Epidemiological comments
On July 5th, 2019, the Central Veterinary Laboratory in Algete (National Reference Laboratory for TSEs, accredited under UNE-EN ISO/IEC 17025:2005 standard) received a nerve tissue sample suspected to be infected by bovine spongiform encephalopathy (BSE) from the accredited Regional Laboratory for Animal Health in Lugo, Galicia (official regional laboratory), after a positive result was obtained through a HerdChek BSE-Scrapie Antigen Test Kit (Idexx Laboratories) rapid test.
The National Reference Laboratory carried out the confirmation tests authorized according to EU Regulation No. 1148/2014. The selected combined tests were ELISA, carried out by the regional laboratory (HerdChek), and Prionics Check Western Blot, and positive results were obtained for both tests. Afterwards, tests for BSE strain discrimination were carried out through hybrid immunoblotting with antibodies against PrP, confirming atypical BSE (H type strain).
The sample was taken within the national TSE surveillance program (sampling of dead or non-slaughtered for human consumption animals over 48 months old). The animal was a crossbred cow born on January 30th, 2001.
Bovine spongiform encephalopathy , Spain
Information received on 15/07/2019 from Dr Valentín Almansa, Director General, Sanidad de la Produccion Agraria, Ministerio de Agricultura, Alimentación y Medio Ambiente, Madrid, Spain
Summary
| Report type | Immediate notification (Final report) |
| Date of start of the event | 03/07/2019 |
| Date of confirmation of the event | 10/07/2019 |
| Report date | 15/07/2019 |
| Date submitted to OIE | 16/07/2019 |
| Date event resolved | 16/07/2019 |
| Reason for notification | Recurrence of a listed disease |
| Date of previous occurrence | 19/02/2019 |
| Manifestation of disease | Clinical disease |
| Causal agent | Prion (atypical BSE type H) |
| Nature of diagnosis | Laboratory (advanced) |
| This event pertains to | a defined zone within the country |
New outbreaks
| Summary of outbreaks | Total outbreaks: 1 | ||||||||||||
| Outbreak Location |
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| Total animals affected |
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| Outbreak statistics |
* Removed from the susceptible population through death, destruction and/or slaughter; |
Epidemiology
| Source of the outbreak(s) or origin of infection |
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| Epidemiological comments | On July 5th, 2019, the Central Veterinary Laboratory in Algete (National Reference Laboratory for TSEs, accredited under UNE-EN ISO/IEC 17025:2005 standard) received a nerve tissue sample suspected to be infected by bovine spongiform encephalopathy (BSE) from the accredited Regional Laboratory for Animal Health in Lugo, Galicia (official regional laboratory), after a positive result was obtained through a HerdChek BSE-Scrapie Antigen Test Kit (Idexx Laboratories) rapid test. The National Reference Laboratory carried out the confirmation tests authorized according to EU Regulation No. 1148/2014. The selected combined tests were ELISA, carried out by the regional laboratory (HerdChek), and Prionics Check Western Blot, and positive results were obtained for both tests. Afterwards, tests for BSE strain discrimination were carried out through hybrid immunoblotting with antibodies against PrP, confirming atypical BSE (H type strain). The sample was taken within the national TSE surveillance program (sampling of dead or non-slaughtered for human consumption animals over 48 months old). The animal was a crossbred cow born on January 30th, 2001. |
Control measures
| Measures applied |
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| Measures to be applied |
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Diagnostic test results
| Laboratory name and type | Central Veterinary Laboratory in Algete ( National laboratory ) | ||||||||
| Tests and results |
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Future Reporting
| The event is resolved. No more reports will be submitted. |
Encéphalopathie spongiforme bovine ,Espagne
Information reçue le 15/07/2019 de Dr Valentín Almansa, Director General, Sanidad de la Produccion Agraria, Ministerio de Agricultura, Alimentación y Medio Ambiente, Madrid, Espagne
Résumé
| Type de rapport | Notification immédiate (rapport final) |
| Date de début de l’événement | 03/07/2019 |
| Date de confirmation de l´événement | 10/07/2019 |
| Date du rapport | 15/07/2019 |
| Date d'envoi à l'OIE | 16/07/2019 |
| Date de clôture de l'événement | 16/07/2019 |
| Raison de notification | Réapparition d’une maladie listée par l'OIE |
| Date de la précédente apparition de la maladie | 19/02/2019 |
| Manifestation de la maladie | Maladie clinique |
| Agent causal | Prion (ESB atypique de type H) |
| Nature du diagnostic | Tests approfondis en laboratoire (i.e. virologie, microscopie électronique, biologie moléculaire, immunologie) |
| Cet événement se rapporte à | une zone définie à l'intérieur du pays |
Nouveaux foyers
| Récapitulatif des foyers | Nombre total de foyers : 1 | ||||||||||||
| Localisation du foyer |
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| Nombre total d'animaux atteints |
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| Statistiques sur le foyer |
* Soustraits de la population sensible suite à la mort, à l´abattage et/ou à la destruction; |
Epidémiologie
| Source du/des foyer(s) ou origine de l´infection |
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| Autres renseignements épidémiologiques / Commentaires | Le 5 juillet 2019, le Laboratoire central vétérinaire d’Algete (Laboratoire national de référence pour les EST, accrédité selon la norme UNE-EN ISO/IEC 17025:2005) a reçu un échantillon de tissu nerveux suspecté d'être infecté par l’encéphalopathie spongiforme bovine (ESB) envoyé par le laboratoire régional agréé de santé animale de Lugo, Galice (laboratoire régional officiel), suite à l’obtention d’un résultat positif au test rapide HerdChek BSE-Scrapie Antigen Test Kit (laboratoires Idexx). Le laboratoire national de référence a effectué les tests de confirmation autorisés, conformément au Règlement (UE) nº 1148/2014. Combinaison de tests sélectionnés : ELISA, effectué par le laboratoire régional (HerdChek), et immunoblot Prionics Check Western Blot. Les deux tests ont donné des résultats positifs. Ensuite, des tests de discrimination de souches de l’ESB via immuno-empreintes hybrides avec différents anticorps dirigés contre PrP ont été effectués et ont confirmé la présence de l’ESB atypique (souche de type H). L’échantillon a été prélevé dans le cadre du programme national de surveillance des EST (prélèvement sur animaux morts ou non-sacrifiés pour consommation par l‘homme, de plus de 48 mois). L’animal était une vache née d’un croisement de races le 30 janvier 2001. |
Mesures de lutte
| Mesures de lutte appliquées |
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| Mesures à appliquer |
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Résultats des tests de diagnostics
| Nom du laboratoire et type | Laboratoire central vétérinaire d’Algete ( Laboratoire national ) | ||||||||
| Tests et résultats |
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Rapports futurs
| L’événement est terminé. Aucun autre rapport ne sera envoyé. |
Encefalopatía espongiforme bovina ,España
Información recibida el 15/07/2019 desde Dr Valentín Almansa, Director General, Sanidad de la Produccion Agraria, Ministerio de Agricultura, Alimentación y Medio Ambiente, Madrid, España
Resumen
| Tipo de informe | Notificación inmediata(Informe final) |
| Fecha del inicio del evento | 03/07/2019 |
| Fecha de confirmación del evento | 10/07/2019 |
| Fecha del informe | 15/07/2019 |
| Fecha de envio del informe a la OIE | 16/07/2019 |
| Fecha del cierre del evento | 16/07/2019 |
| Motivo de la notificación | Recurrencia de una enfermedad de la Lista de la OIE |
| Fecha de la anterior aparición de la enfermedad | 19/02/2019 |
| Manifestación de la enfermedad | Enfermedad clínica |
| Agente causal | Prión (EEB atípica tipo H) |
| Naturaleza del diagnóstico | Pruebas de diagnóstico de laboratorio avanzadas (ej. virología, microscopía electrónica, biología molecular e inmunología) |
| Este evento concierne | una zona definida dentro del país |
Nuevos focos
| Resumen de los focos | Número total de focos: 1 | ||||||||||||
| Localización del foco |
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| Número total de animales afectados |
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| Estadística del foco |
* Descontados de la población susceptible a raíz de su muerte, destrucción o sacrificio; |
Epidemiología
| Fuente del o de los focos u origen de la infección |
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| Otros detalles epidemiológicos / comentarios | El 5 de julio de 2019, el Laboratorio Central Veterinario de Algete (Laboratorio Nacional de Referencia de EET, acreditado según la norma UNE-EN ISO/IEC 17025:2005), recibió una muestra de tejido nervioso sospechoso de infección por encefalopatía espongiforme bovina (EEB) del Laboratorio Regional de Sanidad Animal acreditado en Lugo, Galicia (laboratorio regional oficial), tras la obtención de un resultado positivo en la prueba rápida HerdChek BSE-Scrapie Antigen Test Kit (Laboratorios Idexx). El LNR llevó a cabo los ensayos de confirmación autorizados según el Reglamento UE nº 1148/2014. Las pruebas combinadas seleccionadas fueron el ELISA realizado por el laboratorio regional (HerdChek) y el inmunoblot Prionics Check Western Blot. Se obtuvieron resultados positivos en ambas pruebas. Posteriormente, se realizaron pruebas de discriminación de cepas de EEB mediante inmunotransferencia híbrida con anticuerpos contra la PrP, lo que confirmó la presencia de EEB atípica (cepa tipo H). La muestra se tomó en el marco del programa nacional de vigilancia de las EET (muestreo de animales muertos o no sacrificados para el consumo humano de más de 48 meses de edad). El animal era una vaca un cruce de razas nacida el 30 de enero de 2001. |
Medidas de Control
| Medidas implementadas |
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| Medidas para implementar |
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Resultados de las pruebas diagnósticas
| Nombre y tipo de laboratorio | Laboratorio Central Veterinaria Algete ( Laboratorio nacional ) | ||||||||
| Pruebas y resultados |
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Informes futuros
| El episodio ha sido resuelto. Ningún otro informe será enviado http://www.oie.int/wahis_2/temp/reports/en_imm_0000031093_20190716_105613.pdf |
P98 The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challengeGreenlee JJ (1), Moore SJ (1), and West Greenlee MH (2) (1) United States Department of Agriculture, Agricultural Research Service, National Animal Disease Center, Virus and Prion Research Unit, Ames, IA, United States (2) Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, United States.reading up on this study from Prion 2018 Conference, very important findings ;***> This study demonstrates that the H-type BSE agent is transmissible by the oronasal route.***> These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.PRION 2018 CONFERENCE ABSTRACTSunday, February 25, 2018PRION ROUND TABLE CONFERENCE 2018 MAY, 22-25 A REVIEWWEDNESDAY, OCTOBER 24, 2018
Experimental Infection of Cattle With a Novel Prion Derived From Atypical H-Type Bovine Spongiform Encephalopathy
Yup, sure enough, not like nobody tried to tell them a decade or so ago. .....tss>>> The occurrence of atypical cases of BSE in countries such as Canada with low BSE prevalence and transmission risk argues for the occurrence of sporadic forms of BSE worldwide. <<<In my opinion ;THE statement above is about as non-scientific as a statement can be.There is no proof what-so-ever that any of the atypical BSE cases or atypical scrapie cases anywhere on the globe was a spontaneous case without any route and source of the TSE agent... This is a myth.The USDA and the OIE are trying to make the atypical BSE cases and they have already made the atypical Scrapie cases a legal trading commodity, without any transmission studies first confirming that in fact these atypical TSE will not transmit via feed.I suppose it is a human transmission study in progress.IT's like what happened in England with c-BSE and the transmission to humans via nvCJD never happened to the OIE and the USDA. Canada does not have a low prevalence of BSE either, they have a high prevalence.WHO knows about North America ?it's just that the U.S.A. try's much harder at concealing cases of mad cow disease.THIS was proven with the first stumbling and staggering mad cow in Texas, that was Wisk away to be rendered without any test at all.Then, you had the second case of mad cow disease that the USDA et al was almost as successful with as the first one, but the O.I.G. stepped in and demanded testing over seas, this after many scientist around the globe spoke out. Finally, after an act of Congress, the second case of mad cow disease in Texas was confirmed.all this was done for a reason, and that reason was the OIE USDA BSE MRR policy.Again, This study reeks of TRADE policy wrangling.There is NO proof that the atypical TSE are spontaneous.please show me these transmission studies ?on the other hand, we now know that the L-type atypical BSE is much more virulent than the typical C-BSE, and we now know that theH-type atypical BSE will transmit to humans.WHY can it not be that these atypical cases are simply from feed that had different strains of TSE ? WHY is it that no one will comment on the studies that was suppose to show infectivity of tissues from atypical BSE ?WHY is it I had to file a FOIA on that issue?L-type atypical BSE (BASE) is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice.BSE-H is also transmissible in our humanized Tg mice.SEE Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 et al 2009 ;Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009snip...I ask Professor Kong ;Thursday, December 04, 2008 3:37 PMSubject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk AssessmentIS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....Professor Kong reply ;.....snipAs to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.Best regards,Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USAP.4.23 Transmission of atypical BSE in humanized mouse modelsLiuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USABackground: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were argely undefined.Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice.Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time. The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.see full text ;snip...full test Singeltary et al PLOSTHURSDAY, JULY 20, 2017USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow.This new prionopathy in humans?the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow,WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ???there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$ALABAMA MAD COW g-h-BSEalabamaIn this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America.This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD.This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene.We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.Saturday, August 14, 2010BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008). This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol 457|26 February 2009Thursday, July 24, 2014*** Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical BSE investigationsThursday, July 24, 2014*** Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical BSE investigationsTHURSDAY, FEBRUARY 14, 2013Unique Properties of the Classical Bovine Spongiform Encephalopathy Strain and Its Emergence From H-Type Bovine Spongiform Encephalopathy Substantiated by VM Transmission StudiesSaturday, August 14, 2010BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY(see mad cow feed in COMMERCE IN ALABAMA...TSS)2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006P02.35Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSEBiacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, SwedenWestern blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK-resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.FRIDAY, FEBRUARY 22, 2019
Spain Bovine Spongiform Encephalopathy BSE TSE Confirmed
MONDAY, JUNE 19, 2017
PRION 2017 CONFERENCE ABSTRACT P61 vCJD strain properties in a Spanish mother and son replicate as those of a young UK case
FRIDAY, MAY 12, 2017
SPAIN OIE Bovine Spongiform Encephalopathy atypical L-type Camargo, CANTABRIA
FRIDAY, MARCH 10, 2017
OIE Spain Prion (Atypical BSE type L) Bovine Spongiform Encephalopathy Mad Cow Disease
FRIDAY, JANUARY 09, 2009
Mad cow disease detected on Madrid farm
SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma) SEACAgenda 102nd Meeting on Wednesday 4 March 2009 Room 808, Nobel House, 17 Smith Square, Defra, London SW1P 3JR10.05 Approval of draft minutes from SEAC 101
snip...
ITEM 3 - CURRENT ISSUES 8. SEAC was informed about the following issues: . A mother and son in Spain had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. As both the mother and son lived in a region of Spain with a history of BSE, had frequently shared meals of cattle brain, and as no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure. Furthermore, the similar times of onset of disease of the cases did not suggest transmission had occurred from one to the other.
snip...
Thursday, February 26, 2009
SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma)
STATEMENT ON A VARIANT CJD FAMILY CLUSTER
Prof Robert G Will,NCJDSU 25th September 2008r.g.will@ed.a c.uk
Three cases of pathologically confirmed variant CJD have been identified in Spain in recent years, including a man in his early 40s who died earlier this year. The clinical illness in this individual was typical of variant CJD, including the appearances on the MRI brain scan.
A few months ago his mother, who was in her 60s, developed a rapidly progressive neurological illness and died about 5 months from the onset of this illness. An MRI brain scan showed appearances suggestive of variant CJD and preliminary results from post-mortem examination suggest that the suspected diagnosis of variant CJD is correct. Further results, which may confirm this diagnosis, should be available within a few days.
Since 1994 there have been 167 cases of variant CJD in the UK, 23 cases in France and 15 cases in other countries, excluding Spain. The occurrence of variant CJD in more than one member of the same family has not been seen before and it has been the general view that family members of variant CJD cases are not themselves at greater risk of developing this condition. This raises the question as to why two cases of variant CJD have now been found in a family in Spain. There is no evidence of a genetic form of CJD in these Spanish cases and preliminary investigation has not shown any risk of CJD through medical or surgical treatment.
There is no evidence of any risk of transmission of CJD through direct personal contact. The mother and son lived in an area of Spain in which BSE has been found and it is possible that direct consumption of material with high levels of BSE infection may have been the source of the infection. In the UK and other countries it is believed that processed bovine tissues were the most likely source of BSE infection and it is possible that different forms of exposure to BSE infection may explain the occurrence of variant CJD in two family members in Spain and not elsewhere.
This, however, is uncertain and public health policies in relation to variant CJD may have to be reviewed in the light of these two cases in Spain.
Monday, September 01, 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
Spanish woman and son could be first relatives to die of BSE
Submitted by Marina Dimova on Wed, 08/27/2008 - 11:53.
The Spanish health authorities are investigating the death of a woman whose son died earlier of Creutzfeldt-Jakob Disease (CJD), the human variant of bovine spongiform encephalopathy (BSE) or mad cow disease, experts said Wednesday in Madrid.
If it is confirmed that the woman and her son died from the same cause, they would be the first members of the same family in the world to succumb to CJD, neuropathologist Alberto Rabano said.
The woman, who was about 60-years-old, passed away last week in the northern city of Leon. The son died in February. Their identities were not given.
Veterinary experts said the son caught the disease by eating infected meat before 2001, when preventative measures were adopted.
Terry S. Singeltary Sr. Doctor Antonio Ruiz Villaespesa, pathologist and CJD researcher deceased because of Creutzfeldt-Jakob Disease SPAIN. 21 Apr 2009. [Accessed 11 Apr 2012]. In: Monitoring the occurrence of emerging forms of CJD [blog]. Available from:
snip...see full text ;
MONDAY, SEPTEMBER 01, 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
SUNDAY, MARCH 28, 2010
SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION
FRIDAY, JANUARY 09, 2009
Mad cow disease detected on Madrid farm Friday, January 9, 2009
Eurosurveillance, Volume 10, Issue 31, 04 August 2005
Articles
Citation style for this article: Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(31):pii=2764. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2764 ;
First case of vCJD reported in Spain
Centro Nacional de Epidemiología, Instituto de Salud Carlos III, Madrid, Spain (http://cne.isciii.es/) ;
The Spanish Ministry of Health has reported the detection of a case of variant Creutzfeldt-Jakob disease (vCJD) in Spain [1].
The patient was a 26 year old woman in Madrid who died on 10 July 2005. She had no specific risk factors for vCJD (no prior blood transfusions or visits to the United Kingdom).
The patient’s symptoms began in November 2004, with rapid progression to dementia, normal MRI and EEG, and 14-3-3 protein found in cerebral spinal fluid. Other neurological manifestations including ataxia, dysarthria, apraxia and myoclonus appeared in early 2005. The first abnormal MRI was observed in April 2005. The patient was methionine homozygous at codon 129 of the prion protein gene (PRPN). There were no identified PRPN mutations or family history of CJD.
The case was notified to the Spanish CJD state registry in May 2005 and initially fitted the criteria for both probable sporadic CJD (sCJD) and possible vCJD; it was registered as probable sCJD. Brain neuropathology results of tests conducted at Fundación Hospital de Alcorcón at Madrid subsequently confirmed vCJD. Materials have been sent to the European reference centre at the United Kingdom’s National CJD Surveillance Unit in Edinburgh for further study, and results are expected later in August.
References:
Ministerio de Sanidad y Consumo. La Comunidad de Madrid comunica al Ministerio de Sanidad la probable detección del primer caso de la variante de la enfermedad de Creutzfeldt-Jakob en España. Press release 29 July 2005. (http://www.msc.es/gabinetePrensa/notaPrensa/desarrolloNotaPrensa.jsp?id=385)
PPo4-15:A Surprisingly High Number of the Plaque-Like VV sCJD Subtype Among the Polish sCJD-is There a Connection with BASE?Beata Sikorska and Pawel P. Liberski Department of Molecular Pathology and Neuropathology; Medical University of Lodz; Lodz, PolandRecently described bovine amyloidotic spongiform encephalopathy (BASE) or L type BSE-was is overrepresented in Poland (15% of all cases of BSE). Moreover, the number of BASE cases in Poland per million bovines is the highest in Europe. A potential human risk from BASE is evident from experimental transmission to "humanized" transgenic animals and primates. Taking into consideration that non-human primate inoculated with BASE had a shorter incubation period than monkeys infected with classical BSE, and that humanized Tg mice have been found to be highly susceptible to infection with atypical form of BSE, it seems probable that BASE may be more pathogenic for humans than BSE, but the transmitted disease may differ from BSE-derived vCJD. Among 47 cases which have been diagnosed as definite in our laboratory, in 19 cases complete histopathological examination and codon 129 status were available. On the basis of the histological pattern and codon 129 status the cases of sCJD were divided into subtypes according to the Parchi&Gambetti classification. The results are as follows: type 1 (MMorMV)- 42%, type 2 (VV)-32%, type 3 (MV)-10.5%, type 4c (MM)- 10.5% and type 5 (VV)-5 %. Although the number of cases is too low to conclude a significantly different distribution of sCJD subtypes in Polish population those data show surprisingly high number of the plaque-like VV sCJD subtype. Interestingly, it was shown before that Tg mice inoculated with BASE showed granular and plaque-like aggregates or PrPSc in brains resembling those observed in VV2 subtype of sCJD.PPo2-26:Transmission of Classical and Atypical (L-type) Bovine Spongiform Encephalopathy (BSE) Prions to Cynomolgus macaquesFumiko Ono,1 Yoshio Yamakawa,2 Minoru Tobiume,3 Yuko Sato,3 Harutaka Katano,3 Kenichi Hagiwara,2 Iori Itagaki,1 Akio Hiyaoka,1 Katuhiko Komatuzaki,1 Yasunori Emoto,1 Hiroaki Shibata,4 Yuichi Murayama,5 Keiji Terao,4 Yasuhiro Yasutomi4 and Tetsutaro Sata31The Corporation for Production and Research of Laboratory Primates; Tsukuba City, Japan; 2Departments of Cell Biology and Biochemistry; and 3Pathology; National Institute of Infectious Diseases; Tokyo, Japan; 4Tsukuba Primate Research Center; National Institute of Biomedical Innovation; Tsukuba City, Japan; 5Prion Disease Research Team; National Institute of Animal Health; Tsukuba City, JapanKey words: L-type BSE, cBSE, cynomolgus macaques, transmissionBSE prion derived from classical BSE (cBSE) or L-type BSE was characterized by inoculation into the brain of cynomolgus macaques. The neurologic manifestation was developed in all cynomolgus macaques at 27-43 months after intracerebral inoculation of brain homogenate from cBSE-affected cattle (BSE JP/6). Second transmission of cBSE from macaque to macaque shortened incubation period to 13-18 months. cBSE-affected macaques showed the similar clinical signs including hyperekplexia, tremor and paralysis in both primary and second transmission.Two macaques were intracerebrally inoculated brain homogenate from the L-type BSE-affected cattle (BSE JP/24). The incubation periods were 19-20 months in primary transmission.The clinical course of the L-type BSE-affected macaques differed from that in cBSE-affected macaques in the points of severe myoclonus without hyperekplexia. The glycoform profile of PrPSc detected in macaque CNS was consistent with original pattern of either cBSE or L-typeBSE PrPSc, respectively. Although severe spongiform change in the brain was remarkable in all BSE-affected macaques, severe spongiform spread widely in cerebral cortex in L-type BSE-affected macaques. Heavy accumulation of PrPSc surrounded by vacuola formed florid plaques in cerebral cortex of cBSE-affected macaques. Deposit of PrPSc in L-type BSE-affected macaque was weak and diffuse synaptic pattern in cerebrum, but large PrPSc plaques were evident at cerebellum. MRI analysis, T2, T1, DW and flair sequences, at the time of autopsy revealed that brain atrophy and dilatation of cerebral ventricles were significantly severe in L-type BSE-affected macaques. These results suggest that L-type BSE is more virulent strain to primates comparing to cBSE.SP1-4:Evidence from Molecular Strain TypingGianluigi Zanusso Department of Neurological and Visual Sciences; Section of Clinical Neurology; University of Verona; Verona, ItalyKey words: molecular analysis, strain typing, atypical BSE, CJDIn 2001, active surveillance for bovine spongiform encephalopathy (BSE) led to the discovery of atypical BSE phenotypes in aged cattle distinct from classical BSE (C-type). These atypical BSE cases had been classified as low L-type (BASE) or high H-type BSE based on the molecular mass and the degree of glycosylation of of the pathological prion protein (PrPSc). Transmission studies in TgBov mice showed that H-type BSE, C-type BSE and BASE behave as distinct prion strains with different incubation periods, PrPSc molecular patterns and pathological phenotypes. A still unclear issue concerns the potential transmissibility and phenotypes of atypical BSEs in humans. We previously indicated that BASE was similar to a distinct subgroup of sporadic form of Creutzfeldt-Jakob disease (sCJD) MV2, based on molecular similarities and on neuropathological pattern of PrP deposition. To investigate a possible link between BASE and sCJD, Kong et al. and Comoy et al. experimentally inoculated TgHu mice (129MM) and a non-human primate respectively, showing in both models that BASE was more virulent compare to BSE. Further, non-human primate reproduced a clinical phenotype resembling to that of sCJD subtype MM2. Here, we presented a comparative analysis of the biochemical fingerprints of PrPSc between the different sCJD subtypes and animal TSEs and after experimental transmission to animals.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK.It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive.The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995.Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE...32 Clark, W., Hourrigan, J. and Hadlow, W. (1995) Encephalopathy in Cattle Experimentally Infected with the Scrapie Agent, American Journal of Veterinary Research, 56, 606-1233 YB88/10.00/1.1Technical Abstract:Prion strains may vary in their ability to transmit to humans and animals. Few experimental studies have been done to provide evidence of differences between U.S. strains of scrapie, which can be distinguished by incubation times in inbred mice, microscopic lesions, immunoreactivity to various antibodies, or molecular profile (electrophoretic mobility and glycoform ratio). Recent work on two U.S. isolates of sheep scrapie supports that at least two distinct strains exist based on differences in incubation time and genotype of sheep affected. One isolate (No. 13-7) inoculated intracerebrally caused scrapie in sheep AA at codon 136 (AA136) and QQ at codon 171 (QQ171) of the prion protein in an average of 19 months post-inoculation (PI) whereas a second isolate (No. x124) caused disease in less than 12 months after oral inoculation in AV136/QQ171 sheep. Striking differences were evident when further strain analysis was done in R111, VM, C57Bl6, and C57Bl6xVM (F1) mice. No. 13-7 did not induce disease in any mouse strain at any time post-inoculation (PI) nor were brain tissues positive by western blot (WB). Positive WB results were obtained from mice inoculated with isolate No. x124 starting at day 380 PI. Incubation times averaged 508, 559, 601, and 633 days PI for RIII, C57Bl6, VM, and F1 mice, respectively. Further passage will be required to characterize these scrapie strains in mice.***>This work provides evidence that multiple scrapie strains exist in U.S. sheep.One of these isolates (TR316211) behaved like the CH1641 isolate, with PrPres features in mice similar to those in the sheep brain. From two other isolates (O100 and O104), two distinct PrPres phenotypes were identified in mouse brains, with either high (h-type) or low (l-type) apparent molecular masses of unglycosylated PrPres, the latter being similar to that observed with CH1641, TR316211, or BSE. Both phenotypes could be found in variable proportions in the brains of the individual mice. In contrast with BSE, l-type PrPres from "CH1641-like" isolates showed lower levels of diglycosylated PrPres. From one of these cases (O104), a second passage in mice was performed for two mice with distinct PrPres profiles. This showed a partial selection of the l-type phenotype in mice infected with a mouse brain with predominant l-type PrPres, and it was accompanied by a significant increase in the proportions of the diglycosylated band. These results are discussed in relation to the diversity of scrapie and BSE strains.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.- 59-P-088 Transmission of experimental CH1641-like scrapie to bovine PrP overexpression miceKohtaro Miyazawa1, Kentaro Masujin1, Hiroyuki Okada1, Yuichi Matsuura1, Takashi Yokoyama21Influenza and Prion Disease Research Center, National Institute of Animal Health, NARO, Japan; 2Department of Planning and General Administration, National Institute of Animal Health, NAROIntroduction: Scrapie is a prion disease in sheep and goats. CH1641-lke scrapie is characterized by a lower molecular mass of the unglycosylated form of abnormal prion protein (PrpSc) compared to that of classical scrapie. It is worthy of attention because of the biochemical similarities of the Prpsc from CH1641-like and BSE affected sheep. We have reported that experimental CH1641-like scrapie is transmissible to bovine PrP overexpression (TgBoPrP) mice (Yokoyama et al. 2010). We report here the further details of this transmission study and compare the biological and biochemical properties to those of classical scrapie affected TgBoPrP mice.Methods: The details of sheep brain homogenates used in this study are described in our previous report (Yokoyama et al. 2010). TgBoPrP mice were intracerebrally inoculated with a 10% brain homogenate of each scrapie strain. The brains of mice were subjected to histopathological and biochemical analyses.Results: Prpsc banding pattern of CH1641-like scrapie affected TgBoPrP mice was similar to that of classical scrapie affected mice. Mean survival period of CH1641-like scrapie affected TgBoPrP mice was 170 days at the 3rd passage and it was significantly shorter than that of classical scrapie affected mice (439 days). Lesion profiles and Prpsc distributions in the brains also differed between CH1641-like and classical scrapie affected mice.Conclusion: We succeeded in stable transmission of CH1641-like scrapie to TgBoPrP mice. Our transmission study demonstrates that CH 1641-like scrapie is likely to be more virulent than classical scrapie in cattle.Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIESTitle: Comparison of two US sheep scrapie isolates supports identification as separate strainsAuthorsitem Moore, Sarah - item Smith, Jodi item West Greenlee, Mary - item Nicholson, Eric item Richt, Juergen item Greenlee, JustinSubmitted to: Veterinary Pathology Publication Type: Peer Reviewed Journal Publication Acceptance Date: December 22, 2015 Publication Date: N/AInterpretive Summary: Scrapie is a fatal disease of sheep and goats that causes damaging changes in the brain. The infectious agent is an abnormal protein called a prion that has misfolded from its normal state. Whether or not a sheep will get scrapie is determined primarily by their genetics. Furthermore, different scrapie strains exist that may result in a different expression of disease such as shorter incubation periods, unusual clinical signs, or unique patterns of lesions within the brain. This study evaluated two U.S. scrapie isolates in groups of sheep with varying susceptibilities to scrapie. Our data indicates that there are differences in incubation periods, sheep genotype susceptibilities, and lesion profiles that support designating these scrapie isolates as unique strains. The identification of a new scrapie strain in the United States means that control measures, methods of decontamination, and the potential for transmission to other species may need to be reevaluated. This information is useful to sheep farmers and breeders that are selectively breeding animals with genotypes resistant to the most prevalent strain of scrapie and could impact future regulations for the control of scrapie in the United States. Technical Abstract: Scrapie is a naturally occurring transmissible spongiform encephalopathy (TSE) of sheep and goats. There are different strains of sheep scrapie that are associated with unique molecular, transmission, and phenotype characteristics, but very little is known about the potential presence of scrapie strains within sheep in the US. Scrapie strain and PRNP genotype could both affect susceptibility, potential for transmission, incubation period, and control measures required for eliminating scrapie from a flock. Here we evaluate two US scrapie isolates, No. 13-7 and x124, after intranasal inoculation to compare clinical signs, incubation periods (IP), spongiform lesions, and patterns of PrPSc deposition in sheep with scrapie-susceptible PRNP genotypes (QQ171). After inoculation with x124, susceptibility and IP were associated with valine at codon 136 (V136) of the prion protein: VV136 had short IPs (6.9 months), AV136 sheep were 11.9 months, and AA136 sheep did not develop scrapie. All No.13-7 inoculated sheep developed scrapie with IP’s of 20.1 months for AA136 sheep, 22.8 months for AV136 sheep, and 26.7 months for VV136 sheep. Patterns of immunoreactivity in the brain were influenced by challenge isolate and host genotype. Differences in PrPSc profiles versus isolate were most striking when examining brains from sheep with the VV136 genotype. In summary, intranasal inoculation with isolates x124 and No. 13-7 resulted in differences in IP, sheep genotype susceptibility, and PrPSc profile that support designation as separate strains.Last Modified: 6/6/201631Appendix I VISIT TO USA - OR A E WRATHALL — INFO ON BSE AND SCRAPIEDr Clark lately of the scrapie Research Unit, Mission Texas hassuccessfully transmitted ovine and caprine scrapie to cattle. Theexperimental results have not been published but there are plans to dothis. This work was initiated in 1978. A summary of it is:-Expt A 6 Her x Jer calves born in 1978 were inoculated as follows witha 2nd Suffolk scrapie passage:-i/c 1ml; i/m, 5ml; s/c 5ml; oral 30ml.1/6 went down after 48 months with a scrapie/BSE-like disease.Expt B 6 Her or Jer or HxJ calves were inoculated with angora Goatvirus 2/6 went down similarly after 36 months.Expt C Mice inoculated from brains of calves/cattle in expts A & B were resistant, only 1/20 going down with scrapie and this was the reason given for not publishing.Diagnosis in A, B, C was by histopath. No reports on SAF were given.Dr Warren Foote indicated success so far in eliminating scrapie in offspring from experimentally— (and naturally) infected sheep by ET. He had found difficulty in obtaining embryos from naturally infected sheep (cf SPA).Prof. A Robertson gave a brief accout of BSE. The us approach was to32accord it a very low profile indeed. Dr A Thiermann showed the picture in the "Independent" with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs.BSE was not reported in USA.4. Scrapie incidents (ie affected flocks) have shown a dramatic increase since 1978. In 1953 when the National Control scheme was started there were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.5. Scrapie agent was reported to have been isolated from a solitary fetus.6. A western blotting diagnostic technique (? on PrP) shows some promise.7. Results of a questionnaire sent to 33 states on the subject of the national sheep scrapie programme survey indicated17/33 wished to drop it6/33 wished to develop it8/33 had few sheep and were neutralInformation obtained from Dr Wrathall‘s notes of a meeting of the u.s.Animal Health Association at Little Rock, Arkansas Nov. 1988.33In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...also see hand written notes ;Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected CattleOver the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.snip...The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSUREThis is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........REPORT OF THE WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY 1989snip...4.2.9 ...Also, if it resulted from a localised chance transmission of the scrapie strain from sheep to cattle giving rise to a mutant, a different pattern of disease would have been expected: its range would have increased with time. Thus the evidence from Britain is against the disease being due to a new strain of the agent, but we note that in the United States from 1984 to 1988 outbreaks of scrapie in sheep flocks are reported to have Increased markedly, now being nearly 3 times as high as during any previous period (18).Monday, September 13, 2010atypical BSE strains and sporadic CJD strains, is there a connection and why shouldn't there be $snip...see full text;INDEED, thanks to the O...I.E., and the U.S.D.A. SSS policy, and the fact they are using science dated back to 1985 still in some cases. THE infamous June 2004 enhanced bse surveillance program was set up to fail from the beginning, and fail they did, not only with the surveillance for TSE, but also the partial and voluntary feed ban of August 4, 1997 was nothing but ink on paper. What i predicted a decade ago, has in fact come to pass, the TSE agent has mutated in every species from CWD now at two documented strains, BSE with 4 strains documented to date (c-BSE, h-BSE [typical h-BSE or the one and only documented g-h-BSEalabama strain?], l-BSE, and the IBNC BSE), and the different Scrapie strains are too many to count, not included the atypical Nor-98 and or BSE in sheep. with the 3 strains of BSE documented in North America to date, the two strains of CWD, TME, and all the strains of Scrapie, with 5 cases of the Nor-98 atypical scrapie cases already documented in 2010 here in the USA, all of which of the past two decades have been rendered and fed to food producing animals for animals and humans, and any human TSE there from ??? please note that the sporadic CJD case here in the USA has had a steady increase since 1997. it's not rocket science. what the USDA, FDA et al have used is junk science, bought and paid for by your local cattle dealer i.e. INDUSTRY. the BSE MRR policy was nothing more than a legal tool to do the same thing when the U.K. poisoned the globe with BSE, except now it's legal $$$these are the facts as i have come to know them. just my take. ...Let's take a look at the facts shall we $BOVINE SPONGIFORM ENCEPHALOPATHY, SCRAPIE, CWD, CJD, NORTH AMERICA TYPICAL AND ATYPICALExperimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Micesnip...see full text;IBNC BSE TSE Prion mad cow disease***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***Posted by Terry S. Singeltary Sr. on 03 Jul 2015 at 16:53 GMT***> NEW TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION DISEASE (MAD CAMEL DISEASE) IN A NEW SPECIES <***NEW OUTBREAK OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION DISEASE IN A NEW SPECIESSubject: Prion Disease in Dromedary Camels, AlgeriaOur identification of this prion disease in a geographically widespread livestock species requires urgent enforcement of surveillance and assessment of the potential risks to human and animal health.Wednesday, May 30, 2018Dromedary camels in northern Africa have a neurodegenerative prion disease that may have originated decades ago***> IMPORTS AND EXPORTS <***SEE MASSIVE AMOUNTS OF BANNED ANIMAL PROTEIN AKA MAD COW FEED IN COMMERCE USA DECADES AFTER POST BANWEDNESDAY, JULY 11, 2018CONFIDENTIAL IN CONFIDENCE SPONGIFORM ENCEPHALOPATHY OF PIGS FDA EMERGENCY REQUEST FOR RULE CHANGE USA Section 21 C.F.R. 589.2000TUESDAY, JULY 10, 2018CONFIDENTIAL IN CONFIDENCE SPONGIFORM ENCEPHALOPATHY OF PIGS*** ''but feeding of other ruminant protein, including scrapie-infected sheep, can continue to pigs.''CONFIDENTIAL SPONGIFORM ENCEPHALOPATHY OF PIGS
***> Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility. <***Transmission of scrapie prions to primate after an extended silent incubation periodEmmanuel E. Comoy, Jacqueline Mikol, Sophie Luccantoni-Freire, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Valérie Durand, Capucine Dehen, Olivier Andreoletti, Cristina Casalone, Juergen A. Richt, Justin J. Greenlee, Thierry Baron, Sylvie L. Benestad, Paul Brown & Jean-Philippe Deslys Scientific Reports volume 5, Article number: 11573 (2015) | Download CitationAbstractClassical bovine spongiform encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures for animal and human health against animal prion diseases. Recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-BSE. We here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly relevant model for human prion diseases, after a 10-year silent incubation period, with features similar to those reported for human cases of sporadic CJD. Scrapie is thus actually transmissible to primates with incubation periods compatible with their life expectancy, although fourfold longer than BSE. Long-term experimental transmission studies are necessary to better assess the zoonotic potential of other prion diseases with high prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98 scrapie.SNIP...Discussion We describe the transmission of spongiform encephalopathy in a non-human primate inoculated 10 years earlier with a strain of sheep c-scrapie. Because of this extended incubation period in a facility in which other prion diseases are under study, we are obliged to consider two alternative possibilities that might explain its occurrence. We first considered the possibility of a sporadic origin (like CJD in humans). Such an event is extremely improbable because the inoculated animal was 14 years old when the clinical signs appeared, i.e. about 40% through the expected natural lifetime of this species, compared to a peak age incidence of 60–65 years in human sporadic CJD, or about 80% through their expected lifetimes. Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.The second possibility is a laboratory cross-contamination. Three facts make this possibility equally unlikely. First, handling of specimens in our laboratory is performed with fastidious attention to the avoidance of any such cross-contamination. Second, no laboratory cross-contamination has ever been documented in other primate laboratories, including the NIH, even between infected and uninfected animals housed in the same or adjacent cages with daily intimate contact (P. Brown, personal communication). Third, the cerebral lesion profile is different from all the other prion diseases we have studied in this model19, with a correlation between cerebellar lesions (massive spongiform change of Purkinje cells, intense PrPres staining and reactive gliosis26) and ataxia. The iron deposits present in the globus pallidus are a non specific finding that have been reported previously in neurodegenerative diseases and aging27. Conversely, the thalamic lesion was reminiscent of a metabolic disease due to thiamine deficiency28 but blood thiamine levels were within normal limits (data not shown). The preferential distribution of spongiform change in cortex associated with a limited distribution in the brainstem is reminiscent of the lesion profile in MM2c and VV1 sCJD patients29, but interspecies comparison of lesion profiles should be interpreted with caution. It is of note that the same classical scrapie isolate induced TSE in C57Bl/6 mice with similar incubation periods and lesional profiles as a sample derived from a MM1 sCJD patient30.We are therefore confident that the illness in this cynomolgus macaque represents a true transmission of a sheep c-scrapie isolate directly to an old-world monkey, which taxonomically resides in the primate subdivision (parvorder of catarrhini) that includes humans. With an homology of its PrP protein with humans of 96.4%31, cynomolgus macaque constitutes a highly relevant model for assessing zoonotic risk of prion diseases. Since our initial aim was to show the absence of transmission of scrapie to macaques in the worst-case scenario, we obtained materials from a flock of naturally-infected sheep, affecting animals with different genotypes32. This c-scrapie isolate exhibited complete transmission in ARQ/ARQ sheep (332 ± 56 days) and Tg338 transgenic mice expressing ovine VRQ/VRQ prion protein (220 ± 5 days) (O. Andreoletti, personal communication). From the standpoint of zoonotic risk, it is important to note that sheep with c-scrapie (including the isolate used in our study) have demonstrable infectivity throughout their lymphoreticular system early in the incubation period of the disease (3 months-old for all the lymphoid organs, and as early as 2 months-old in gut-associated lymph nodes)33. In addition, scrapie infectivity has been identified in blood34, milk35 and skeletal muscle36 from asymptomatic but scrapie infected small ruminants which implies a potential dietary exposure for consumers.Two earlier studies have reported the occurrence of clinical TSE in cynomolgus macaques after exposures to scrapie isolates. In the first study, the “Compton” scrapie isolate (derived from an English sheep) and serially propagated for 9 passages in goats did not transmit TSE in cynomolgus macaque, rhesus macaque or chimpanzee within 7 years following intracerebral challenge1; conversely, after 8 supplementary passages in conventional mice, this “Compton” isolate induced TSE in a cynomolgus macaque 5 years after intracerebral challenge, but rhesus macaques and chimpanzee remained asymptomatic 8.5 years post-exposure8. However, multiple successive passages that are classically used to select laboratory-adapted prion strains can significantly modify the initial properties of a scrapie isolate, thus questioning the relevance of zoonotic potential for the initial sheep-derived isolate. The same isolate had also induced disease into squirrel monkeys (new-world monkey)9. A second historical observation reported that a cynomolgus macaque developed TSE 6 years post-inoculation with brain homogenate from a scrapie-infected Suffolk ewe (derived from USA), whereas a rhesus macaque and a chimpanzee exposed to the same inoculum remained healthy 9 years post-exposure1. This inoculum also induced TSE in squirrel monkeys after 4 passages in mice. Other scrapie transmission attempts in macaque failed but had more shorter periods of observation in comparison to the current study. Further, it is possible that there are differences in the zoonotic potential of different scrapie strains.The most striking observation in our study is the extended incubation period of scrapie in the macaque model, which has several implications. Firstly, our observations constitute experimental evidence in favor of the zoonotic potential of c-scrapie, at least for this isolate that has been extensively studied32,33,34,35,36. The cross-species zoonotic ability of this isolate should be confirmed by performing duplicate intracerebral exposures and assessing the transmissibility by the oral route (a successful transmission of prion strains through the intracerebral route may not necessarily indicate the potential for oral transmission37). However, such confirmatory experiments may require more than one decade, which is hardly compatible with current general management and support of scientific projects; thus this study should be rather considered as a case report.Secondly, transmission of c-BSE to primates occurred within 8 years post exposure for the lowest doses able to transmit the disease (the survival period after inoculation is inversely proportional to the initial amount of infectious inoculum). The occurrence of scrapie 10 years after exposure to a high dose (25 mg) of scrapie-infected sheep brain suggests that the macaque has a higher species barrier for sheep c-scrapie than c-BSE, although it is notable that previous studies based on in vitro conversion of PrP suggested that BSE and scrapie prions would have a similar conversion potential for human PrP38.Thirdly, prion diseases typically have longer incubation periods after oral exposure than after intracerebral inoculations: since humans can develop Kuru 47 years after oral exposure39, an incubation time of several decades after oral exposure to scrapie would therefore be expected, leading the disease to occur in older adults, i.e. the peak age for cases considered to be sporadic disease, and making a distinction between scrapie-associated and truly sporadic disease extremely difficult to appreciate.Fourthly, epidemiologic evidence is necessary to confirm the zoonotic potential of an animal disease suggested by experimental studies. A relatively short incubation period and a peculiar epidemiological situation (e.g., all the first vCJD cases occurring in the country with the most important ongoing c-BSE epizootic) led to a high degree of suspicion that c-BSE was the cause of vCJD. Sporadic CJD are considered spontaneous diseases with an almost stable and constant worldwide prevalence (0.5–2 cases per million inhabitants per year), and previous epidemiological studies were unable to draw a link between sCJD and classical scrapie6,7,40,41, even though external causes were hypothesized to explain the occurrence of some sCJD clusters42,43,44. However, extended incubation periods exceeding several decades would impair the predictive values of epidemiological surveillance for prion diseases, already weakened by a limited prevalence of prion diseases and the multiplicity of isolates gathered under the phenotypes of “scrapie” and “sporadic CJD”.Fifthly, considering this 10 year-long incubation period, together with both laboratory and epidemiological evidence of decade or longer intervals between infection and clinical onset of disease, no premature conclusions should be drawn from negative transmission studies in cynomolgus macaques with less than a decade of observation, as in the aforementioned historical transmission studies of scrapie to primates1,8,9. Our observations and those of others45,46 to date are unable to provide definitive evidence regarding the zoonotic potential of CWD, atypical/Nor98 scrapie or H-type BSE. The extended incubation period of the scrapie-affected macaque in the current study also underscores the limitations of rodent models expressing human PrP for assessing the zoonotic potential of some prion diseases since their lifespan remains limited to approximately two years21,47,48. This point is illustrated by the fact that the recently reported transmission of scrapie to humanized mice was not associated with clinical signs for up to 750 days and occurred in an extreme minority of mice with only a marginal increase in attack rate upon second passage13. The low attack rate in these studies is certainly linked to the limited lifespan of mice compared to the very long periods of observation necessary to demonstrate the development of scrapie. Alternatively, one could estimate that a successful second passage is the result of strain adaptation to the species barrier, thus poorly relevant of the real zoonotic potential of the original scrapie isolate of sheep origin49. The development of scrapie in this primate after an incubation period compatible with its lifespan complements the study conducted in transgenic (humanized) mice; taken together these studies suggest that some isolates of sheep scrapie can promote misfolding of the human prion protein and that scrapie can develop within the lifespan of some primate species.In addition to previous studies on scrapie transmission to primate1,8,9 and the recently published study on transgenic humanized mice13, our results constitute new evidence for recommending that the potential risk of scrapie for human health should not be dismissed. Indeed, human PrP transgenic mice and primates are the most relevant models for investigating the human transmission barrier. To what extent such models are informative for measuring the zoonotic potential of an animal TSE under field exposure conditions is unknown. During the past decades, many protective measures have been successfully implemented to protect cattle from the spread of c-BSE, and some of these measures have been extended to sheep and goats to protect from scrapie according to the principle of precaution. Since cases of c-BSE have greatly reduced in number, those protective measures are currently being challenged and relaxed in the absence of other known zoonotic animal prion disease. We recommend that risk managers should be aware of the long term potential risk to human health of at least certain scrapie isolates, notably for lymphotropic strains like the classical scrapie strain used in the current study. Relatively high amounts of infectivity in peripheral lymphoid organs in animals infected with these strains could lead to contamination of food products produced for human consumption. Efforts should also be maintained to further assess the zoonotic potential of other animal prion strains in long-term studies, notably lymphotropic strains with high prevalence like CWD, which is spreading across North America, and atypical/Nor98 scrapie (Nor98)50 that was first detected in the past two decades and now represents approximately half of all reported cases of prion diseases in small ruminants worldwide, including territories previously considered as scrapie free... Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populationsEmmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, FrancePrion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),***is the third potentially zoonotic PD (with BSE and L-type BSE),***thus questioning the origin of human sporadic cases.We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.===============***thus questioning the origin of human sporadic cases***===============***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.==============***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.PRION 2016 TOKYOSaturday, April 23, 2016SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X onlineTaylor & FrancisPrion 2016 Animal Prion Disease Workshop AbstractsWS-01: Prion diseases in animals and zoonotic potentialJuan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,Natalia Fernandez-Borges a. and Alba Marin-Moreno a"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. FranceDietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.why do we not want to do TSE transmission studies on chimpanzees $5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.snip...R. BRADLEYTitle: 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.WEDNESDAY, JULY 19, 2017USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE and BANNED FEED USDA Detects a Case of Atypical Bovine Spongiform Encephalopathy in AlabamaUSDA Animal and Plant Health Inspection Service sent this bulletin at 07/18/2017 07:05 PM EDTUSDA Detects a Case of Atypical Bovine Spongiform Encephalopathy in AlabamaWashington, D.C., July 18, 2017 – The U.S. Department of Agriculture (USDA) announced an atypical case of Bovine Spongiform Encephalopathy (BSE), a neurologic disease of cattle, in an eleven-year old cow in Alabama. This animal never entered slaughter channels and at no time presented a risk to the food supply, or to human health in the United States.snip...endTUESDAY, AUGUST 7, 2018Unexpected prion phenotypes in experimentally transfused animals: predictive models for humans?TUESDAY, JULY 31, 2018***> USA CJD TSE Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined May 1, 2018 <***WEDNESDAY, JULY 04, 2018CREUTZFELDT-JAKOB DISEASE: GUIDELINES FOR SOCIAL WORKERS IN ENGLAND June 2018MONDAY, JUNE 18, 2018Ecuador Six Case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito***atypical spontaneous BSE in France LOL***FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$***so 20 cases of atypical BSE in France, compared to the remaining 40 cases in the remaining 12 Countries, divided by the remaining 12 Countries, about 3+ cases per country, besides Frances 20 cases. you cannot explain this away with any spontaneous BSe. ...TSSSunday, October 5, 2014France stops BSE testing for Mad Cow DiseaseThursday, March 24, 2016FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes***atypical spontaneous BSE in France LOL***FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$If you Compare France to other Countries with atypical BSE, in my opinion, you cannot explain this with ‘spontaneous’.Table 1: Number of Atypical BSE cases reported by EU Member States in the period 2001–2014 by country and by type (L- and H-BSE) (extracted from EU BSE databases on 1 July 2014). By 2015, these data might be more comprehensive following a request from the European Commission to Member States for re-testing and retrospective classification of all positive bovine isolates in the EU in the years 2003–2009BSE typeCountry 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013(a) 2014(a) TotalH-BSE Austria 1 1France(b) 1 2 3 1 2 2 2 2 15Germany 1 1 2Ireland 1 1 2 1 5The Netherlands 1 1Poland 1 1 2Portugal 1 1Spain 1 1 2Sweden 1 1United Kingdom 1 1 1 1 1 5Total 2 3 3 1 1 2 2 2 4 4 5 1 4 1 35L-BSE Austria 1 1 2Denmark 1 1France(b) 1 1 1 1 2 1 3 2 1 1 14Germany 1 1 2Italy 1 1 1 1 1 5The Netherlands 1 1 1 3Poland 1 2 2 1 2 1 2 1 12Spain 2 2United Kingdom 1 1 1 1 4Total 0 5 3 4 3 3 6 3 3 4 3 6 1 1 45Total Atypical cases (H + L)2 8 6 5 4 5 8 5 7 8 8 7 5 2 80(a): Data for 2013-2014 are incomplete and may not include all cases/countries reported.(b): France has performed extensive retrospective testing to classify BSE cases, which is probably the explanation for the higher number of Atypical BSE cases reported in this country.The number of Atypical BSE cases detected in countries that have already identified them seems to be similar from year to year. In France, a retrospective study of all TSE-positive cattle identified through the compulsory EU surveillance between 2001 and 2007 indicated that the prevalence of H-BSE and L-BSE was 0.35 and 0.41 cases per million adult cattle tested, respectively, which increased to 1.9 and 1.7 cases per million, respectively, in tested animals over eight years old (Biacabe et al., 2008). No comprehensive study on the prevalence of Atypical BSE cases has yet been carried out in other EU Member States. All cases of Atypical BSE reported in the EU BSE databases have been identified by active surveillance testing (59 % in fallen stock, 38 % in healthy slaughtered cattle and 4 % in emergency slaughtered cattle). Cases were reported in animals over eight years of age, with the exception of two cases (one H-BSE and one L-BSE) detected in Spain in 2011/2012. One additional case of H-BSE was detected in Switzerland in 2012 in a cow born in Germany in 2005 (Guldimann et al., 2012).***> Poland is Proof atypical BSE is NOT an old cow spontaneous disease...tss
we have seen the spontaneous BSE epidemic in France, what about the other HIGH INCIDENCE ATYPICAL BSE COUNTRY OF POLAND, another atypical spontaneous event of high incidence. how can this be blamed on a happenstance of nothing, i.e. old age? goes against all junk science to date on the spontaneous atypical BSE i.e.
> In 2015, the OIE determined that atypical BSE occurred spontaneously at a low rate in all cattle populations and would be excluded for BSE risk. ...
>Atypical BSE occurs in older cattle, usually 8 years of age or greater, and does not appear to be associated with contaminated feed. Like classic or sporadic CJD in humans, it seems to arise rarely and spontaneously.
POLAND ATYPICAL BSE AND SPORADIC CJD
we have seen the spontaneous BSE epidemic in France, what about the other HIGH INCIDENCE ATYPICAL BSE COUNTRY OF POLAND, another atypical spontaneous event of high incidence. how can this be blamed on a happenstance of nothing, i.e. old age? goes against all junk science to date on the spontaneous atypical BSE i.e.
> In 2015, the OIE determined that atypical BSE occurred spontaneously at a low rate in all cattle populations and would be excluded for BSE risk. ...
>Atypical BSE occurs in older cattle, usually 8 years of age or greater, and does not appear to be associated with contaminated feed. Like classic or sporadic CJD in humans, it seems to arise rarely and spontaneously.
POLAND ATYPICAL BSE AND SPORADIC CJD
Atypical status of bovine spongiform encephalopathy in Poland: a molecular typing study
Summary
The aim of this study was to analyze molecular features of protease-resistant prion protein (PrPres) in Western blots of BSE cases diagnosed in Poland with respect to a possible atypical status. Confirmed cases were analyzed by Western blotting with several monoclonal antibodies directed at N-terminal and core epitopes of prion protein (PrP). Most cases showed the classical glycoprofile characterized by the dominance of the di- over the monoglycosylated PrPres band, yielding di-/mono- ratios well above 2 and by reactivity with antibodies having their epitopes in bovine PrP region 110–242 (C-type cases). Surprisingly, seven cases of BSE were atypical. Six were classified as L-type based on a slightly lower molecular mass (Mr) of the non- glycosylated band with respect to C-types and a conspicuously low di-/mono- ratio of glycosylated PrPres bands approaching unity. One case was classified as H-type because of a higher Mr of PrPres bands on the blot when compared with C-type cases. A characteristic epitope of H-type PrPres occurred in the 101–110 region of PrP for which only antibody 12B2 had a sufficient affinity. The occurrence of atypical cases only in animals 9 years of age and older raises questions about the mechanisms of prion diseases and the origin of BSE.
Atypical status of bovine spongiform encephalopathy in Poland: a molecular typing study
M. P. Polak1, J. F. Zmudzinski1, J. G. Jacobs2, J. P. M. Langeveld2
1 National Veterinary Research Institute, Pulawy, Poland
2 Central Institute for Animal Disease Control (CIDC-Lelystad), Lelystad, The Netherlands Received 24 April 2007; Accepted 27 August 2007; Published online 26 September 2007 # Springer-Verlag 2007
snip...
Clarification of whether these atypical cases represent genuine strains of BSE would be accomplished by transmission studies in mice. Such studies have already been performed in France, Germany and Italy [3, 5, 14, 15]. For H-type cases in France, successful transmission was achieved in both wild-type, and transgenic mice expressing bovine and ovine PrPC. In Germany, successful transmission of both an L-type and an H-type case to transgenic mice overexpressing bovine PrPC has been described. PrPres from those mice was identical to the inoculum used in the study, proving the existence of distinct strains of BSE. All atypical features of those isolates were maintained in the inoculated mice, indicating the existence of several prion strains in cattle, or alternatively a possible evolution to a single BSE strain, as suggested from data obtained by Capobianco et al. with wild-type inbred mice [15]. This second hypothesis could fit with data from the United Kingdom, where over 180,000 cases of BSE were diagnosed by passive surveillance. British and European experience based on tissue analysis from clinically affected animals showed consistent characteristics of BSE agent not only on histological sections from cattle brains but also when inoculating mice, pointing to the existence of one uniform strain of BSE. Therefore, it is possible that a sporadic form of BSE present in the cattle population at a very low rate in the past could have spread to naive animals via contaminated meatand-bone meals. Spontaneous BSE, if it occurs, must be a very rare phenomenon. However, data for Poland, where 14% of all cases comprised an atypical form of BSE, seems to be in contradiction to this hypothesis. But when the average age of all positive cases in Poland is taken into account, BSE is generally found in older animals (mean age of 7.7). Analysis of the age structure of cattle in Poland in the period of 2002–2006 shows that 56–60% of all animals were 7 years old and above. A much larger number of cattle should be tested to get better insight into the real prevalence of atypical BSE. However, current tendencies based on economic analysis point to a decrease in the number of tests performed rather than expanding this scheme any further. It would be sensible to maintain a certain level of testing focused on the older age group to distinguish between a stable, thus sporadic-based, situation of BSE, or alternatively a fade-out, thus epidemic-based, situation. Exploring the subject of spontaneous BSE in the cattle population may be ceased for economic reasons, and it may never be known while this answer is in our reach thanks to great financial efforts in recent years.
Poland is Proof atypical BSE is NOT an old cow spontaneous disease...tss
Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide* (excluding the United Kingdom) Country/Year
Poland
89 90 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09 10 11 12 13 14 15 16
0 0 0 0 0 0 0 0 0 0 0 0 0 4f 5 11 19 10 9 5 4 2 1m 3 1 0 0 0
Source: USDA, APHIS, VS
What is the level of passenger traffic arriving in the United States from Poland?
A total of 188,946 passengers arrived at US airports on direct flights from Poland in fiscal year 2000.
An undetermined number of passengers arrived in the US from Poland via indirect flights.
Under APHIS-PPQ’s agricultural quarantine inspection monitoring, 451 air passengers from Poland were sampled for items of agricultural interest in fiscal year 2000.
Thirteen (13) of these passengers, or 2.9 percent, carried a total of 26.2 kg of meat items that could potentially harbor the pathogen(s) that cause BSE.
None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the US.
Source: US Department of Transportation, and APHIS-PPQ Agricultural Quarantine Inspection data base CEI’s plans for follow up: CEI has no plans to provide additional information on this situation. If you need more information or wish to comment, you may contact Judy Akkina at (970) 490-7852 or Carol Tuszynski at (970) 490-7893.
snip...see full text;
MONDAY, FEBRUARY 04, 2019
POLAND DETECTS BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION
Poland is Proof atypical BSE is NOT an old cow spontaneous disease...tss
FRIDAY, FEBRUARY 01, 2019
Poland Exported 5,500 Pounds of Meat From Sick Cows to EU, what about mad cow disease?
Poland is Proof atypical BSE is NOT an old cow spontaneous disease...tss
Wednesday, July 15, 2015Additional BSE TSE prion testing detects pathologic lesion in unusual brain location and PrPsc by PMCA only, how many cases have we missed?snip...see full text;MONDAY, MAY 09, 2016A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211 cattle following intracranial inoculationWEDNESDAY, AUGUST 15, 2018The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challengeSATURDAY, JUNE 01, 2019
Brazil reports another cases of mad cow disease atypical BSE TSE Prion
PLEASE BE ADVISED THERE IS NO SCIENTIFIC PROOF THAT ANY ATYPICAL BSE TSE PRION IS OF A SPONTANEOUS OLD AGE DISEASE, NOT CAUSED BY FEED, THIS IS FALSE AND UNPROVEN, IN FACT, ATYPICAL BSE OF THE L AND H TYPE ARE TRANSMISSIBLE BY ORAL ROUTE. THIS STATEMENT THAT ATYPICAL BSE IS A SPONTANEOUS EVENT CAUSED BY OLD AGE, CAUSED BY NOTHING, IS ABSOLUTELY A LIE, AND THE GOVERNMENT OF BRAZIL, AND OTHER GOVERNMENTS THAT PRODUCE SUCH STATEMENTS, KNOWS THIS IS AN UNPROVEN STATEMENT...TERRY SINGELTARY SR.
TUESDAY, MARCH 26, 2019
Joint Statement from President Donald J. Trump USA and President Jair Bolsonaro Brazil FOREIGN POLICY BSE TSE Prion aka mad cow disease
WEDNESDAY, MAY 29, 2019
The European Union Summary Report On Surveillance For The Presence Of Transmissible Spongiform Encephalopathies (TSE): The Situation In 2017
SATURDAY, JUNE 1, 2019
Traceability of animal protein byproducts in ruminants by multivariate analysis of isotope ratio mass spectrometry to prevent transmission of prion diseases
This is a comment from Terry Singeltary
The is a Comment on the Animal and Plant Health Inspection Service (APHIS) Notice: Concurrence with the World Organization for Animal Health's Risk Designation for Bovine Spongiform Encephalopathy For related information, Open Docket Folder Comment APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission
Greetings APHIS et al,
I would kindly like to comment on APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], and my comments are as follows, with the latest peer review and transmission studies as references of evidence.
THE OIE/USDA BSE Minimal Risk Region MRR is nothing more than free pass to import and export the Transmissible Spongiform Encephalopathy TSE Prion disease. December 2003, when the USDA et al lost it's supposedly 'GOLD CARD' ie BSE FREE STATUS (that was based on nothing more than not looking and not finding BSE), once the USA lost it's gold card BSE Free status, the USDA OIE et al worked hard and fast to change the BSE Geographical Risk Statuses i.e. the BSE GBR's, and replaced it with the BSE MRR policy, the legal tool to trade mad cow type disease TSE Prion Globally. The USA is doing just what the UK did, when they shipped mad cow disease around the world, except with the BSE MRR policy, it's now legal.
Also, the whole concept of the BSE MRR policy is based on a false pretense, that atypical BSE is not transmissible, and that only typical c-BSE is transmissible via feed. This notion that atypical BSE TSE Prion is an old age cow disease that is not infectious is absolutely false, there is NO science to show this, and on the contrary, we now know that atypical BSE will transmit by ORAL ROUTES, but even much more concerning now, recent science has shown that Chronic Wasting Disease CWD TSE Prion in deer and elk which is rampant with no stopping is sight in the USA, and Scrapie TSE Prion in sheep and goat, will transmit to PIGS by oral routes, this is our worst nightmare, showing even more risk factors for the USA FDA PART 589 TSE PRION FEED ban.
The FDA PART 589 TSE PRION FEED ban has failed terribly bad, and is still failing, since August 1997. there is tonnage and tonnage of banned potential mad cow feed that went into commerce, and still is, with one decade, 10 YEARS, post August 1997 FDA PART 589 TSE PRION FEED ban, 2007, with 10,000,000 POUNDS, with REASON, Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. you can see all these feed ban warning letters and tonnage of mad cow feed in commerce, year after year, that is not accessible on the internet anymore like it use to be, you can see history of the FDA failure August 1997 FDA PART 589 TSE PRION FEED ban here, but remember this, we have a new outbreak of TSE Prion disease in a new livestock species, the camel, and this too is very worrisome.
WITH the OIE and the USDA et al weakening the global TSE prion surveillance, by not classifying the atypical Scrapie as TSE Prion disease, and the notion that they want to do the same thing with typical scrapie and atypical BSE, it's just not scientific.
WE MUST abolish the BSE MRR policy, go back to the BSE GBR risk assessments by country, and enhance them to include all strains of TSE Prion disease in all species. With Chronic Wasting CWD TSE Prion disease spreading in Europe, now including, Norway, Finland, Sweden, also in Korea, Canada and the USA, and the TSE Prion in Camels, the fact the the USA is feeding potentially CWD, Scrapie, BSE, typical and atypical, to other animals, and shipping both this feed and or live animals or even grains around the globe, potentially exposed or infected with the TSE Prion. this APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], under it's present definition, does NOT show the true risk of the TSE Prion in any country. as i said, it's nothing more than a legal tool to trade the TSE Prion around the globe, nothing but ink on paper.
AS long as the BSE MRR policy stays in effect, TSE Prion disease will continued to be bought and sold as food for both humans and animals around the globe, and the future ramifications from friendly fire there from, i.e. iatrogenic exposure and transmission there from from all of the above, should not be underestimated. ...
Attachments (1) APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary...
WEDNESDAY, APRIL 24, 2019
USDA Announces Atypical Bovine Spongiform Encephalopathy Detection Aug 29, 2018 A Review of Science 2019
WEDNESDAY, APRIL 24, 2019
USDA Announces Atypical Bovine Spongiform Encephalopathy Detection Aug 29, 2018 A Review of Science 2019
THURSDAY, MARCH 14, 2019
USDA APHIS CDC FDA BSE TSE PRION UPDATE 2019
WEDNESDAY, JULY 03, 2019
chronic wasting disease cwd tse prion is like mad cow disease on steroids
SUNDAY, JULY 14, 2019
Korea Chronic Wasting Disease CWD TSE Prion additional cases were observed in red deer, sika deer, and their crossbred deer in 2010 and 2016, beyond that, anyone's guess
FRIDAY, JUNE 21, 2019
CJD TSE Prion cases update USA, Texas, Canada, and UK
MONDAY, JUNE 24, 2019
APHIS, FSIS, USDA, FDA, Transmissible Spongiform Encephalopathy TSE, BSE, CWD, Scrapie, Camel TSE Prion Disease, CJD Humans
Terry S. Singeltary Sr.
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