OIE Bovine spongiform encephalopathy ,Canada

Bovine spongiform encephalopathy ,Canada

Information received on 18/02/2015 from Dr Martine Dubuc, OIE Delegate for Canada, Chief Food Safety Officer Vice-President, Science Branch, Health Ministry, Canadian Food Inspection Agency, Ottawa, Canada

Summary

Report type	Immediate notification
Date of start of the event	04/02/2015
Date of pre-confirmation of the event	07/02/2015
Report date	18/02/2015
Date submitted to OIE	18/02/2015
Reason for notification	Reoccurrence of a listed disease
Date of previous occurrence	02/2011
Manifestation of disease	Sub-clinical infection
Causal agent	C-type BSE (classical)
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	ALBERTA ( Edmonton )
Total animals affected	Species Susceptible Cases Deaths Destroyed Slaughtered Cattle 1 0 1 0
Outbreak statistics	Species Apparent morbidity rate Apparent mortality rate Apparent case fatality rate Proportion susceptible animals lost* Cattle ** ** 0.00% ** * Removed from the susceptible population through death, destruction and/or slaughter; ** Not calculated because of missing information;

Epidemiology

Source of the outbreak(s) or origin of infection

Unknown or inconclusive

Epidemiological comments

On 4 February 2015, a private veterinarian visited a farm reporting a downer (non-ambulatory) cow and took samples as part of the national surveillance program for BSE in Canada. Rapid tests conducted at the provincial laboratory in Edmonton, Alberta were non-negative which was reported to Canadian Food Inspection Agency (CFIA) on 7 February 2015. These results were confirmed by CFIA's OIE BSE reference laboratory in Lethbridge, Alberta on 11 February 2015 as a classical case of BSE. The carcass was held and did not enter the human food or animal feed chains. The ongoing investigation has identified the herd of origin and the cow's birth date of March 2009. Movement controls and quarantines have been put in place pending completion of the investigation. The detection of a BSE case in Canada underscores the ongoing effectiveness of Canada's robust targeted BSE surveillance program. Canada continues to have in place an enhanced feed ban that includes strict controls for the exclusion of specified risk materials from the entire animal feed chain and fertilizers.

Control measures

Measures applied	Stamping out Quarantine Screening No vaccination No treatment of affected animals
Measures to be applied	No other measures

Diagnostic test results

Laboratory name and type	Alberta Agriculture and Rural Development ( Local laboratory )
Tests and results	Species Test Test date Result Cattle antigen (Ag) detection ELISA 07/02/2015 Positive
Laboratory name and type	BSE Reference Laboratory (CFIA-Lethbridge) ( OIE’s Reference Laboratory )
Tests and results	Species Test Test date Result Cattle antigen (Ag) detection ELISA 10/02/2015 Positive Cattle rapid tests 10/02/2015 Positive Cattle western blot 11/02/2015 Positive

Future Reporting

The event is continuing. Weekly follow-up reports will be submitted.

Encéphalopathie spongiforme bovine ,Canada

Information reçue le 18/02/2015 de Dr Martine Dubuc, OIE Delegate for Canada, Chief Food Safety Officer Vice-President, Science Branch, Health Ministry, Canadian Food Inspection Agency, Ottawa, Canada

Résumé

Type de rapport	Notification immédiate
Date de début de l’événement	04/02/2015
Date de pré-confirmation de l´événement	07/02/2015
Date du rapport	18/02/2015
Date d'envoi à l'OIE	18/02/2015
Raison de notification	Réapparition d’une maladie listée par l'OIE
Date de la précédente apparition de la maladie	02/2011
Manifestation de la maladie	Infection sub-clinique
Agent causal	ESB type-C (classique)
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	ALBERTA ( Edmonton )
Nombre total d'animaux atteints	Espèce(s) Sensibles Cas Morts Détruits Abattus Bovins 1 0 1 0
Statistiques sur le foyer	Espèce(s) Taux de morbidité apparent Taux de mortalité apparent Taux de fatalité apparent Proportion d'animaux sensibles perdus* Bovins ** ** 0.00% ** * Soustraits de la population sensible suite à la mort, à l´abattage et/ou à la destruction; ** Non calculé par manque de données;

Epidémiologie

Source du/des foyer(s) ou origine de l´infection

Inconnue ou incertaine

Autres renseignements épidémiologiques / Commentaires

Le 4 février 2015, un vétérinaire privé avait visité un élevage ayant informé d’une vache incapable de marcher (non ambulatoire) et avait prélevé des échantillons dans le cadre du Programme national de surveillance de l’ESB au Canada. Des tests de détection rapide effectués au Laboratoire provincial à Edmonton, Alberta, avaient été non négatifs, ce qui avait été notifié à l’Agence canadienne d’inspection des aliments (ACIA) le 7 février 2015. Ces résultats avaient été confirmés comme étant un cas d’ESB classique par le Laboratoire de référence de l’OIE pour l’ESB de l’ACIA à Lethbridge, Alberta, le 11 février 2015. La carcasse avait été retenue et n’avait entrée dans la chaine alimentaire humaine ou animale. L’enquête en cours a identifié le troupeau d’origine et mars 2009 comme étant la date de naissance de la vache. Des mesures de restriction des déplacements et de quarantaine sont en place jusqu’à l’achèvement de l’enquête. La détection d’un cas d’ESB au Canada met en évidence l’actuelle efficacité du solide programme de surveillance de l’ESB ciblé du Canada. Le Canada maintient en vigueur une interdiction renforcée sur les aliments du bétail qui comprend des contrôles stricts pour l'exclusion des matériels à risque spécifiés de la chaîne alimentaire animale et des engrais.

Mesures de lutte

Mesure de lutte appliquées	Abattage sanitaire Quarantaine Dépistage Pas de vaccination Aucun traitement des animaux atteints
Mesures à appliquer	Aucune autre mesure

Résultats des tests de diagnostics

Nom du laboratoire et type	Laboratoire de référence pour l'ESB (ACIA-Lethbridge) ( Laboratoire de référence de l’OIE )
Tests et résultats	Espèce(s) Test Date du test Résultat Bovins test de détection rapide 10/02/2015 Positif Bovins tests ELISA de détection de l'antigène 10/02/2015 Positif Bovins western blot 11/02/2015 Positif
Nom du laboratoire et type	Ministère de l'Agriculture et du Développement rural de l'Alberta ( Laboratoire local )
Tests et résultats	Espèce(s) Test Date du test Résultat Bovins tests ELISA de détection de l'antigène 07/02/2015 Positif

Rapports futurs

Cet événement se poursuit. Des rapports de suivi hebdomadaires devront être envoyés.

Encefalopatía espongiforme bovina ,Canadá

Información recibida el 18/02/2015 desde Dr Martine Dubuc, OIE Delegate for Canada, Chief Food Safety Officer Vice-President, Science Branch, Health Ministry, Canadian Food Inspection Agency, Ottawa, Canadá

Resumen

Tipo de informe	Notificación inmediata
Fecha del inicio del evento	04/02/2015
Fecha de pre-confirmación del evento	07/02/2015
Fecha del informe	18/02/2015
Fecha de envio del informe a la OIE	18/02/2015
Motivo de la notificación	Reaparición de una enfermedad de la Lista de la OIE
Fecha de la anterior aparición de la enfermedad	02/2011
Manifestación de la enfermedad	Infección sub-clínica
Agente causal	EEB tipo-C (clásica)
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	ALBERTA ( Edmonton )
Número total de animales afectados	Especies Susceptibles Casos Muertos Destruidos Sacrificados Bovinos 1 0 1 0
Estadística del foco	Especies Tasa de morbilidad aparente Tasa de mortalidad aparente Tasa de fatalidad aparente Proporción de animales susceptibles perdidos* Bovinos ** ** 0.00% ** * Descontados de la población susceptible a raíz de su muerte, destrucción o sacrificio; ** No calculado por falta de datos;

Epidemiología

Fuente del o de los focos u origen de la infección

Desconocida o no concluyente

Otros detalles epidemiológicos / comentarios

El 4 de febrero de 2015, un veterinario privado visitó una explotación que había informado de una vaca incapaz de andar (no ambulatoria) y tomó muestras dentro del Programa nacional de vigilancia de la EEB en Canadá. Las pruebas rápidas efectuadas en el Laboratorio provincial en Edmonton, Alberta, fueron no negativas, de lo cual se informó a la Agencia canadiense de inspección de alimentos (CFIA) el 7 de febrero de 2015. Esos resultados fueron confirmados como un caso de EEB clásica por el Laboratorio de referencia de la OIE para la EEB de la CFIA en Lethbridge, Alberta, el 11 de febrero de 2015. La carcasa se retuvo y no entró en la cadena alimentaria humana o animal. La investigación en curso ha identificado la manada de origen y marzo de 2009 como la fecha de nacimiento de la vaca. Están en vigor medidas de restricción de los desplazamientos y de cuarentena hasta la finalización de la investigación. La detección de un caso de EEB en Canadá pone de relieve la actual eficacia del sólido programa de vigilancia de la EEB específico de Canadá. Canadá mantiene en vigor una prohibición reforzada relativa a los piensos que incluye controles estrictos para la exclusión de los materiales de riesgo especificados de la cadena alimentaria animal y de los abonos.

Medidas de Control

Medidas implementadas	Sacrificio sanitario Cuarentena Tamizaje Vacunación: no Ningún tratamiento de los animales afectados
Medidas para implementar	Ninguna otra medida

Resultados de las pruebas diagnósticas

Nombre y tipo de laboratorio	Laboratorio de referencia para la EEB (CFIA-Lethbridge) ( Laboratorio de referencia de la OIE )
Pruebas y resultados	Especies Prueba Fecha de la prueba Resultados Bovinos prueba ELISA de detección del antígeno 10/02/2015 Positivo Bovinos prueba rápida 10/02/2015 Positivo Bovinos western blot 11/02/2015 Positivo
Nombre y tipo de laboratorio	Ministerio de agricultura y desarrollo rural de Alberta ( Laboratorio local )
Pruebas y resultados	Especies Prueba Fecha de la prueba Resultados Bovinos prueba ELISA de detección del antígeno 07/02/2015 Positivo

Informes futuros

El episodio continúa. Informes de seguimiento semanales serán enviados

<!--[endif]—>

 

http://www.oie.int/wahis_2/temp/reports/en_imm_0000017205_20150218_184604.pdf

 

Saturday, February 14, 2015

Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta

http://madcowusda.blogspot.com/2015/02/canadian-food-inspection-agency.html



Tuesday, February 17, 2015

 

 Could we spot the next BSE?, asks BVA President
 

 

http://bseusa.blogspot.com/2015/02/could-we-spot-next-bse-asks-bva.html

 

Friday, February 20, 2015

 

A BSE CANADIAN COW MAD COW UPDATE Transcript - Briefing (February 18, 2015)

 

http://transmissiblespongiformencephalopathy.blogspot.com/2015/02/a-bse-canadian-cow-mad-cow-update.html

 

 

 

TSS

 

Norway detects "probable" case of mad cow disease

Norway detects "probable" case of mad cow disease

 

Norway said Wednesday it had detected a "probable" case of mad cow disease but urged consumers not to panic as it may not be the same variant as the British 1990s epidemic.

 

A second positive test for bovine spongiform encephalopathy (BSE) on a 15-year-old cow reinforced suspicions that it had mad cow disease, the Norwegian Veterinary Institute said.

 

"We have a likely and strong suspicion of a possible variant of BSE," Bjoern Roethe Knudtsen of the Food and Safety Authority told public broadcaster NRK.

 

The authorities however said there was a distinction between the type of BSE caused by cows eating meat-based feed—banned in Europe since 2001 after the British epidemic—and an atypical version which has sporadically appeared in older cows in several European countries in recent years.

 

A definitive diagnosis can only be made by a European reference laboratory in Britain.

 

"We take this seriously and we are handling it as if our suspicion were confirmed," Food and Safety Authority official Solfrid Aamdal said in a statement.

 

The authority stressed that "more and more" BSE cases in Europe are of the atypical kind and that beef and milk consumption remains safe.

 

The cow's carcass, from a farm in west-central Norway, was destroyed and safety measures put in place for the rest of the herd.

 

http://medicalxpress.com/news/2015-01-norway-probable-case-mad-cow.html

 

http://www.vetinst.no/eng/Surveillance-programmes/Cattle-sheep-goats-deer-TSE

 

sporadic or spontaneous excuse for the atypical BSE cases have never been proven. it’s a myth. ...

 

atypical BSE a spontaneous event ???

 

if that's the case, then France is having one hell of an epidemic of atypical BSE, probably why they stopped testing for BSE $$$

 

Sunday, October 5, 2014

 

France stops BSE testing for Mad Cow Disease

 

http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html

 

Thursday, 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 investigations

 

http://bse-atypical.blogspot.com/2014/07/protocol-for-further-laboratory.html

 

>>> The generally older age of the identified H-BSE and L-BSE cases, and their apparently low prevalence in the population, suggest that these Atypical BSE forms could be arising spontaneously.

 

if that is the case, then FRANCE has an exceedingly high rate of spontaneous atypical BSE cases.

 

*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

 

http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143

 

SUMMARY REPORT CALIFORNIA ATYPICAL L-TYPE BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012 CALIFORNIA

 

Summary Report BSE 2012

 

Executive Summary

 

http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/summary-report-california-bovine.html

 

Saturday, August 4, 2012

 

Final Feed Investigation Summary - California atypical L-type BSE Case - July 2012

 

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

 

Saturday, August 4, 2012

 

Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation

 

http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/update-from-aphis-regarding-release-of.html

 

Tuesday, December 23, 2014

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION

 

http://madcowusda.blogspot.com/2014/12/fda-part-589-substances-prohibited-from.htm

 

Friday, December 5, 2014

 

***SPECIAL ALERT The OIE recommends strengthening animal disease surveillance worldwide

 

http://transmissiblespongiformencephalopathy.blogspot.com/2014/12/special-alert-oie-recommends.html

 

Sunday, December 28, 2014

 

*** Reverse Freedom of Information Act request rFOIA FSIS USDA APHIS TSE PRION aka BSE MAD COW TYPE DISEASE December 2014

 

http://madcowusda.blogspot.com/2014/12/reverse-freedom-of-information-act.html

 

http://www.aphis.usda.gov/import_export/animals/animal_import/downloads/bse_stakeholder_letter_february_4_2014.pdf

 

IN A NUT SHELL ;

 

(Adopted by the International Committee of the OIE on 23 May 2006)

 

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,

 

http://www.oie.int/eng/Session2007/RF2006.pdf

 

Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014

 

Transmissible Spongiform Encephalopathy TSE Prion Disease have now been discovered in a wide verity of species across North America. typical C-BSE, atypical L-type BASE BSE, atypical H-type BSE, atypical H-G BSE, of the bovine, typical and atypical Scrapie strains, in sheep and goats, with atypical Nor-98 Scrapie spreading coast to coast in about 5 years. Chronic Wasting Disease CWD in cervid is slowly spreading without any stopping it in Canada and the USA and now has mutated into many different strains. Transmissible Mink Encephalopathy TME outbreaks. These Transmissible Spongiform Encephalopathy TSE Prion Disease have been silently mutating and spreading in different species in North America for decades. The USDA, FDA, et al have assured us of a robust Triple BSE TSE prion Firewall, of which we now know without a doubt, that it was nothing but ink on paper. Since the 1997 mad cow feed ban in the USA, literally tons and tons of banned mad cow feed has been put out into commerce, never to return, as late as December of 2013, serious, serious breaches in the FDA mad cow feed ban have been documented. The 2004 enhanced BSE surveillance program was so flawed, that one of the top TSE prion Scientist for the CDC, Dr. Paul Brown stated ; Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

 

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

 

The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month. nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment. sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?

 

Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end

 

REFERENCES

 

Sunday, June 29, 2014

 

Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014

 

http://transmissiblespongiformencephalopathy.blogspot.com/2014/06/transmissible-spongiform-encephalopathy.html

 

Saturday, January 17, 2015

 

*** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease

 

http://creutzfeldt-jakob-disease.blogspot.com/2015/01/becky-lockhart-46-utahs-first-female.html

 

http://www.regulations.gov/#!documentDetail;D=APHIS-2014-0107-0003

 

who’s kidding whom $$$ i.e. USDA INC AND THE OIE

 

2014

 

***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

 

***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent. Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

 

snip...

 

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4213560/pdf/viruses-06-03766.pdf

 

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

 

*** Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2.

 

These data suggest that more than one BSEderived prion strain might infect humans;

 

***it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

 

snip...

 

These studies further strengthen the evidence that vCJD is caused by a BSE-like prion strain.

 

Also, remarkably, the key neuropathological hallmark of vCJD, the presence of abundant florid PrP plaques, can be recapitulated on BSE or vCJD transmission to these mice.

 

***However, the most surprising aspect of the studies was the finding that an alternate pattern of disease can be induced in 129MM Tg35 mice from primary transmission of BSE, with a molecular phenotype indistinguishable from that of a subtype of sporadic CJD. This finding has important potential implications as it raises the possibility that some humans infected with BSE prions may develop a clinical disease indistinguishable from classical CJD associated with type 2 PrPSc. This is, in our experience, the commonest molecular sub-type of sporadic CJD. In this regard, it is of interest that the reported incidence of sporadic CJD has risen in the UK since the 1970s (Cousens et al., 1997)...

 

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC136957/pdf/cdf653.pdf

 

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE.

 

***In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

 

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2

 

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

 

Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD

 

Date: Thu, 28 Nov 2002 10:23:43 -0000

 

From: "Asante, Emmanuel A" e.asante@ic.ac.uk

 

To: "'flounder@wt.net'" flounder@wt.net

 

Dear Terry,

 

I have been asked by Professor Collinge to respond to your request. I am a Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have attached a pdf copy of the paper for your attention.

 

Thank you for your interest in the paper.

 

In respect of your first question, the simple answer is, ***yes. As you will find in the paper, we have managed to associate the alternate phenotype to type 2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim any further sub-classification in respect of Heidenhain variant CJD or Vicky Rimmer's version. It will take further studies, which are on-going, to establish if there are sub-types to our initial finding which we are now reporting. The main point of the paper is that, as well as leading to the expected new variant CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc.

 

I hope reading the paper will enlighten you more on the subject. If I can be of any further assistance please to not hesitate to ask. Best wishes.

 

Emmanuel Asante

 

<>

 

____________________________________

 

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@ic.ac.uk (until 9/12/02) New e-mail: e.asante@prion.ucl.ac.uk (active from now)

 

____________________________________

 

Thursday, August 12, 2010

 

Seven main threats for the future linked to prions

 

First threat

 

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

 

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

 

Second threat

 

snip...

 

http://www.neuroprion.org/en/np-neuroprion.html

 

Monday, October 10, 2011

 

EFSA Journal 2011 The European Response to BSE: A Success Story

 

snip...

 

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far

 

*** but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

 

snip...

 

http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1

 

http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf

 

Tuesday, November 04, 2014

 

The pathological and molecular but not clinical phenotypes are maintained after second passage of experimental atypical bovine spongiform encephalopathy in cattle

 

http://bse-atypical.blogspot.com/2014/11/the-pathological-and-molecular-but-not.html

 

*** HUMAN MAD COW DISEASE nvCJD TEXAS CASE NOT LINKED TO EUROPEAN TRAVEL CDC ***

 

Sunday, November 23, 2014

 

*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June 2014 confirmed as USA case NOT European

 

the patient had resided in Kuwait, Russia and Lebanon. The completed investigation did not support the patient's having had extended travel to European countries, including the United Kingdom, or travel to Saudi Arabia. The specific overseas country where this patient’s infection occurred is less clear largely because the investigation did not definitely link him to a country where other known vCJD cases likely had been infected.

 

http://vcjd.blogspot.com/2014/11/confirmed-variant-creutzfeldt-jakob.html

 

Sunday, December 14, 2014

 

ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD strains, TSE prion aka Mad Cow Disease United States of America Update December 14, 2014 Report

 

http://transmissiblespongiformencephalopathy.blogspot.com/2014/12/alert-new-variant-creutzfeldt-jakob.html

 

 

 

TSS