just happen spontaneously, no, it’s because of unknown environmental factors, and or iatrogenic factors, imho…terry

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025

https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html

WEDNESDAY, SEPTEMBER 2, 2026

A new chapter for CWRU’s Prion Center

https://prionunitusaupdate.blogspot.com/2026/09/a-new-chapter-for-cwrus-prion-center.html

https://prpsc.proboards.com/thread/239/new-chapter-cwru-prion-center

Terry S. Singeltary Sr.