SCRAPIE USA

Transmissible Spongiform Encephalopathy TSE Prion PrP sheep and goats

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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Tuesday, February 08, 2022

Romania Outbreaks of scrapie in two sheep farms 750 animals are affected

Romania Outbreaks of scrapie in two sheep farms 750 animals are affected

Outbreaks of scrapie in two sheep farms! 750 animals are affected!

Angelica Lefter -February 4, 2022 10:44 AM Outbreaks of scrapie 
in two sheep farms! Authorities have also placed restrictions on farms in Bocsig commune, Arad county, which total 750 sheep.

The disease was detected in several animals in which the first tests were positive for scrapie. A second set of samples is currently being processed, before deciding on the possible slaughter of the animals, according to aradon.ro. A second set of evidence is expected to be confirmed

The first animals had specific symptoms a few days ago, and the samples were sent to the Institute of Diagnosis and Animal Health (IDSA) Bucharest, as mentioned by the Director of the Veterinary and Food Safety Directorate of Arad, Marcel Roşu.

"The first test performed was positive, but in such cases a second test is made, more sensitive, to confirm the disease. For the time being, the farms have been put under surveillance and restrictions, and if the final result confirms that the sheep have scrapie, IDSA will decide on the action plan to be implemented. It is possible to slaughter sheep even for consumption, but it can also be decided to kill and neutralize ", said Marcel Roşu.

He explained that scrapie is a fatal degenerative disease that affects the central nervous system of sheep and goats. The disease is similar to mad cow disease. The first symptoms of sheep scrapie

According to the National Sanitary Veterinary and Food Safety Authority (ANSVSA), scrapie is caused by an infectious agent that "appears to be transmitted from sheep to lambs and from goats to goats, as well as to other sheep or goats."

"The disease can also occur from the accidental consumption of animal protein meal by sheep and goats - contaminated feed supplements, animal protein minerals, meat meal, bones, blood, organs resulting from the processing of carcasses, products and by-products. of infected cattle, sheep, goats ”, is shown in the practical guide for farmers from ANSVSA.

Animal breeders may notice symptoms and suspect any animal that has had a nervous or behavioral disorder. "Any living or slaughtered or dead animal showing or showing a nervous or behavioral disorder or a progressive deterioration of the general condition associated with a deterioration of the central nervous system and for which the information gathered on the basis of clinical examination, response to treatment, examination post-mortem or ante-mortem or post-mortem laboratory tests do not allow the establishment of an alternative diagnosis ", the guide also states.

Sheep farms in Romania have been dealing with scrapie since 2002, when it was officially confirmed by the Institute of Diagnosis and Animal Health. Informaţiile publicate de Agrointeligența – AGROINTEL.RO pot fi preluate doar în limita a 500 de caractere şi cu citarea în PRIMUL PARAGRAF a sursei cu LINK ACTIV. Orice abatere de la această regulă constituie o încălcare a Legii 8/1996 privind dreptul de autor, ca atare vom acționa în consecință.



AGRICULTURAL NEWS

Cases of scrapie in sheep in Romania. What are the signs of the disease?

Stefania Zavod IstrateStefania Zavod IstrateAPRIL 19, 2021

Cases of scrapie in sheep in Romania. What are the signs of the disease?Cases of scrapie in sheep in Romania. What are the signs of the disease?

ShariShari The President of the National Sanitary Veterinary and Food Safety Authority announced the presence of outbreaks of scrapie in sheep and goats in three counties in Romania. Several sheep farmers have lost their entire herd after buying scrapie rams from fairs.

Thus, many of them ended up signaling on social networks signs of the disease that could exempt buyers from sacrificing the entire herd.

In 2021, outbreaks have already been reported in three counties

The president of ANSVSA stated, during the show Forza Fermierilor, that in 2020 there were 15 outbreaks in 6 counties, with 53 cases diagnosed in sheep and 5 in goats. So far, 3 outbreaks have already been reported this year in Mureș, Cluj and Vaslui counties, with 4 cases diagnosed in sheep and one in goats.

Outbreaks appear to be exacerbated during the first half of the year, according to experts. Thus, the damage to breeders is enormous. That is why some of the most important actions of the shepherds are those of prevention. One of these preventative measures is genotyping, with the aim of raising only animals that are resistant to this disease.

Signs of scrapie in sheep

The official name of scrapie is spongiform encephalopathy, a communicable disease of sheep and goats over 18 months. Scrapie affects the central nervous system of animals, and is popularly known as "crazy sheep disease." The disease is associated with the presence of abnormal proteins. Scrapie is clinically manifested by depression, uncoordinated gait, severe itching, and death. Among the first symptoms of the disease are lack of appetite, weight loss and gnashing of teeth. The disease can be transmitted directly, most often from mother to lamb, but there are other possibilities, such as contaminated feed, water or pasture.

Experts say that a sheep can transmit the disease to several lambs until it shows signs. In addition, another form of transmission is from one herd to another, especially when infected young sheep come in contact with healthy ones before they have any symptoms.

The most important methods of prevention are related to genotyping , but also to the attention paid to the import of animals from the affected regions. After the report of the disease, the shepherds can no longer raise or sell sheep for three years in the respective farm, being subject to the third degree quarantine.

Mandatory measures to report the disease Farmers are obliged to act when they have reported the occurrence of the disease, by the following measures:

quarantine of the whole herd, killing and incineration first of sick animals and then of the whole herd

garbage, as well as utensils used, are subjected to rigorous disinfection and destruction, including by burning

shelters and metal inventory are repeatedly disinfected

if these measures cannot be applied correctly, sick animals may be slaughtered and incinerated, traffic may be restricted and contact with other herds, especially free herds, may be avoided. , fetal coverings are harvested immediately and burned or buried deep).

in herds with confirmed cases, the breeding will be stopped, so that all the contaminated herd will be eliminated in the shortest period of time, avoiding the transmission of the disease to other generations through the contaminated calves

If scrapie has been found in a herd, the entire herd will be slaughtered. The sheep farmer will only be compensated if the animals have been registered with the Sanitary Veterinary Directorate.


SCRAPIE, DEADLY SHEEP DISEASE, AT A SHEEP FARM IN HUNEDOARA! 

Agroinfo Posted: July 28, 2019 - 12:27 PM 

DISEASE WITHOUT HEALING THAT CAUSES GREAT DAMAGE TO SHEEP! 

An outbreak of scrapie was found on a sheep farm in Hunedoara County. The disease is deadly, untreated, and the herd in which a case of scrapie is found could be slaughtered in its entirety to stop the spread of the disease. There is a scrapie epidemic in Romania. Last year, several herds from several counties were liquidated.

There has been an outbreak of scrap for some time, identified by an owner in the area, but know that it is properly managed, the animals have been isolated. It is not sold, it is not bought from there, properly identified, it is directed to the slaughterhouse, it is slaughtered, the hearth will be extinguished in the shortest time , said Dr. Radu Iordache, director of the Sanitary Veterinary and Food Safety Directorate, DSVSA Hunedoara, for the Hunedoara village.

There is a national program to control scrapie, a deadly disease for sheep without a cure, which is causing great harm to sheep farmers.

This program is doubled by one with strict rules, adds the director of DSVSA Hunedoara. There are well-founded and implemented measures to liquidate the herd and extinguish the outbreak , he added.

Scrapie is a disease that the Romanian national veterinary authorities are almost silencing. Meanwhile, this misfortune is spreading from one farm to another. Many of the shepherds complained that the so-called rams of origin, with certificates of origin, contaminated with scrapie, had been sold and that this was how the disease had spread. What is certain is that last year several herds were liquidated due to scrapie. In one county, a large sheep farm in Paleu commune, Uileacul de Munte village, was liquidated. A herd of 2,000 sheep was taken to the slaughterhouse.

Now, it is officially recognized that there is an outbreak of scrapie in Hunedoara County. For a long time, several sheep breeders have been accusing in the public space that scrapie has spread mainly from this county where most purebred rams have been sold. It is possible, but no veterinary authority confirms that this is how the misfortune spread to the sheepfolds.

We remind you of essential information about this disease, extracted from ANSVSA documents:

CAREFUL! SCRAPIA IS A MANDATORY DISEASE, ie ANY SUSPICION MUST BE REPORTED AS SOON AS IT WAS OBSERVED. It is important to report any suspicious animals at all times. Even if you are not sure if the animal is infected, tell your veterinarian. There is no problem if the scrapie is not confirmed, even in case of a false alarm it is important that you have notified the veterinarian , warns ANSVSA.

WHAT IS SCRAPIA?

Scrapie is a degenerative and fatal disease that affects the central nervous system of sheep and goats. The disease is associated with the presence of abnormal proteins called prions and is part of a group of neurological conditions known as transmissible spongiform encephalopathies.

The occurrence of this disease and its increased degree of danger to livestock in operation led to the establishment of legislation to control the disease and stop its spread.

In Romania, the legal basis is Regulation (EC) no. Regulation (EC) No 999/2001 of the European Parliament and of the Council of 22 May 2001 laying down rules for the prevention, control and eradication of certain transmissible spongiform encephalopathies.

Scrapia has been known in the world for over 200 years, but in Romania it has been present only since 2002, when it was officially confirmed by LNR-EST within the Institute of Diagnosis and Animal Health.

ARE COMPENSATIONS GRANTED? YES! Owners shall receive compensation for the loss of slaughtered animals or products of animal origin destroyed if the presence of a form of TSE is suspected or officially confirmed. The application of disease control and eradication measures provides compensation to owners of animals for slaughtered, killed or otherwise affected animals, in addition to insurance and payments recovered by recovering products and by-products from slaughtered, dead or killed animals, in accordance with the law. The authorized free practice veterinarian who carries out his activity in your area will guide you on the procedure to be followed, in accordance with the legal provisions in force.

WHAT IS GENOTYPING AND HOW CAN IT HELP YOU?

There are studies that have shown that there is a gene in sheep that determines whether the animal can develop the disease when exposed to infection. Some sheep, if exposed to scrapie, may develop the disease unlike others. Goats are more uniformly susceptible, but the same gene can control the time until clinical signs appear.

It is possible to identify a sheep / goat that is resistant or susceptible to scrapie by performing a test on a blood sample taken from a sheep / goat. This test is called genotyping, and the result is the genotype of the animal.

This makes it possible to reduce the number of sick animals in a herd by selecting animals for breeding in order to obtain scrap-resistant products.

If you want to know which of your sheep or goats is resistant to scrapie, consult your authorized free practice veterinarian who will guide you in the procedure to be followed in accordance with the legal provisions in force.

Is there an active surveillance program for EST / Scrapie?

YES! In accordance with the provisions of Regulation 999/2001, Romania is obliged to test by laboratory examinations a certain number of sheep and goats slaughtered for human consumption, on brain samples collected from them. These samples must be collected from slaughterhouses. The same regulation stipulates that Romania is obliged to test by laboratory examinations a certain number of dead or suspicious sheep and goats. over 18 months of age, on brain samples taken from them. These samples must be collected directly from the farm or household, says ANSVSA in a document published on its official website.

Romanian farmers should know that they risk prison sentences and huge fines if they do not notify the veterinarian that they have an animal suspected of scrapie on the farm, according to Regulation (EC) no. 999/2001.


The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2018


P.76: Evaluating the effectiveness of genetic screening for the identification and classification of increasing sheep resistance in Romania

Florica Barbuceanu, 1 Cristina Diaconu, 1 Stefania Mariana Raita, 2 Daniela Stamate, 1 Constantin Lupescu, 1 and Gabriel Predoi 2 1 Morphopathology Department; Institute for Diagnosis and Animal Health; Bucharest, Romania; 2

Preclinical Sciences Department; Faculty of Veterinary Medicine; University of Agricultural Sciences and Veterinary Medicine; Bucharest, Romania

Keywords: haplotypes, scrapie resistance, sheep, breeding program

Introduction. Following Regulation (EC) no. 999/2001 of the European Parliament and of the Council laying down rules for the prevention, control and eradication of certain transmissible spongiform encephalopathies, that include intensive genetic selection for scrapie resistance, our pilot study is focused on evaluating the effectiveness of such screening for the identification and classification of sheep resistance to scrapie in Romania. 

Material and Methods. Study was conducted in two selected farms for a period of two years, without significantly reducing the number of animals and consequently avoids economic losses. A total number of 2515 sheep (1280 sheep from the first farm and 1235 sheep from the second) were examined for codons 136, 154 and 171 polymorphisms in order to identify a convenient and applicable method to improve the sheep population resistance to scrapie. 

Results. Five haplotypes (ARR, AHQ, ARH, ARQ and VRQ) were observed in this study. Of the total number sheep being analyzed, 421 were classified as category 1 (resistant to scrapie homozygous form), 1045 in category 2 (resistant to scrapie heterozygous form), 884 in category 3 (medium susceptibility Prion Diseases in Animals 60 Prion Volume 8 Supplement to scrapie), 65 in category 4 (genetically resistant, but with VRQ haplotype) and 100 in category 5 (highly susceptible – at least one VRQ haplotype). 

Conclusion. For the following breeding season, our recommendation is to remove the scrapie susceptible categories 3, 4 and 5 from the breeding program. This study provided valuable information on future actions to improve genetic resistance scrapie in sheep.


INCREASING FOOD CHAIN SECURITY FOR SCRAPIE BY MARKER ASSISTED SELECTION IN SHEEP POPULATIONS CREŞTEREA SECURITĂŢII LANŢULUI ALIMENTAR PENTRU SCRAPIE PRIN SELECŢIA ASISTATĂ DE MARKERI MOLECULARI ÎN POPULAŢIILE DE OVINE

Results and Discussions

In1996, research performed at INRA, on 600 Romanov sheep, demonstrated the role of different alleles in genetic determinism of resistance/sensitivity to scrapie. During 3 years, the analyses revealed 250 cases of scrapie. The study led to the following results: - animals with codone 154 codifying Hystidine (H) and codone 171 codifying Arginine (R) on Prn-p gene, are resistant to scrapie; - animals with codone 154 for Arginine (H) and codone 171 for Glutamine (Q) on Prn-p gene, are sensitive in homozygous state, but this sensitivity increases if codone 136 codifies Valine (V), not Alanine (A). In the same year, INRA (Sarradin P. et al., 1997) put the basis of a prototype genetic selection pattern for scrapie resistance. During 5 years (1996-2001) only rams of at least one copy of ARR allele carriers were used for reproduction. The share of ARQ/ARQ sensitive rams decreases from 24% to 7%, while share of entirely scrapie resistant ARR/ARR rams increases from 28% to 46%. The rams carriers of hypersensitive scrapie alleles were totally eliminated. Within a recent epidemiologic study, 1,587 cases of sheep with scrapie (Le Dur A., 2005) confirmed by hystopathologic examination, were analyzed. No carrier of ARR/ARR genotype was found, while 13.7% of individuals with scrapie were carriers of VRQ/VRQ genotype. 

https://citeseerx.ist.psu.edu/viewdoc/download;jsessionid=21EFB1F00AF1E0CA1E8C6AB342A3E014?doi=10.1.1.459.4282&rep=rep1&type=pdf

Bulletin UASVM, Veterinary Medicine 66(1)/2009 ISSN 1843-5270; Electronic ISSN 1843-5378

Correlation between Results of Rapid Tests and Histopathological Examination in Scrapie in Sheep

Cornelia Aurica BIRO*, Mariana TULEA* Directia Sanitara Veterinara si pentru Siguranta Alimentelor Hunedoara- Laboratorul Sanitar Veterinar de Stat, Morfopatologie

Abstract. During 2004-2008, in Hunedoara County, have been found positive for scrapie testing by a rapid 20 indigenous sheep (17 females and 3 males) from Turcan race, age between 2 and 7 years, for various categories of interest for transmissible spongiform encephalopathies (normal slaughter, emergency slaughter and death) (9, 10, 11, 14).

All of them were examined by histopathological examination of the bulb rahidian by the method of coloring Hematoxilina-Eozina, a total of 10 sheep, which were evident morfopathologic lesions specific scrapie.

All these samples were confirmed positive by confirmatory tests for transmissible spongiform encephalopathies in the national reference laboratory for transmissible spongiform encephalopathies of the Institute of Diagnosis and Animal Health Bucharest.

Keywords: scrapie, sheep, TSE 


ROMANIA SELF-DECLARATION OF SCRAPIE FREE ZONES STATUS (LOL)



OIE SCRAPIE FREE STATUS


According to the OIE Bulletin No. 3/2013, published on 16 October, Romania has declared as free from scrapie for 19 counties. Although the information sources indicate the presence of disease in the world for over 200 years, scrapie has been officially confirmed in Romania in 2002, by the Institute of Diagnosis and Animal Health, only in one case (Regulation (EC) no. 999/2001/). 


SANTE DATA COLLECTION PLATFORM Output on: 2021/02/01 16:36 (Europe/Luxembourg) Status: closed (submitted) Created: 2020/03/28 11:55 Last updated: 2020/04/07 16:27

Eradication: Final report for Transmissible Spongiform Encephalopathies 2019

For each approved annual or multi-annual programme Member States shall submit to the Commission by the 30 April each year an annual detailed technical and financial report covering the previous year. That report shall include the results achieved and a detailed account of eligible costs incurred (Art 14 of Regulation (EU) No 652/2014). 

Since 2001 scrapie has also been notifiable disease in Hungary based on the same legislation as BSE. Many years before scrapie became notifiable disease it had already been compulsory to report each ruminant showing neurological signs as it was mentioned before. The first scrapie case occurred in 1964 at an import quarantine station among imported sheep. 

***> The second case was confirmed in January 2005 in a sheep imported from Romania for immediate slaughter. 


The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2020

European Food Safety Authority (EFSA)

First published: 30 November 2021 https://doi.org/10.2903/j.efsa.2021.6934

Requestor: European Commission

Question number: EFSA-Q-2020-00789

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In total, 453,194 small ruminants were tested in 2020 in the EU27 and the UK: 332,579 sheep (a 1.6% decrease from 2019) and 120,615 goats (a 16% decrease). In addition, 26,053 sheep were tested by four of the seven other non-EU, namely Iceland, North Macedonia, Norway and Serbia, and 712 goats by three, namely, Iceland, North Macedonia and Norway.

In sheep, 688 scrapie cases were reported in the EU27 and the UK in 2020, 309 less cases than in 2019. In total, 65 cases of scrapie in sheep were reported by two (Iceland and Norway) of the four other non-EU that tested sheep. Classical scrapie (CS) was reported by seven MS and one other non-EU, namely Bulgaria, Cyprus, Greece, Italy, Portugal, Romania, Spain and Iceland; whereas atypical scrapie (AS) was reported by 14 MS, namely Belgium, Finland, France, Germany, Greece, Hungary, Ireland, Italy, Poland, Portugal, Romania, Slovakia, Spain, Sweden; plus the United Kingdom and other non-EU (Norway). An additional total of 12 cases in sheep were reported as inconclusive by Italy, which are not included in the total scrapie caseload for this country. Most of the ovine cases in the EU27 and the UK (97.4%) were reported by four countries: Greece, Italy, Romania and Spain, as it was the case in previous years.

In total, 589 ovine cases in the EU27 and the UK were CS (85.7%), 98 were AS (14.3%) and Italy reported one CH1641-like case. Among the other non-EU, 53 CS cases were reported by Iceland and 12 AS cases by Norway. In sheep, 24.6% (169) of all cases in the EU27 and the UK reported in 2020 were index cases (IC), with a much higher proportion in AS cases (89.8%) compared with CS cases (13.8%). In total, 97% of the CS cases in sheep reported in 2020 with known National Scrapie Plan (NSP) genotypes belonged to animals holding genotypes of the susceptible groups (NSP3, NSP3O, NSP4 or NSP5). One CS case was reported by Romania in a sheep carrying the ARR/ARR genotype, a very rare occurrence.

In goats, in total, 328 scrapie cases were reported in the EU27 and the UK: nine were AS cases and 319 CS (97.3 with Cyprus accounting for 74% of these). Compared with 2019, when 379 CS cases were reported, there was a 15.8% reduction (−60) mainly due to the situation in Cyprus that has improved continuously over the last 7 years. Four MS (Bulgaria, Cyprus, Romania, Greece) and the UK reported CS, whereas three MS (Denmark, France, and Portugal) reported AS. Italy and Spain reported both CS and AS. The three other non-EU (Iceland, Serbia and Norway) that reported tested goats did not report any scrapie cases.

In goats, 18.6% of all cases reported in the EU27 and the UK in 2020 were IC, higher than in 2019 (8.7%), with a higher proportion in AS (100%) than in CS (16.3%).

CS is still the most frequently reported type of scrapie in the EU in both species.

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P.04: Conduct of diagnosis in the case of occurrence of bovine spongiform encephalopathy in Romania 

Florica Barbuceanu1, Marion Simmons2, Melanie Chaplin2, Cristina Diaconu1, S Nicolae1, Stefania Raita3, C Belu3 and G Predoi3

1Institute for Diagnosis and Animal Health; Bucharest, Romania

2Animal and Plant Health Agency; EU-RL-TSE; Weybridge, UK

3University of Agronomic Sciences and Veterinary Medicine of Bucharest - Faculty of Veterinary Medicine; Bucharest, Romania

Bovine spongiform encephalopathy (BSE) is a fatal infectious disease neurodegenerative, caused by prions that affects mainly cattle, characterized by spongiform and vacuolar changes in the central nervous system (CNS). Although in the last period of time, the appearance of the classic cases of BSE had fallen, is the appearance of atypical BSE forms, in many countries of Europe, USA, Japan and Canada. The paper presents the conduct of diagnosis for the first cases of BSE in Romania, reported on the basis of laboratory investigations, the rapid test positive to bovine animals slaughtered normally diagnosed after confirmatory tests carried out by the National Reference Laboratory for Transmissible Spongiform Encephalopathies (NRL-TSEs) within the Institute for Diagnosis and Animal Health (IDAH). After carrying out tests for the differentiation of strains of BSE samples sent to the European Union Reference Laboratory for the Transmissible Spongiform Encephalopathies (EU-RL-TSE) from the Animal and Plant Health Agency (APHA) Weybridge, United Kingdom, were diagnosed 2 cases of atypical BSE, type L.


SUNDAY, JUNE 25, 2017 PRION 2017 

P124 The Present Romanian Epidemiological Context With Regard To The Bovine Spongiform Encephalopathy


Many times media portrays atypical BSE strains as a spontaneous or sporadic event caused by old age. Sciences has shown us otherwise. All atypical BSE cases are not sporadic/spontaneous, OIE has recognized this. Atypical BSE is a risk factor for feed, science has shown us this, we must now recognize this risk factor in the FDA 589.2001 BSE feed regulatory system.

what does the oie now say about atypical BSE;

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. 


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

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REFERENCES

SNIP...END SEE FULL TEXT;


TUESDAY, SEPTEMBER 07, 2021 

Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom


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

***> 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. 

***> 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.

Atypical L-type BSE

Emerg Infect Dis. 2017 Feb; 23(2): 284–287. doi: 10.3201/eid2302.161416 PMCID: PMC5324790 PMID: 28098532

Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle 


Our study clearly confirms, experimentally, the potential risk for interspecies oral transmission of the agent of L-BSE. In our model, this risk appears higher than that for the agent of classical BSE, which could only be transmitted to mouse lemurs after a first passage in macaques (14). We report oral transmission of the L-BSE agent in young and adult primates. Transmission by the IC route has also been reported in young macaques (6,7). A previous study of L-BSE in transgenic mice expressing human PrP suggested an absence of any transmission barrier between cattle and humans for this particular strain of the agent of BSE, in contrast to findings for the agent of classical BSE (9). 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.


Atypical H-type BSE

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

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

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.


P98 The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challenge 

Greenlee JJ (1), Moore SJ (1), and West Greenlee MH (2) (1) United States Department of Agriculture, Agricultural Research Service, National Animal Disease Center, Virus and Prion Research Unit, Ames, IA, United States (2) Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, United States. 

With the experiment currently at 55 months post-inoculation, no other cattle in this study have developed clinical signs suggestive of prion disease. This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. 

These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains. 

PRION CONFERENCE 2018 CONFERENCE ABSTRACT

Published: 23 June 2011

Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

The present study demonstrated successful intraspecies transmission of H-type BSE to cattle and the distribution and immunolabeling patterns of PrPSc in the brain of the H-type BSE-challenged cattle. TSE agent virulence can be minimally defined by oral transmission of different TSE agents (C-type, L-type, and H-type BSE agents) [59]. Oral transmission studies with H-type BSE-infected cattle have been initiated and are underway to provide information regarding the extent of similarity in the immunohistochemical and molecular features before and after transmission. In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.

References...END


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

PLEASE NOTE;

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

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. 


''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.'' 

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

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

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

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations 
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France 

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

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

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

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

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

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

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

=============== 

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

=============== 

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

============== 

https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf 

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

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

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

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

PRION 2016 TOKYO

Saturday, April 23, 2016

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

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

Taylor & Francis

Prion 2016 Animal Prion Disease Workshop Abstracts

WS-01: Prion diseases in animals and zoonotic potential

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

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

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

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

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

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

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

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

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

snip...

The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

PMID: 6997404


Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6


Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).


TUESDAY, SEPTEMBER 07, 2021 

Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom


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

ATYPICAL SCRAPIE ROUGHLY HAS 50 50 CHANCE ATYPICAL SCRAPIE IS CONTAGIOUS, AS NON-CONTAGIOUS, TAKE YOUR PICK, BUT I SAID IT LONG AGO WHEN USDA OIE ET AL MADE ATYPICAL SCRAPIE A LEGAL TRADING COMODITY, I SAID YOUR PUTTING THE CART BEFORE THE HORSE, AND THAT'S EXACTLY WHAT THEY DID, and it's called in Texas, TEXAS TSE PRION HOLDEM POKER, WHO'S ALL IN $$$

THURSDAY, JULY 8, 2021

EFSA Scientific report on the analysis of the 2‐year compulsory intensified monitoring of atypical scrapie


MONDAY, JUNE 28, 2021 

BSE can propagate in sheep co‑infected or pre‑infected with scrapie


FRIDAY, DECEMBER 10, 2021 

USDA APHIS National Scrapie Eradication Program October 2021 Monthly Report Fiscal Year 2022


WEDNESDAY, DECEMBER 8, 2021 

Importation of Sheep, Goats, and Certain Other Ruminants AGENCY: Animal APHIA, USDA, FINAL RULE [Docket No. APHIS–2009–0095] RIN 0579–AD10


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


WEDNESDAY, MAY 29, 2019 

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

USDA HERE'S YOUR SIGN!


FRIDAY, DECEMBER 10, 2021 

USDA APHIS National Scrapie Eradication Program October 2021 Monthly Report Fiscal Year 2022




Terry S. Singeltary Sr.