Friday, November 28, 2014

BOVINE SPONGIFORM ENCEPHALOPATHY BSE AKA MAD COW DISEASE PORTUGAL CONFIRMED

BOVINE SPONGIFORM ENCEPHALOPATHY BSE AKA MAD COW DISEASE  PORTUGAL CONFIRMED

Subject: PRT 28-11-14 OIE Alert - Alerta - Alerte - Bovine spongiform encephalopathy - Encéphalopathie spongiforme bovine - Encefalopatía espongiforme bovina
 

Bovine spongiform encephalopathy ,Portugal

Information received on 28/11/2014 from Prof. Dr Álvaro Mendonça, Director General, Direcção Geral de Alimentação e Veterinária, Ministério da Agricultura E do Mar, Lisboa, Portugal

Summary

Report type
Immediate notification
Date of start of the event
07/11/2014
Date of pre-confirmation of the event
07/11/2014
Report date
28/11/2014
Date submitted to OIE
28/11/2014
Reason for notification
Reoccurrence of a listed disease
Date of previous occurrence
06/2012
Manifestation of disease
Sub-clinical infection
Causal agent
Resistant prion protein (PrPres) - Classical BSE
Nature of diagnosis
Laboratory (basic), Laboratory (advanced)
This event pertains to
the whole country

New outbreaks

Summary of outbreaks
Total outbreaks: 1
Outbreak Location
  • DSVR DO NORTE ( DUME, BRAGA )
Total animals affected
Species
Susceptible
Cases
Deaths
Destroyed
Slaughtered
Cattle
31
1
0
1
0
Outbreak statistics
Species
Apparent morbidity rate
Apparent mortality rate
Apparent case fatality rate
Proportion susceptible animals lost*
Cattle
3.23%
0.00%
0.00%
3.23%

* Removed from the susceptible population through death, destruction and/or slaughter;

Epidemiology

Source of the outbreak(s) or origin of infection
  • Unknown or inconclusive
Epidemiological comments
A work male animal of a national autochthonous breed born in 1998. He was tested under the surveillance plan on the category of emergency slaughter. Epidemiological investigation is ongoing.

Control measures

Measures applied
  • Quarantine
  • No vaccination
  • No treatment of affected animals
Measures to be applied
  • Modified stamping out

Diagnostic test results

Laboratory name and type
National Institute for Agrarian and Veterinary Research (INIAV) ( National laboratory )
Tests and results
Species
Test
Test date
Result
Cattle
rapid tests
07/11/2014
Positive
Cattle
western blot
14/11/2014
Positive

Future Reporting

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


Encéphalopathie spongiforme bovine ,Portugal

Information reçue le 28/11/2014 de Prof. Dr Álvaro Mendonça, Director General, Direcção Geral de Alimentação e Veterinária, Ministério da Agricultura E do Mar, Lisboa, Portugal

Résumé

Type de rapport
Notification immédiate
Date de début de l’événement
07/11/2014
Date de pré-confirmation de l´événement
07/11/2014
Date du rapport
28/11/2014
Date d'envoi à l'OIE
28/11/2014
Raison de notification
Réapparition d’une maladie appartenant à la liste de l'OIE
Date de la précédente apparition de la maladie
06/2012
Manifestation de la maladie
Infection sub-clinique
Agent causal
Protéine prion résistante (PrPres) - ESB classique
Nature du diagnostic
Tests élémentaires en laboratoire (i.e. parasitologie, bactériologie, mycologie, histopathologie), Tests approfondis en laboratoire (i.e. virologie, microscopie électronique, biologie moléculaire, immunologie)
Cet événement se rapporte à
tout le pays

Nouveaux foyers

Récapitulatif des foyers
Nombre total de foyers : 1
Localisation du foyer
  • DSVR DO NORTE ( DUME, BRAGA )
Nombre total d'animaux atteints
Espèce(s)
Sensibles
Cas
Morts
Détruits
Abattus
Bovins
31
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
3.23%
0.00%
0.00%
3.23%

* 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
  • Inconnue ou incertaine
Autres renseignements épidémiologiques / Commentaires
Un animal mâle de trait d’une race autochtone né en 1998. Il a été testé dans le cadre du plan de surveillance dans la catégorie d’abattage d’urgence. Une enquête épidémiologique est en cours.

Mesures de lutte

Mesure de lutte appliquées
  • Quarantaine
  • Pas de vaccination
  • Aucun traitement des animaux atteints
Mesures à appliquer
  • Abattage sanitaire partiel

Résultats des tests de diagnostics

Nom du laboratoire et type
Institut national de recherche vétérinaire et agraire (INIAV) ( Laboratoire national )
Tests et résultats
Espèce(s)
Test
Date du test
Résultat
Bovins
test de détection rapide
07/11/2014
Positif
Bovins
western blot
14/11/2014
Positif

Rapports futurs

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


Encefalopatía espongiforme bovina ,Portugal

Información recibida el 28/11/2014 desde Prof. Dr Álvaro Mendonça, Director General, Direcção Geral de Alimentação e Veterinária, Ministério da Agricultura E do Mar, Lisboa, Portugal

Resumen

Tipo de informe
Notificación inmediata
Fecha del inicio del evento
07/11/2014
Fecha de pre-confirmación del evento
07/11/2014
Fecha del informe
28/11/2014
Fecha de envio del informe a la OIE
28/11/2014
Motivo de la notificación
Reaparición de una enfermedad de la Lista de la OIE
Fecha de la anterior aparición de la enfermedad
06/2012
Manifestación de la enfermedad
Infección sub-clínica
Agente causal
Proteína priónica resistente (PrPres) - EEB clásica
Naturaleza del diagnóstico
Pruebas básicas de laboratorio (ej. parasitología, bacteriología, micología, histopatología), Pruebas de diagnóstico de laboratorio avanzadas (ej. virología, microscopía electrónica, biología molecular e inmunología)
Este evento concierne
todo el país

Nuevos focos

Resumen de los focos
Número total de focos: 1
Localización del foco
  • DSVR DO NORTE ( DUME, BRAGA )
Número total de animales afectados
Especies
Susceptibles
Casos
Muertos
Destruidos
Sacrificados
Bovinos
31
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
3.23%
0.00%
0.00%
3.23%

* 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
  • Desconocida o no concluyente
Otros detalles epidemiológicos / comentarios
Un animal macho de trabajo de una raza autóctona nacido en 1998. Se realizaron pruebas de laboratorio en el marco del plan de vigilancia en la categoría de sacrificio de urgencia. Se está llevando a cabo una investigación epidemiológica.

Medidas de Control

Medidas implementadas
  • Cuarentena
  • Vacunación: no
  • Ningún tratamiento de los animales afectados
Medidas para implementar
  • Sacrificio sanitario parcial

Resultados de las pruebas diagnósticas

Nombre y tipo de laboratorio
Instituto nacional de investigación veterinaria y agraria (INIAV) ( Laboratorio nacional )
Pruebas y resultados
Especies
Prueba
Fecha de la prueba
Resultados
Bovinos
prueba rápida
07/11/2014
Positivo
Bovinos
western blot
14/11/2014
Positivo

Informes futuros

El episodio continúa. Informes de seguimiento semanales serán enviados



<!--[endif]—>
 

BSE PORTUGAL

Country/Year
 
Portugal

 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

 Portugal

0 1b 1b 1b 3b 12 15 31 30 127 159 149a 110 86 133 92a 46 33 14 18 8 6 5 2 0 1c

a Portugal: includes 1 imported case.

b Imported case(s).

c Portugal - Data as of 28 November 2014.

tss


http://www.oie.int/en/animal-health-in-the-world/bse-specific-data/number-of-reported-cases-worldwide-excluding-the-united-kingdom/
 

 
From: TSS
 
Subject: vCJD 1st probable case in Portugal diagnosed in 12 year old boy Date: June 23, 2005 at 1:19 pm PST
 
First probable case of vCJD reported in Portugal
 
Direcção de Serviços de Informação e Análise, Direcção-Geral da Saúde, Lisboa, Portugal
 
The Portuguese Direcção-Geral da Saúde (Directorate-Genearl for Health) was recently informed of the first probable case of variant Creutzfeldt-Jakob disease (vCJD) in Portugal, with laboratory evidence (tonsil biopsy) [1]. The patient is 12 year old boy currently living with his parents and receiving specialist medical care. The case meets the European and Allied Countries Collaborative Study Group of CJD’s (EUROCJD) definition of probable vCJD (http://www.eurocjd.ed.ac.uk/def.htm) and has been confirmed by the United Kingdom’s National CJD Surveillance Unit. The patient does not have a history of travel to the United Kingdom.
 
References: Direcção-Geral da Saúde. Variante da Doença de Creutzfeldt-Jakob - Comunicado. Press release, 9 June 2005. (http://www.dgsaude.pt/upload/membro.id/ficheiros/i007107.pdf)
 
 
Ministério da Saúde
 
Direcção-Geral da Saúde
 
Comunicado
 
A Direcção-Geral da Saúde foi recentemente notificada da existência de um
 
primeiro caso provável de variante da Doença de Creutzfeldt-Jakob (vDCJ), com
 
evidência laboratorial.
 
Como é do conhecimento público, casos desta doença têm sido diagnosticados em
 
diversos países da União Europeia.
 
Trata-se de um doente jovem do sexo masculino, residente no respectivo domicílio
 
com os pais, cuja identidade deve ser protegida por razões técnicas e éticas, tanto
 
mais que se trata de doença que não se transmite através de contactos pessoa a
 
pessoa, pelo que não constitui qualquer risco para os seus conviventes ou contactos.
 
O doente encontra-se com assistência médica especializada.
 
Não existem indícios de quaisquer outros casos desta doença nem de quadros
 
clínicos suspeitos de variante da Doença de Creutzfeldt-Jakob.
 
Atendendo ao desejo expresso pela família não serão prestadas declarações
 
adicionais.
 
Lisboa, 2005-06-09
 
O Alto-Comissário e Director-Geral da Saúde
 
Professor Doutor José Pereira Miguel
 
 
TSS
 
 
----- Original Message -----
 
From: Terry S. Singeltary Sr.
 
 
Sent: Friday, December 11, 2009 9:06 PM
 
Subject: Sporadic Creutzfeldt-Jakob disease causing a 2-years slowly progressive isolated dementia
 
 Sporadic Creutzfeldt-Jakob disease causing a 2-years slowly progressive isolated dementia
 
Journal Behavioural Neurology Publisher IOS Press ISSN 0953-4180 (Print) 1875-8584 (Online) Issue Volume 21, Number 3-4 / 2009 DOI 10.3233/BEN-2009-0238 Pages 175-179 Subject Group Neurosciences
 
Authors Álvaro Machado1, Manuel Ribeiro2, Margarida Rodrigues1, Carla Ferreira1, Inês Baldeiras3, M. Helena Ribeiro3, Isabel Santana4, Rui Almeida5, Lígia Castro6, Stirling Carpenter6 1Neurology Department, Hospital de São Marcos, Braga, Portugal 2Neuroradiology Department, Hospital de São Marcos, Braga, Portugal 3Neurochemistry Laboratory, Hospitais da Universidade de Coimbra, Coimbra, Portugal 4Neurology Department, Hospitais da Universidade de Coimbra, Coimbra, Portugal 5Neurosurgery Department, Hospital de São Marcos, Braga, Portugal 6Pathology Department, Hospital de São João, Porto, Portugal
 
Abstract
 
A 47-year-old woman was seen for progressive behavioural and cognitive disturbances slowly evolving over a 1-year period. Neuropsychological evaluation disclosed moderate to severe impairment of all cortical functions. Besides this no other clinical abnormality was found. MRI diffusion weighted imaging disclosed hyperintense cortical lesions in a ribbon-like fashion, with restricted diffusivity. EEG showed no periodic sharp waves and CSF examination was normal, including protein 14.3.3. She was heterozygote on codon 129. Her cognitive function continued to decline and she was readmitted for further investigation at the 24th month of disease. Again no ataxia or involuntary movements were observed. MRI disclosed widespread hyperintense lesions over the entire cortex and, for the first time, also caudato-putaminal hyperintensity in T2-weighted images. EEG again failed to show periodic activity. Stereotactic biopsy disclosed moderate spongiform changes, astrocytosis and perivacuolar staining with prion-directed antibodies. Western blot analysis revealed prion type 2 mobility pattern. We discuss the clinical significance of this case: as dementia was the sole finding, and this was slowly-evolving over a 2-year period, MRI findings were the key factor suggesting a prion disease in a woman that otherwise would probably be diagnosed with a primary degenerative dementia.
 
Keywords Creutzfeldt-Jacob disease, DWI, MV2, MRI, MRS, SPECT
 
 
 Thursday, December 3, 2009
 
FINAL REPORT OF A MISSION CARRIED OUT IN PORTUGAL FROM 11 TO 20 MAY 2009 IN ORDER TO EVALUATE MEASURES CONCERNING BOVINE SPONGIFORM ENCEPHALOPATHY
 
Eurosurveillance, Volume 10, Issue 25, 23 June 2005 Articles Direcção de Serviços de Informação e Análise, Direcção-Geral da Saúde1
 
--------------------------------------------------------------------------------
 
Citation style for this article: Direcção de Serviços de Informação e Análise, Direcção-Geral da Saúde.
 
First probable case of vCJD reported in Portugal.
 
Euro Surveill. 2005;10(25):pii=2732. Available online:
 
 
 Date of submission:
 
--------------------------------------------------------------------------------
 
First probable case of vCJD reported in Portugal
 
Direcção de Serviços de Informação e Análise, Direcção-Geral da Saúde, Lisboa, Portugal
 
The Portuguese Direcção-Geral da Saúde (Directorate-Genearl for Health) was recently informed of the first probable case of variant Creutzfeldt-Jakob disease (vCJD) in Portugal, with laboratory evidence (tonsil biopsy) [1]. The patient is 12 year old boy currently living with his parents and receiving specialist medical care. The case meets the European and Allied Countries Collaborative Study Group of CJD’s (EUROCJD) definition of probable vCJD (http://www.eurocjd.ed.ac.uk/def.htm) and has been confirmed by the United Kingdom’s National CJD Surveillance Unit. The patient does not have a history of travel to the United Kingdom.
 
References: 1.Direcção-Geral da Saúde. Variante da Doença de Creutzfeldt-Jakob - Comunicado. Press release, 9 June 2005.
 
 
 
 Ministério da Saúde
 
Direcção-Geral da Saúde
 
Comunicado
 
A Direcção-Geral da Saúde foi recentemente notificada da existência de um
 
primeiro caso provável de variante da Doença de Creutzfeldt-Jakob (vDCJ), com
 
evidência laboratorial.
 
Como é do conhecimento público, casos desta doença têm sido diagnosticados em
 
diversos países da União Europeia.
 
Trata-se de um doente jovem do sexo masculino, residente no respectivo domicílio
 
com os pais, cuja identidade deve ser protegida por razões técnicas e éticas, tanto
 
mais que se trata de doença que não se transmite através de contactos pessoa a
 
pessoa, pelo que não constitui qualquer risco para os seus conviventes ou contactos.
 
O doente encontra-se com assistência médica especializada.
 
Não existem indícios de quaisquer outros casos desta doença nem de quadros
 
clínicos suspeitos de variante da Doença de Creutzfeldt-Jakob.
 
Atendendo ao desejo expresso pela família não serão prestadas declarações
 
adicionais.
 
Lisboa, 2005-06-09
 
O Alto-Comissário e Director-Geral da Saúde
 
Professor Doutor José Pereira Miguel
 
 
 New vCJD case in Portugal The Portuguese Ministry of Health has announced a second suspected case of vCJD. The report is in Portuguese, titled “Segundo caso provável de variante da Doença de Creutzfeldt-Jacob,” dated February, 2, 2007. Direcção-Geral da Saúde
 
 
 Comunicado A Direcção-Geral da Saúde recebeu no dia 19 de Fevereiro de 2007 a notificação, com evidência laboratorial, de um segundo caso provável de variante da Doença de Creutzfeldt-Jacob (vDCJ) numa jovem portuguesa residente no Continente. Por imposição ética não são fornecidos pormenores sobre o caso ora notificado. Informa-se, todavia, que não existem riscos para a Saúde Pública, incluindo para os contactos próximos da doente, uma vez que a doença em causa não se transmite de pessoa a pessoa. Lisboa, 20 de Fevereiro de 2007
 
snip
 
COMUNICADO
 
Em Fevereiro de 2007 foi notificado um caso de possível variante de Creutzfeldt- Jakob numa jovem de 14 anos.
 
Hoje, dia 12 de Fevereiro de 2009, comunica-se a morte dessa jovem. Durante todo este período a jovem doente foi acompanhada por uma equipa composta por pessoal médico e de enfermagem do Serviço Nacional de Saúde e por técnicos da Segurança Social.
 
Comunica-se, também, que relativamente a este caso estão a ser observadas todas as normas nacionais e europeias previstas para estas situações.
 
Este é o segundo óbito registado em Portugal com diagnóstico da variante da doença de Creutzfeldt-Jakob, não havendo outros casos notificados até à presente data.
 
De forma a proteger a família enlutada, não serão divulgados mais pormenores relativos ao óbito de hoje.
 
Lisboa, 12 de Fevereiro de 2009 O Director-Geral da Saúde Francisco George
 
 
 R.I.P. ...TSS
 
J Neurol Neurosurg Psychiatry 2008;79:180-182 doi:10.1136/jnnp.2007.128389
 
Short report
 
Variant Creutzfeldt–Jacob disease: the second case in Portugal and in the same geographical region
 
Á Machado1, H Soares2, H Antunes2, Z Magalhães3, C Ferreira1, I Baldeiras4, M H Ribeiro4, I Santana5, J Ramalheira6, L Castro7, S Carpenter7 + Author Affiliations
 
1Neurology Department, Hospital de São Marcos, Braga, Portugal 2Adolescent Unit, Paediatrics Department, Hospital de São Marcos, Braga, Portugal 3Neuroradiology Department, Hospital de São Marcos, Braga, Portugal 4Neurochemistry Laboratory, Hospitais da Universidade de Coimbra, Coimbra, Portugal 5Neurology Department, Hospitais da Universidade de Coimbra, Coimbra, Portugal 6Neurophysiology Department, Hospital Geral de Santo António, Porto, Portugal 7Pathology Department, Hospital de São João, Porto, Portugal Dr Álvaro Machado, Serriço de Neurologia, Hospital de Sâo Marcos, Largo Carlos Amarante, Apartado 2242, 4700-Braga, Portngal; alvmac@gmail.com Received 26 June 2007 Revised 14 August 2007 Accepted 17 August 2007 Published Online First 31 August 2007
 
Abstract
 
We present the second variant Creutzfeldt–Jacob patient in the same district of northwest Portugal as was previously reported. A 14-year-old previously healthy girl had unexplained pain in the left leg, as well as psychiatric disturbances. This was shortly followed by progressive cognitive impairment, ataxia and generalised choreoatethosis. Neuropsychological assessment revealed severe frontal and medial temporal dysfunction, the posterior cortices being spared. An electroencephalogram was normal. CSF 14.3.3 protein was slightly positive. Magnetic resonance imaging showed the “hockey stick sign” and hyperintensities in the periaquedutal grey matter and in the right parietal cortex, the last with restriction to water molecule movement. SPECT revealed perfusion defects in the left frontotemporal and right parietal regions. PRNP gene sequencing showed no mutations, the patient being homozygous to methionine in codon 129. Five months after onset, immunocytochemical and immunoblotting analysis confirmed deposition of prion protein and a PrP4t electrophoretic pattern. The patient never travelled outside Portugal or received blood transfusions. She had surgical herniorrhaphy in 1998 (when catgut was used) and 2003. This is the second case in Portugal in a 2-year period and 20 km apart from each other, with no known common exposure apart from ingestion of cow meat. We discuss these case peculiarities and underline its epidemiological significance.
 
 
The numbers of tested and positive cattle in each category in each EU Member State are published and updated regularly. Although the number of cases in the EU was increasing in 2001 and 2002, since 2003 the number of cases in the EU altogether is decreasing (EC, 2003; 2004). A total of over 10 million cattle were tested in the EU in 2004. Of these, 686 cattle were positive. Spain and Portugal were the only countries in the EU 15 Member States with an increase of cases in 2003, and Germany in 2004.
 
 
 Portugal BSE CASES
 
2001-110
 
2002-86
 
2003-133
 
2004-92
 
2005-13*
 
* Portugal - Data as of 31 March 2005.
 
 
 51 CASES IN 2005 TOTAL
 
 
 GBR IV: confirmed at a higher level United Kingdom, Portugal
 
 
 A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features
 
 
 Eurosurveillance, Volume 1, Issue 6, 01 June 1996
 
Creutzfeldt-Jakob disease: results of an inquiry in the fifteen Member States of the European Union
 
see Portugal ;
 
 
Subject: Final report Portugal ON TSEs
 
Date: June 13, 2003 at 7:42 am PST
 
Veterinary Inspections
 
Final report of a mission carried out in Portugal from 25 to 29 November 2002 in order to evaluate the implementation of certain EU measures aimed at the eradication, control and prevention of Transmissible Spongiform Encephalopathies (TSEs) (8636/2002)
 
 
TSS
 
From: TSS (216-119-162-40.ipset44.wt.net)
 
Subject: Portugal mad cow cases edge up in first half 2002 !
 
Date: July 19, 2002 at 1:07 pm PST
 
Portugal mad cow cases edge up in first half 2002
 
Portugal (July 17, 2002) - Portugal detected 49 cases of mad cow disease in the first half of 2002, eight more than a year ago, but officials said the figures were not directly comparable.
 
An official at the Agriculture Ministry's veterinary department noted that data for the first six months of 2001 did not include 60,000 cows slaughtered under European Union orders, many of which may have had mad cow disease.
 
Of the 49 positive cases of Bovine Spongiform Encephalopathy (BSE), or "mad cow" disease, 36 were detected using early screening, which had been stepped up since last year.
 
"This shows that were are identifying positive cases earlier. The number of cases from animals with suspicous symptoms is down," the official said.
 
Portugal conducted more than 42,000 screening tests in the first six months of 2002 for BSE, up from 8,500 a year ago.
 
Scientists have linked beef from animals infected with BSE to the spread of variant Creutzfeld-Jakob disease, which has killed more than 100 people, mostly in Britain.
 
A year ago, the European Union lifted a three-year embargo on beef exports from Portugal, which had been imposed due to high levels of BSE.
 
A ban on live animals remained in place, and only de-boned beef can be exported, although Portugal never was a major beef exporter.
 
Portuguese state news agency Lusa reported that the country's only plant licenced to export beef had received no export orders since the embargo was lifted.
 
Source : Reuters
 
 
TSS
 
From: TSS (216-119-130-168.ipset10.wt.net)
 
Subject: BSE aka madcow disease update SPAIN/PORTUGAL
 
Date: November 24, 2000 at 12:29 pm PST
 
BOVINE SPONGIFORM ENCEPHALOPATHY IN SPAIN
 
(Disease never previously reported).
 
Emergency report
 
Translation of a fax from Dr Juan José Badiola, Faculty of Veterinary Medicine, Zaragoza (National Reference Centre for Transmissible Spongiform Encephalopathies), forwarded to the OIE Central Bureau by Dr Quintiliano Pérez Bonilla, Director General of Animal Production, Ministry of Agriculture, Fisheries and Food, Madrid:
 
Report date: 22 November 2000.
 
Nature of diagnosis: clinical and post-mortem.
 
Identification of the sample:
 
The sample, taken from a five-year-old bovine (identification number LU-21492-C) was collected under the programme for monitoring bovine spongiform encephalopathy (BSE) in the Autonomous Community of Galicia. The animal had presented neurological symptoms.
 
The sample was submitted by the Animal Health and Production Laboratory(1).
 
The sample consisted of medulla oblongata and brain stem preserved in 10% formol, which were duly sent for histopathological and immunohistochemical examination.
 
Anatomopathological report:
 
A. Histopathological findings:
 
- Obex: intense vacuolation in neuropil of the dorsal nucleus of the vagus, solitary tract and spinal tract of the trigeminal nerve. Vacuolation in the pericaryon of the neurons of the dorsal nucleus of the vagus.
 
- Bridge: intense vacuolation in the pericaryon of the neurons of the vestibular nuclei.
 
- Mesencephalon: intense generalized vacuolation in neuropil.
 
B. Immunohistochemical findings:
 
An immunohistochemical examination was made of the obex and the following was observed:
 
- Positive marker located in the neuropil: deposits of the pathological protein PrPSc, which appears to be most marked in the dorsal nucleus of the vagus, the solitary tract, the spinal tract of the trigeminal nerve and the reticular formation.
 
- Positive marker located in the pericaryon of neurons and axons located principally in the dorsal nucleus of the vagus.
 
Conclusion:
 
The histopathological and immunohistochemical findings show that this animal was suffering from bovine spongiform encephalopathy.
 
This result was confirmed by the VLA Weybridge(2) (OIE Reference Laboratory for BSE).
 
(1) Laboratorio de Sanidad e Producción Animal (registration number of the laboratory: 6518-1), Dirección Xeral de Produccion Agropecuaria, Consellería de Agricultura, Ganadería e Política Agroalimentaria, Xunta de Galicia.
 
(2) Veterinary Laboratories Agency, Weybridge, New Haw, Addlestone, Surrey, United Kingdom.
 
* * *
 
BOVINE SPONGIFORM ENCEPHALOPATHY IN PORTUGAL in the Azores islands
 
Text of a fax received on 23 November 2000 from Dr Rui Marques Leitão, Director General of Veterinary Services, Ministry of Agriculture, Rural Development and Fisheries, Lisbon:
 
Report date: 23 November 2000.
 
A case of bovine spongiform encephalopathy (BSE) was detected in a Holstein-Friesian cow (identification number I339582) of the island of São Miguel, Azores.
 
This cow was the subject of emergency slaughter on 2 October 2000 in the abattoir at Ponta Delgada, São Miguel, in the scope of an emergency slaughter and was sampled under the BSE monitoring programme.
 
Diagnosis:
 
The sample underwent histopathological examination and immunocytochemistry at the National Laboratory of Veterinary Research, Lisbon, with positive results on 22 November.
 
Epidemiology:
 
Epidemiological investigations indicate that the cow was born on 23 September 1995, and was imported to São Miguel on 27 October 1998 from a European country.
 
 
From: TSS (216-119-136-2.ipset16.wt.n
 
Subject: PORTUGAL UPDATE BSE SURVEILLANCE Date: Mon, 17 Jul 2000 10:43:56 -0700 From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy To: BSE-L@uni-karlsruhe.de
 
######### Bovine Spongiform Encephalopathy #########
 
Report on a veterinary mission to Portugal with regard to certain protective measures against BSE, and in particular, implementation of Commission Decision 98/653/EC and Commission Decision 98/272/EC (13 to 17 March 2000)
 
III. CONCLUSIONS.
 
III.2 TSE epidemio surveillance
 
The tendency to consider suspect for BSE only when an animal shows 'typical' nervous disorders can result in an underestimation of the presence of BSE in the Regions visited. In particular in the slaughterhouses visited the staff simply excluded the possibility to take in consideration other symptoms, and this could be explained in a lack of training specifically tailored for the different tasks of the veterinary services, as already recommended in previous inspections (XXIV/1061/99 AND DG(SANCO)/1227/1999);
 
The necessity to extend the monitoring program also to the emergency slaughtered animals and to the fallen stock was accepted after the last mission (DG (SANCO)1227/1999) but it is not yet operational, mainly because the necessity to allocate financial resources.
 
The number of animals sampled in the framework of Commission Decision 98/272/EC is very low in the Regions visited and it does not comply both with criteria and the minimum number of samples decided by DGV for the specific Regions, even though the DRAs have the information to properly sample most of the sub populations of bovines.
 
There is a clear tendency to focus on the suspect only as first step of the eradication measure, and not also in the framework of the BSE surveillance. This could result in a lack of sampling of the other sub- population foreseen in Commission Decision 98/272/EC.
 
The BSE case not notified to DRA's and DGV has to be considered a lack of investigation. The absence of corrective measures against the farmer does not allow to prevent similar problems in the future...
 
 
From: Terry S. Singeltary Sr. (216-119-138-112.ipset18.wt.net)
 
Subject: PORTUGAL DISCOVERS MAD COW CASE IN IMPORTED DANISH COW
 
Date: April 13, 2000 at 6:42 am PST
 
 Portugal Discovers Mad Cow Case In Imported Danish Cow
 
COPENHAGEN (Agence France-Presse) -- Portuguese authorities have uncovered a case of so-called "mad cow" disease in a cow imported from Denmark, the Danish Veterinary and Food Administration said in a statement Wednesday.
 
Although it could not be ruled out that the cow had caught bovine spongiform encephalopathy (BSE) before it left Denmark, the case did not constitute a risk to food safety there, said the organisation.
 
The cow was born in Nordvestjylland, Denmark in March 1992 and was exported to Portugal in October 1994 from a herd of only 16, none of which was still in Denmark.
 
While Portugal has reported 13 new cases of BSE so far this year and registered 170 cases in 1999, the first case of BSE in a native Danish cattle herd was announced in late February.
 
A number of countries immediately announced bans on Danish beef and Denmark pulled beef products from its shelves.
 
The Danish government admitted March 1 it had failed to follow EU guidelines fast enough to prevent the first case.
 
Denmark had previously been criticised by the European Union for acting half-heartedly on recommendations to prevent BSE, with the government proving unwilling to test dead or sick animals, or introduce new methods in abattoirs.
 
Until this year, Denmark believed its cattle were free from BSE, the only previous case having occurred in 1992 -- an import from Scotland that aroused little domestic concern.
 
Denmark has two million head of cattle.
 
 
this is just getting ridiculous, when are country's going to start taking this BSE seriously. the longer country's keep insisting they do not have BSE, without looking, the longer this disease is going to spread, and kill not only animals, but humans as well. will they ever learn???
 
kind regards, Terry S. Singeltary Sr., Bacliff, Texas USA
 
From: TSS (216-119-143-94.ipset23.wt.net) Subject: FINAL REPORT OF A MISSION CARRIED OUT IN PORTUGAL FROM 16 TO 20 FEBRUARY 2004 CONCERNING BSE Date: July 14, 2004 at 2:25 pm PST
 
 FINAL REPORT OF A MISSION CARRIED OUT IN PORTUGAL FROM 16 TO 20 FEBRUARY 2004 CONCERNING PROTECTIVE MEASURES AGAINST BOVINE SPONGIFORM ENCEPHALOPATHY (BSE)
 
DG(SANCO)/7214/2004 -- MR Final
 
EXECUTIVE SUMMARY
 
This report describes the outcome of a mission carried out by the Food and Veterinary Office (FVO) in Portugal, from 16 to 20 February 2004.
 
The overall objective of the mission was to evaluate the implementation of certain protective measures against BSE and the system of identification, registration and traceability of live bovine animals. More in particular, it concerned the measures put in place, and then application, to give effect to EU rules in force for the prevention, control and eradication of certain TSEs, as laid down in the Regulation (EC) No 999/2001 of the European Parliament and of the Council, as amended, and specifically addressing:
 
- both active and passive BSE epidemio-surveillance,
 
- removal and handling of Specified Risk Material (SRM),
 
- the prohibition of feeding processed animal proteins to farmed animals, and exceptions applicable to this total feed ban.
 
In terms of scope, the mission focused on bovine identification and registration, BSE epidemio-surveillance in bovine animals, removal and disposal of SRM and the feed ban, including the control and supervisory measures in place.
 
The report overall concludes that BSE surveillance in mainland Portugal has further progressed due to improvements in the bovine identification and registration system, providing more reliable data and allowing a better assessment of the rate of testing in the different sub-populations in all regions. However, sampling of all fallen stock remained problematic in 2003, in particular in Acores. Corrective measures have already been taken, notably as regards improved supervision, resulting in noticeable progress in 2004. Systems in place for removal, processing and disposal of SRM meet EU requirements, although deficiencies were found in their supervision. As regards the feed ban, and despite improvements, some shortcomings still remain in relation with planning and implementation of official controls.
 
As a potential consequence of the incomplete sampling of falling stock in the 2nd semester of 2003, calculation of the true overall BSE incidence in Portugal would be hindered. However, from January 2004, the problem seems to be largely solved, except/or Acores. Additionally, the current control plan for the total feed ban cannot fully demonstrate the efficacy of the latter.
 
The report makes a number of recommendations addressed to the Portuguese competent authorities, aimed at rectifying the identified shortcomings and/or further enhancing the implementing and control measures in place.
 
 
- Report on the Assessment of the Geographical BSE - Risk of Portugal (July 2000)
 
snip...
 
- 44 - 5. Conclusion on the Geographical BSE-Risk (GBR) 5.1 The current GBR The current geographical BSE-risk (GBR) level is IV: BSE is confirmed in domestic cattle at a higher level. • The observed incidence of clinical cases over the last 12 months (16 June 1999-15 June 2000) was 215.8 per Million adult cattle. This figure is generated by a surveillance system that was only depending on notification of suspects until recently (end 99, early 2000) some active components, targeting fallen stock, were introduced. 5.2 The expected development of the GBR • Assuming that measures in place continue to be appropriately implemented and no new external challenge occurs, the GBR will decrease over time, even if the incidence figures will only decrease sharply once the birth cohorts before 1999 will have left the system. The degree of compliance with the measures will determine the rate of decrease of the GBR. Report on the assessment of the Geographical BSE-risk of PORTUGAL July 2000 - 45 - 5.3 Recommendations for influencing the future GBR • Ensuring optimal compliance with the introduced measures is of utmost importance for supporting the decline of the GBR. All additional measures that could enhance the stability of the system further are helpful. Strengthening the active surveillance, targeting at-risk sub-populations such as fallen stock and emergency slaughter will be most useful to better assess the current extend and to monitor the development of the epidemic. By improving the ability of the system to exclude animals being at risk to incubate BSE it will also accelerate the decline of the epidemic.
 
 
 
-------- Original Message --------
 
Subject: New Scrapie Strain Identified in Portugal 2004
 
Date: Mon, 29 Mar 2004 21:59:04 –0600
 
From: "Terry S. Singeltary Sr." To: bse-l
 
Voluntary Report – public distribution Date: 1/20/2004 GAIN Report Number: PO4002 PO4002 Portugal Sanitary/Phytosanitary/Food Safety New Scrapie Strain Identified in Portugal 2004
 
Approved by: Lloyd Fleck U.S. Embassy Prepared by: Leonor Ramos
 
Report Highlights: The British reference laboratory at Weybridge has confirmed two TSE cases in Portuguese sheep and has issued a provisional diagnosis of a new variant of scrapie. 1 USD = €0.78.
 
Includes PSD Changes: No Includes Trade Matrix: No Unscheduled Report Madrid [SP1] [PO]
 
FIRST CASES OF SCRAPIE FOUND IN PORTUGAL
 
The Government of Portugal has just announced the identification of two cases of a new variant of scrapie in two sheep, of which one raised in a farm in northern Portugal, and the other imported from Spain for slaughter. Both cases had been detected three months ago by routine brain testing of slaughtered sheep and goats over 18 months of age; these tests are required by the national Plan of Surveillance and Control of the “epizootic sheep tremor” set up in coordination with the EU. According to Portuguese veterinary authorities (DGV), neither of the animals, aged 24 and 26 months, showed any clinical sign of the disease. After the quick Eliza tests carried out at the slaughter plant revealed the presence of Transmittable Spongiform Encephalopathy (TSE) samples from the animals were tested in the Portuguese National Laboratory. After confirmation, they were remitted to the Weybridge reference laboratory in the U.K. where, according to the EU legislation, all cases of new diseases must also be confirmed. The Weybridge laboratory confirmed TSE but reported that the brain lesions observed did now show the typical pattern of scrapie. The laboratory later issued a provisional diagnosis that defined these two cases as a new scrapie variant. In order to be able to give a greater assurance of the diagnosis, the Weybridge laboratory will carry out further analysis.
 
These are the first cases of scrapie detected in Portugal. As a consequence, local sanitary authorities have implemented the measures required by the TSE legislation in force: sequestration of the small farm where the Portuguese-born animal was raised and slaughter and brain sampling of all co-habitants. The results of all these tests have been negative, but the authorities continue to research all relevant epidemiological features, to trace back the Portuguese case to its origins, and to determine all relevant circumstances to understand the pathology’s occurrence. According to local veterinary sources, there are other suspect cases under investigation.
 
The announcement that these are scrapie and not BSE cases has also soothed local public opinion. The Consumers Defense Association (DECO) has already reported that it is too soon to recommend any precaution regarding consumption of sheep and goat meat. Nevertheless, it also has encouraged the authorities to clarify potential risks to consumers, emphasizing the need for the Portuguese Food Safety Agency to come into force.
 
Sheep and goat production is the least significant livestock activity in Portugal, accounting for a value of production estimated at €166 million in 2002, compared to €380 million for cattle, €380 million for poultry and more than €430 million for hog production. The 2.92 million head national sheep inventory is located on 71,000 farms, most of which concentrated in the Alentejo province.
 
For analysis of official documentation and updates on the new scrapie variant in Portugal, see the EU Commission website, at
 
 
1 USD = €0.78
 
UNCLASSIFIED USDA FOREIGN AGRICULTURAL SERVICE
 
 
TSS
 
 -------- Original Message --------
 
Subject: SCRAPIE IN PORTUGAL Emergency report 13 February 2004
 
Date: Fri, 13 Feb 2004 23:35:51 –0600
 
From: "Terry S. Singeltary Sr." To: BSE-l
 
SCRAPIE IN PORTUGAL
 
(Disease never reported before in Portugal).
 
Emergency report
 
Translation of information received on 10 February 2004 from Dr Carlos Agrela Pinheiro, Director General of Veterinary Services, Ministry of Agriculture, Rural Development and Fisheries, Lisbon:
 
Date of the report: 9 February 2004.
 
Nature of diagnosis: laboratory.
 
Outbreaks:
 
Location No. of outbreaks Viana do Castelo district, Paredes de Coura municipality, Cunha parish, Cerdeira locality (in north-western Portugal) 1
 
Total number of animals in the outbreak:
 
species susceptible cases deaths destroyed slaughtered ovi 31* 1 0 31 0
 
* adult animals (aged over 18 months): 16 females and 1 male; progeny (aged under 6 months): 9 females and 5 males.
 
Diagnosis:
 
A. Laboratory where diagnosis was made: VLA Weybridge, United Kingdom (OIE Reference Laboratory for scrapie).
 
B. Diagnostic tests used:
 
- ELISA(1) rapid test: positive result on 30 September 2003;
 
- western blot: negative result;
 
- histopathological and immunohistochemical tests: positive results on 31 December 2003.
 
Epidemiology:
 
A. Source of agent / origin of infection: unknown.
 
B. Other epidemiological details: all animals on the affected farm were screened for transmissible spongiform encephalopathies and gave negative results to the rapid test.
 
Control measures: stamping out.
 
(1) ELISA: enzyme-linked immunosorbent assay
 
* * *
 
 
TSS
 
 New Scrapie Strain Identified in Portugal
 
2004
 
Approved by:
 
Lloyd Fleck U.S. Embassy
 
Prepared by:
 
Leonor Ramos
 
Report Highlights:
 
The British reference laboratory at Weybridge has confirmed two TSE cases in Portuguese sheep and has issued a provisional diagnosis of a new variant of scrapie. 1 USD = €0.78.
 
FIRST CASES OF SCRAPIE FOUND IN PORTUGAL
 
The Government of Portugal has just announced the identification of two cases of a new variant of scrapie in two sheep, of which one raised in a farm in northern Portugal, and the other imported from Spain for slaughter. Both cases had been detected three months ago by routine brain testing of slaughtered sheep and goats over 18 months of age; these tests are required by the national Plan of Surveillance and Control of the “epizootic sheep tremor” set up in coordination with the EU. According to Portuguese veterinary authorities (DGV), neither of the animals, aged 24 and 26 months, showed any clinical sign of the disease. After the quick Eliza tests carried out at the slaughter plant revealed the presence of Transmittable Spongiform Encephalopathy (TSE) samples from the animals were tested in the Portuguese National Laboratory. After confirmation, they were remitted to the Weybridge reference laboratory in the U.K. where, according to the EU legislation, all cases of new diseases must also be confirmed. The Weybridge laboratory confirmed TSE but reported that the brain lesions observed did now show the typical pattern of scrapie. The laboratory later issued a provisional diagnosis that defined these two cases as a new scrapie variant. In order to be able to give a greater assurance of the diagnosis, the Weybridge laboratory will carry out further analysis.
 
These are the first cases of scrapie detected in Portugal. As a consequence, local sanitary authorities have implemented the measures required by the TSE legislation in force: sequestration of the small farm where the Portuguese-born animal was raised and slaughter and brain sampling of all co-habitants. The results of all these tests have been negative, but the authorities continue to research all relevant epidemiological features, to trace back the Portuguese case to its origins, and to determine all relevant circumstances to understand the pathology’s occurrence. According to local veterinary sources, there are other suspect cases under investigation.
 
The announcement that these are scrapie and not BSE cases has also soothed local public opinion. The Consumers Defense Association (DECO) has already reported that it is too soon to recommend any precaution regarding consumption of sheep and goat meat. Nevertheless, it also has encouraged the authorities to clarify potential risks to consumers, emphasizing the need for the Portuguese Food Safety Agency to come into force.
 
Sheep and goat production is the least significant livestock activity in Portugal, accounting for a value of production estimated at €166 million in 2002, compared to €380 million for cattle, €380 million for poultry and more than €430 million for hog production. The 2.92 million head national sheep inventory is located on 71,000 farms, most of which concentrated in the Alentejo province.
 
For analysis of official documentation and updates on the new scrapie variant in Portugal, see the EU Commission website.
 
TSS
 
 
-------- Original Message --------
 
Subject: Identification of putative atypical scrapie in sheep in Portugal
 
Date: Wed, 13 Oct 2004 15:58:20 –0500
 
 From: "Terry S. Singeltary Sr." To: Bovine Spongiform Encephalopathy
 
 Identification of putative atypical scrapie in sheep in Portugal
 
Leonor Orge1,2, Alexandre Galo1, Carla Machado1, Carla Lima1, Cristina Ochoa1, João Silva1, Manuel Ramos1 and J. Pedro Simas2,3
 
1 Laboratório Nacional de Investigação Veterinária, Lisboa, Portugal 2 Instituto Gulbenkian de Ciência, Rua da Quinta Grande 6, 2780-156 Oeiras, Portugal 3 Laboratório de Microbiologia, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
 
Correspondence J. Pedro Simas jpsimas@igc.gulbenkian.pt
 
Experimental transmission of bovine spongiform encephalopathy to sheep has prompted the implementation of a surveillance plan of scrapie in small ruminants by the European Union in all member states. Since its start over 30 000 animals have been tested, and the first seven cases of sheep with detectable PrPres deposition in the central nervous system have been identified in Portugal. Notably, the pattern of PrPres distribution in the brainstem was different from that previously described for scrapie and consistent in all seven animals. Moreover, the profile of the electrophoretic mobility of PrPres after proteinase K treatment was equivalent in all cases analysed but distinct from that observed for scrapie. Notably, four animals had genotypes rarely associated with scrapie, including one animal homozygous for A136R154R171. There were no cases found to exhibit vacuolation, a pattern of PrPres distribution or PrPres electrophoretic mobility corresponding to scrapie. These data reveal a putative atypical scrapie strain in Portugal not linked to specific Prnp genotypes.
 
 
Subject: FSE: FIRST CONFIRMED CASE REPORTED IN PORTUGAL AND POTENTIAL MAD CAT ESCAPES LAB IN USA
 
Date: August 9, 2007 at 2:27 pm PST
 
 DIA-45 FELINE SPONGIFORM ENCEPHALOPATHY: FIRST CONFIRMED CASE REPORTED IN PORTUGAL
 
J.F. Silva1, J.J. Correia, 1 J. Ribeiro2, S. Carmo2 and L.Orge3
 
1 Faculdade de Medicina Veterinária (UTL), Lisbon, Portugal 2 Clínica Veterinária Ani+, Queluz, Portugal 3 Laboratório Nacional de Investigação Veterinária, Unidade de BSE, Lisbon, Portugal
 
Feline spongiform encephalopathy (FSE), affecting domestic and captive feline species, is a prion disease considered to be related to bovine spongiform encephalopathy (BSE). Here we report the first case diagnosed in Portugal, highlighting the neuroapthological findings. In 2004 a 9-year old intact female Siamese cat was referred with chronic progressive behavioural changes, polydipsia, gait abnormalities and episodes of hypersalivation. Clinical signs progressed to tetraparesis and dementia and euthanasia was performed. At necropsy, brain and spinal cord had no significative changes. Tissue samples from brain, cerebellum, brainstem and spinal cord were collected for histopathology and immunohistochemistry for detection of PrPres. Histology revealed neuropil and neuronal perikarion vacuolation in several areas of the central nervous system together with gliosis and cell rarefaction at the granular layer of the cerebellum. Immunohistochemical detection of PrPres showed a strong and widespread PrPres accumulation as granular and linear deposits as well as associated with some neurons. These findings are supportive of FSE. To the authors knowledge this is the first confirmed case of FSE reported in Portugal.
 
 
 
MAD COW PAGE PORTUGAL
 
 
Monday, August 8, 2011 Susceptibility of Domestic Cats to CWD Infection
 
Oral.29: Susceptibility of Domestic Cats to CWD Infection
 
Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†
 
Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu
 
Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness.
 
*** Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.
 
 
 
AD.63:
 
Susceptibility of domestic cats to chronic wasting disease
 
Amy V.Nalls,1 Candace Mathiason,1 Davis Seelig,2 Susan Kraft,1 Kevin Carnes,1 Kelly Anderson,1 Jeanette Hayes-Klug1 and Edward A. Hoover1 1Colorado State University; Fort Collins, CO USA; 2University of Minnesota; Saint Paul, MN USA
 
Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD-infected deer brain. At 40 and 42 mo post-inoculation, two IC-inoculated cats developed signs consistent with prion disease, including a stilted gait, weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail tremors, and ataxia, and progressed to terminal disease within 5 mo. Brains from these two cats were pooled and inoculated into cohorts of cats by IC, PO, and intraperitoneal and subcutaneous (IP/SC) routes. Upon subpassage, feline-adapted CWD (FelCWD) was transmitted to all IC-inoculated cats with a decreased incubation period of 23 to 27 mo. FelCWD was detected in the brains of all the symptomatic cats by western blotting and immunohistochemistry and abnormalities were seen in magnetic resonance imaging, including multifocal T2 fluid attenuated inversion recovery (FLAIR) signal hyper-intensities, ventricular size increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 IP/SQ and 2 of 4 PO inoculared cats have developed abnormal behavior patterns consistent with the early stage of feline CWD.
 
*** These results demonstrate that CWD can be transmitted and adapted to the domestic cat, thus raising the issue of potential cervid-to- feline transmission in nature.
 
 
www.landesbioscience.com
 
PO-081: Chronic wasting disease in the cat— Similarities to feline spongiform encephalopathy (FSE)
 
 
 
FELINE SPONGIFORM ENCEPHALOPATHY FSE
 
 
 
Monday, November 3, 2014
 
USA CJD TSE PRION UNIT, TEXAS, SURVEILLANCE UPDATE NOVEMBER 2014
 
National Prion Disease Pathology Surveillance Center Cases Examined1 (October 7, 2014)
 
***6 Includes 11 cases in which the diagnosis is pending, and 19 inconclusive cases;
 
***7 Includes 12 (11 from 2014) cases with type determination pending in which the diagnosis of vCJD has been excluded.
 
***The sporadic cases include 2660 cases of sporadic Creutzfeldt-Jakob disease (sCJD),
 
***50 cases of Variably Protease-Sensitive Prionopathy (VPSPr)
 
***and 21 cases of sporadic Fatal Insomnia (sFI).
 
 
Sunday, November 23, 2014
 
*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June 2014 confirmed as USA case NOT European
 
 
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis *video*
 
 
Jeff Schwan, sporadic cjd, clustering, and BSE aka mad cow type disease, is there a link ? *video*
 
 
1997-11-10: Panorama - The british disease *video*
 
 
Sunday, September 6, 2009
 
MAD COW USA 1997 *video*
 
 
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
 
 
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
 
 
Tuesday, August 12, 2014
 
MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014
 
 
Thursday, October 02, 2014
 
[Docket No. APHIS-2013-0064] Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy
 
 
Saturday, August 14, 2010
 
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY
 
 
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
 
 
 
 
 
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 ;
 
 
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
 
 
 
TSS

Monday, May 5, 2014

Brazil BSE Mad Cow disease confirmed OIE 02/05/2014

 

 

 From: Terry S. Singeltary Sr.

 

Sent: Monday, May 05, 2014 11:54 AM

 

To: BSE-L BSE-L

 

Cc: CJD-L ; CJDVOICE CJDVOICE ; bloodcjd bloodcjd

 

Subject: Brazil BSE Mad Cow disease confirmed OIE 02/05/2014

 

Bovine spongiform encephalopathy, Brazil

 

Information received on 02/05/2014 from Dr Figueiredo Marques Guilherme Henrique , Director, Departamento de Saúde Animal , Ministério da Agricultura, Pecuaria e Abastecimento , Brasilia, Brazil

 

Summary

 

Report type Immediate notification

 

Date of start of the event 19/03/2014

 

Date of pre-confirmation of the event 14/04/2014

 

Report date 02/05/2014

 

Date submitted to OIE 02/05/2014

 

Reason for notification Reoccurrence of a listed disease

 

Date of previous occurrence 19/12/2010

 

Manifestation of disease Sub-clinical infection

 

Causal agent Prion

 

Nature of diagnosis Laboratory (advanced)

 

This event pertains to the whole country

 

 New outbreaks (1)

 

 

 

Outbreak 1 Porto Esperidião, MATO GROSSO

 

Date of start of the outbreak 19/03/2014

 

Outbreak status Resolved (01/05/2014)

 

Epidemiological unit Farm

 

Affected animals

 

Species Susceptible Cases Deaths Destroyed Slaughtered

 

Cattle 1177 1 0 50 0

 

Buffaloes 11 0 0 0 0

 

Affected population A 12-year-old female bovine tested during emergency slaughter at the slaughterhouse. The animal was born and raised on a full-cycle beef farm on extensive grazing, with a population of 1,177 cattle and 11 buffaloes.

 

 Summary of outbreaks Total outbreaks: 1

 

Total animals affected

 

Species Susceptible Cases Deaths Destroyed Slaughtered

 

Cattle 1177 1 0 50 0

 

Buffaloes 11 0 0 0 0

 

Outbreak statistics

 

Species Apparent morbidity rate Apparent mortality rate Apparent case fatality rate Proportion susceptible animals lost*

 

Cattle 0.08% 0.00% 0.00% 4.25%

 

Buffaloes 0.00% 0.00% - 0.00%

 

*Removed from the susceptible population through death, destruction and/or slaughter

 

Epidemiology

 

Source of the outbreak(s) or origin of infection •Unknown or inconclusive

 

Epidemiological comments As part of the Brazilian surveillance system for Bovine Spongiform Encephalopathy (BSE), the prion marker was identified on 14 April 2014 in a 12-year-old female bovine sent for emergency slaughter because she was found fallen at her arrival at the slaughterhouse following some problems during transport. The animal was born and raised in the same full-cycle beef farm on extensive grazing. Meat and other products from this animal did not enter the food chain and there was no risk for human population. Tracing back animal movements since 2000, it was established that some animals from the birth cohort of this animal had been moved to 10 other properties in 3 municipalities in the state of Mato Grosso. During the epidemiological investigation, 49 animals from the cohort, which did not show clinical signs of the disease, were destroyed. Samples of nervous tissue were taken from the cohort animals and tested for BSE at the National Laboratory and all were negative on 1 May 2014. All control measures according to the OIE Terrestrial Animal Health Code have already been applied in order to close the outbreak and only the results of the typing tests carried out at the Reference Laboratory at Weybridge (United Kingdom) are pending.

 

Control measures

 

Measures applied •Quarantine •Screening •Modified stamping out •No vaccination •No treatment of affected animals

 

Measures to be applied •No other measures

 

Diagnostic test results

 

Laboratory name and type Species Test Test date Result

 

National Agricultural Laboratory (LANAGRO/PE) (National laboratory) Cattle immunohistochemical test 14/04/2014 Positive

 

National Agricultural Laboratory (LANAGRO/PE) (National laboratory) Cattle immunohistochemical test 01/05/2014 Negative

 

Animal Health and Veterinary Laboratories Agency (AHVLA), Weybridge, United Kingdom (OIE’s Reference Laboratory) Cattle immunohistochemical test 01/05/2014 Positive

 

Future Reporting

 

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

 

 Map of outbreak locations

 


 

 

>>>Atypical BSE, or “mad cow” disease, is a form of the prion disease not associated with the animal’s consumption of feed.<<<

 

 

don't you just love it when the officials just make stuff up $$$

 

 

*** What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”

 

 

 The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health. "(The agency) has no foundation on which to base that statement.”

 

 


 

 

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

 

 

 In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.

 

 


 

 

 

 *** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.

 

 

 

 *** It also suggests a similar cause or source for atypical BSE in these countries. ***

 

 

 P.9.21

 

 

 Molecular characterization of BSE in Canada

 

 

 Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada

 

 

 Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle. Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres.

 

 

 Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.

 

 

 Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.

 

 

 Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. *** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries. ***

 

 

 see page 176 of 201 pages...tss

 

 


 

 

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

 


 

 

 P.4.23

 

 

 Transmission of atypical BSE in humanized mouse models

 

 Liuting 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), USA

 

 Background: 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 largely 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.

 


 

 

 P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS

 

 

 Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA

 

 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). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C. *** The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.

 

 III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)

 


 

 

 UPDATE

 

 I ask Professor Kong ; Thursday, December 04, 2008 3:37 PM

 

 Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

 

 ''IS 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 ;

 

 .....snip

 

 ''As 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 USA END...TSS

 

 

 Thursday, December 04, 2008 2:37 PM

 

 "we have found that H-BSE can infect humans."

 

 personal communication with Professor Kong. ...TSS

 

 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.

 


 


 

 

 please see below from PRION2013 ;

 

 

 *** This study imply the possibility that the novel BSE prions with high virulence in cattle will be emerged during intraspecies transmission.

 

 AD.56: The emergence of novel BSE prions by serial passages of H-type BSE in bovinized mice

 

 Kentaro Masujin, Naoko Tabeta, Ritsuko Miwa, Kohtaro Miyazawa, Hiroyuki Okada, Shirou Mohri and Takashi Yokoyama National Institute of Animal Health; Tsukuba, Japan

 

 H-type bovine spongiform encephalopathy (BSE) is an atypical form of BSE, and has been detected in several European countries, and North America. Transmission studies of H-type BSE led to the emergence of the classical BSE (C-BSE) phenotypes during passages in inbred wild type and bovinized PrP-overexpressing transgenic mice. In this study, we conducted serial passages of Canadian H-type BSE isolate in bovinized PrP-overexpressing transgenic mice (TgBoPrP). H-type BSE isolate was transmitted to TgBoPrP with incubation periods of 320 ± 12.2 d at primary passage. The incubation period of 2nd and 3rd passage were constant (~= 220 d), no clear differences were observed in their biological and biochemical properties. However, at the forth passage, 2 different BSE phenotypes were confirmed; one is shorter survival times (109 ± 4 d) and the other is longer survival times. TgBoPrP mice with longer incubation period showed the H-type phenotype of PrPsc profile and pathology. However, those of shorter incubation period were different phenotypes from previously existed BSE prions (C-BSE, L-type BSE, and H-type BSE).

 

 *** This study imply the possibility that the novel BSE prions with high virulence in cattle will be emerged during intraspecies transmission.

 


 

 www.landesbioscience.com

 

 please see ;

 

 Thursday, August 15, 2013

 

 The emergence of novel BSE prions by serial passages of H-type BSE in bovinized mice

 


 

 

 LET'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-BSEalabama In 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, 2010

 

 BSE 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 2009

 


 

 

 P.9.21 Molecular characterization of BSE in Canada

 

 Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada

 

 Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.

 

 Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres.

 

 Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis. Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.

 

 Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries.

 


 

 

 Saturday, August 14, 2010

 

 ***BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 

 *** (see mad cow feed in COMMERCE IN ALABAMA...TSS)

 


 

 

 Sunday, December 15, 2013

 

 FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE

 


 

 

 kind regards, terry

 

 

UPDATE SEE R-CALF CONCERNS

 

 Brazil's Second Reported BSE Case Raises Food Safety Concerns

 

 Billings, Mont. - On Monday the World Organization for Animal Health (OIE) provided notice that Brazil confirmed its second case of bovine spongiform encephalopathy (BSE), this time in a 12-year-old Brazilian cow. While the notice states that none of the meat or other products from the infected cow entered the food chain, a recent audit report by the U.S. Department of Agriculture (USDA) reveals that Brazil has not been complying with BSE safeguard measures required by the United States.

 

 A recent audit report

 


 

 

 by the USDA Food Safety and Inspection Service (FSIS) sent to the Brazilian government on April 16, 2014, reveals that Brazil has not been consistently implementing the United States' mandatory requirement that all specified risk materials (SRMs) from cattle be excluded from the human food chain as a condition for allowing Brazil to export beef to the United States.

 

 Specifically, the audit found that beginning in early 2007, the Brazilian government relaxed its SRM removal policies by issuing a notice that removed the skull, trigeminal ganglia, vertebral column, and dorsal root ganglia in cattle 30 months of age or older from the list of SRMs that must be removed at slaughter. The tissues improperly removed from the list of SRMs by the Brazilian government are tissues known to harbor the BSE agent in infected cattle.

 

 United States food safety inspectors confirmed that Brazil was not routinely removing all high-risk tissues as required for countries that export to the United States.

 

 Despite Brazil's failure to meet U.S. food safety standards, FSIS officials nevertheless determined that Brazil "continues to meet FSIS equivalence criteria at an adequate level for this component (the SRM removal component)."

 

 R-CALF USA CEO Bill Bullard said these facts demonstrate the need to fully enforce the U.S. country-of-origin labeling (COOL) law. "Only with COOL can consumers choose to avoid purchasing their food from countries with questionable food safety systems," he said.

 

 Bullard also said these facts along with USDA's current plan to begin importing beef from Brazilian states that are not free of foot-and-mouth disease (FMD) is deeply troubling.

 

 "More and more the USDA is demonstrating its unwillingness to prioritize food safety and animal health above its politically motivated trade relations goals," he concluded.

 

 # # #

 

 R-CALF USA (Ranchers-Cattlemen Action Legal Fund, United Stockgrowers of America) is the largest producer-only cattle trade association in the United States. It is a national, nonprofit organization dedicated to ensuring the continued profitability and viability of the U.S. cattle industry. For more information, visit www.r-calfusa.com or, call 406-252-2516.

 

 

Thursday, April 24, 2014

 

Brazil investigates possible BSE mad cow case

 


 

 

Thursday, September 26, 2013

 

Brazil evaluate the implementation of health rules on animal by-products and derived products SRM BST TSE PRION aka MAD COW DISEASE

 


 

 

Friday, December 07, 2012

 

ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012

 


 

 

Wednesday, December 19, 2012

 

Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil

 


 

 

 

 

TSS

Thursday, April 24, 2014

Brazil investigates possible BSE mad cow case

Brazil investigates possible BSE case

 

 

Reuters | Updated: 04/24/2014

 

 

 

Brazil is investigating a potential case of atypical mad cow disease, the agriculture ministry said on Thursday, just over a year after several countries banned Brazilian beef imports when a similar case of the disease was confirmed.

 

A routine inspection at a slaughterhouse in Mato Grosso state found an animal that veterinarians suspect of having a neurological problems, a ministry spokesman said in an e-mail.

 

Laboratory tests are under way and atypical bovine spongiform encephalopathy (BSE), or mad cow disease, has not yet been confirmed, the e-mail said.

 

In late 2012 tests showed that a cow that died two years earlier in Parana state had developed the protein that causes BSE, though the animal never developed the disease and died of natural causes.

 

The case was considered "atypical" as the animal contracted the protein spontaneously, rather than through the feed supply. Classical cases of BSE are caused when cattle are fed brain or spinal tissue of other ruminants, which is now forbidden in nearly all beef producing countries including Brazil.

 

The World Animal Health Organization maintained Brazil's status as a country with an insignificant risk of BSE after it confirmed the atypical Parana case in tests carried out in England in 2012.

 

Even so, several countries including South Korea, China and Egypt banned some or all beef imports from Brazil, the world's top exporter.

 

Humans can develop what is known as variant Creutzfeldt-Jakob disease from consuming animals with mad cow, and more than 150 people have died from it. Mad cow was first discovered in Britain in 1986, but strict controls have tempered its spread.

 

 




 
24/04/2014 14:59Defesa sanitária

Mapa investiga caso atípico de EEB


O Ministério da Agricultura, Pecuária e Abastecimento (Mapa) acionou o sistema de defesa animal para averiguar um caso provável de Encefalopatia Espongiforme Bovina (EEB), no estado de Mato Grosso. As investigações de campo indicam tratar-se de uma única suspeita de caso atípico de EEB, já que o animal foi criado exclusivamente em sistema extensivo (a pasto e sal mineral) e foi abatido em idade avançada, com cerca de12 anos de idade. Essas são as principais características de um caso atípico de EEB, pois ocorre de forma esporádica e espontânea, não relacionada à ingestão de alimentos contaminados.
Os produtos derivados desse bovino não ingressaram na cadeia de alimentação humana ou animal e o material de risco foi incinerado. Por precaução, todos os animais contemporâneos ao caso provável foram identificados individualmente e interditados.
O bovino analisado foi enviado para abate no dia 19 de março de 2014, em virtude de problemas reprodutivos ocasionados pela idade avançada e sem sintomas de distúrbios neurológicos. Durante a inspeção ante mortem, o fiscal federal agropecuário responsável pela fiscalização no frigorífico observou que havia um animal caído, ou seja, em “decúbito forçado”. Com esse quadro, o animal não foi considerado apto ao abate de rotina, sendo direcionado ao abate de emergência e submetido à colheita de amostras para o teste de EEB.
O resultado final dos exames realizados em laboratório nacional agropecuário detectou a marcação priônica. Conforme os protocolos brasileiros, foram deflagradas atividades imediatas no sentido de realizar investigação epidemiológica a campo e providências para o envio da amostra ao laboratório de referência internacional da Organização Mundial de Saúde Animal (OIE), em Weybridge, na Inglaterra, para confirmação da suspeita.
O sistema de defesa sanitária animal brasileiro relacionado à EEB já está consolidado há décadas e permitiu ao Brasil ser classificado como país com risco insignificante para a doença perante a OIE. Essa classificação é a melhor que existe no mundo. Todas as informações sobre o caso já foram repassadas pessoalmente por representantes do Mapa à OIE, que enalteceu a transparência e competência brasileira, colocando inclusive sua estrutura à disposição, assim como seus laboratórios de referência.
A notificação oficial e novas informações sobre o caso serão repassadas quando sair o resultado final da avaliação do laboratório da OIE, na próxima semana.


Palavras chave: caso atípico EEB Mapa



http://www.agricultura.gov.br/comunicacao/noticias/2014/04/mapa-investiga-caso-atipico-de-eeb

 

Friday, December 07, 2012

 

ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012

 


 

 

Wednesday, December 19, 2012

 

Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil

 


 

 

Wednesday, February 12, 2014

 

USDA/APHIS NOTICE: Final Rule Regarding Imports and BSE Effective March 4, 2014

 


 

 

Friday, March 21, 2014

 

Rancho Dead Stock Cancer Downers Recall Explained FSIS March 20 2014 ?

 

“As of March 20, 2014, FSIS has completed all checks (effectiveness checks and disposition verification checks) for recalls 002-2014 and 013-2014 regarding Rancho Feeding Corporation. FSIS has determined that based on the number of successful checks (see Directive 8080.1, Attachment 1, Table 3) where businesses were notified of the recall and removed affected products from commerce that the recall activities were effective.”

 


 

 

Friday, April 4, 2014

 

China, Australia, Argentina, Brazil, Uruguay, Morocco, Israel, South Africa and Saudi Arabia still retain BSE-related closures

 


 

 

Tuesday, July 17, 2012

 

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

 


 

 

Saturday, April 19, 2014

 

*** Exploring the zoonotic potential of animal prion diseases: In vivo and in vitro approaches ***

 


 

 

 

TSS

Saturday, April 19, 2014

Estimation of the Exposure of the UK Population to the Bovine Spongiform Encephalopathy Agent through Dietary Intake During the Period 1980 to 1996

Estimation of the Exposure of the UK Population to the Bovine Spongiform Encephalopathy Agent through Dietary Intake During the Period 1980 to 1996

 

Chu-Chih Chen mail,

 

Yin-Han Wang

 

Published: April 15, 2014 •DOI: 10.1371/journal.pone.0094020

 

Abstract

 

Although the incidence of variant Creutzfeldt-Jakob disease (vCJD) has declined to 1 since 2012 in the UK, uncertainty remains regarding possible future cases and the size of the subclinical population that may cause secondary transmission of the disease through blood transfusion. Estimating the number of individuals who were exposed to the bovine spongiform encephalopathy (BSE) infectious agent and may be susceptible to vCJD will help to clarify related public health concerns and plan strategies. In this paper, we explore this estimate by describing the probability of potential exposure due to dietary intake throughout the BSE epidemic period from 1980 to 1996 as a stochastic Poisson process. We estimate the age- and gender-specific exposure intensities in food categories of beef and beef-containing dishes, burgers and kebabs, pies, and sausages, separating the two periods of 1980–1989 and 1990–1996 due to the specified bovine offal legislation of 1989. The estimated total number of (living) exposed individuals during each period is 5,089,027 (95% confidence interval [CI] 4,514,963–6,410,317), which was obtained by multiplying the population size of different birth cohorts by the probability of exposure via dietary intake and the probability of survival until the end of 2013. The estimated number is approximately doubled, assuming a contamination rate of . Among those individuals estimated, 31,855 (95% CI 26,849–42,541) are susceptible to infection. We also examined the threshold hypothesis by fitting an extreme-value distribution to the estimated infectious dose of the exposed individuals and obtained a threshold estimate of 13.7 bID50 (95% CI 6.6–26.2 bID50) (Weibull). The results provide useful information on potential carriers of prion disease who may pose a threat of infection via blood transfusion and thus provide insight into the likelihood of new incidents of vCJD occurring in the future.

 

snip...

 

Discussion

 

In this study, we estimated the number of exposed individuals in the UK during the BSE epidemic period 1980–1996 based on the estimated BSE-infected cattle in the last year of incubation and unreported or differentially slaughtered for consumption [15], [17], [42], the average age-specific bovine meat intake [28]–[32], and the national statistics [40]. We then estimated the numbers of subclinical carriers of abnormal prion for different genotypes at PRNP codon 129 after being exposed from the posterior susceptibility estimate with prior information obtained from the literature [13], [17]. We describe the probability of being exposed via dietary intake through the entire period by a stochastic Poisson process. This approach requires only an estimation of the mean exposure intensity of the infectious agent in bovine meat products. Thus, the assumption of the incubation period distribution and time of infection based on the observed vCJD cases, as in the back-calculation method [15]–[23] and other simulation-based approaches [24]–[27], is avoided, which significantly reduces estimation uncertainty. Furthermore, the possibility of repeated exposure [41] and the data concerning age-specific bovine meat consumption [28]–[32] are naturally taken into account in the estimation procedure. Therefore, the results provide important estimates of the prevalence of subclinical infection from mathematical modeling, in addition to the scale of exposure of the UK population to the BSE infectious agent, which cannot be derived simply from the observed vCJD cases.

 

Observed cases of vCJD occurring via the primary infection route of bovine meat consumption remain very small in recent years [1], as does prediction for future incidents [2]. However, uncertainty regarding the secondary infection route – blood transfusion from asymptomatic infected donors – has raised great concerns for public health and related administration strategies [3]–[7]. The estimate of approximately 32,000 exposed individuals who are potential subclinical carriers of abnormal prions provides a more concise estimate and is consistent with the results obtained from several large-scale biomarker studies on infection prevalence in the UK [5]–[13]. We obtained our estimates mainly based on the survey outcomes of Gill et al. [13], especially for the MV and VV genotypes, because previous surveys did not provide prevalence information on age and genotype [9]–[11]. Also, the infection function given by Valleron et al. [17] is for the MM genotype only. The approximately the same scale across different age groups essentially shows that both the susceptibility estimate of Valleron et al. [17] and the survey outcomes of Gill et al. [13] are reflected in the posterior estimate. A similar explanation applies to estimates for the MV and VV genotypes. These carriers are most likely subclinical to vCJD without developing into a case if they were exposed to a relatively low infectious dose. However, for the null prevalence of certain age categories and the 1990–1996 birth cohort (mainly from Valleron et al. [17]), the numbers may change substantially if positive sample(s) were detected for these categories in future surveys.

 

We have further justified the threshold hypothesis [4], [34]–[39] and provided an explicit threshold estimate of the infectious dose by fitting an extreme-value distribution model to the estimated number of exposed individuals and comparing that with the number of vCJD cases in each birth cohort. The existence of a threshold dose for infection has been conjectured and assessed in the literature [4], [36]–[39]. Based on the dose-response curve observed in mice, Fryer and McLean conclude that there is no evidence of the existence of such a threshold [38]. However, if this were the case in humans, the number of vCJD cases would have been far more than what has been observed to date, given our exposed individual estimate and the exponential growth rate of abnormal prions in the brain once infected [35]. The close model fitting to the observed vCJD cases justifies the threshold hypothesis. Furthermore, the threshold dose estimate of approximately 12 bID50 with an equivalent weight of 1.2 g of a BSE-infected bovine brain [39] also appears reasonable, which may alternatively be interpreted as the species barrier between bovine and human [39], [50].

 

The estimated number of exposed individuals is based on the estimation of the BSE-infected bovines in the last year of incubation and unreported or differentially slaughtered for consumption during the 1980–1996 period [16]. The figure could be much higher if all of the pre-clinical bovines and contaminated meat products made from beef that entered the food chain are considered when deriving the exposure intensity. Also, we exclude trigeminal ganglia, ileum, tonsil, spleen and eyes in our estimate of contaminated MRM because these parts are typically removed before meat consumption. However, bovine intestine was used for the manufacture of natural sausage casings prior to the SBO ban in 1989 [42]. Therefore, it is possible that individuals might be exposed through consumption of sausages with castings from contaminated intestine, which may substantially increase the number of exposed individuals. Because of the thinness, the infectivity in casings (if there is any) would be very low compared to that in contaminated MRM and head meat. Offals such as rectums and small intestines are also reported being exported to Germany for sausage manufacture and casings [45]. Based on these considerations, we choose to ignore the number of exposed individuals through this route. We rule out the possibility of being exposed by consumption of brain from preclinical BSE bovine directly, given that the major sources entering the food chain in the period were MRM and head meat [45] and none of the vCJD cases have reported eating bovine brain [42].

 

We adopt a Bayesian simulation approach to handle the great uncertainties in the proportions of MRM and head meat used in producing beef and beef-containing dishes, burgers and kebabs, pies, and sausages that might have contained BSE infectious agents during the 1980–1996 period. The results show that although the simulated 95% CIs cover a wide range, the estimated numbers are of approximately the same scale. Also, although the excess numbers of estimated individuals exposed due to ingestion of contaminated meat are very large, the amount of the exposure dose may be negligible for most people, except for the subclinical carriers who might be exposed to a certain amount of infectivity. As shown in Table 4, the numbers of possibly exposed individuals and subclinical carriers increase substantially with a CR of . However, the threshold dose estimate remains approximately the same when the mean exposure dose decreases to about a quarter of that given in the scenario of CR = 0. Therefore, the future vCJD prediction is not expected to change because of exposure uncertainty.

 

In summary, the estimated current numbers of exposed individuals and those who are susceptible or carry the vCJD infectious agent may provide necessary information regarding the extent of the potential public health threat in the tail of the vCJD epidemic in the UK. The number of susceptible exposed individuals is especially important for assessing the risk of secondary transmission via blood transfusion, plasma products, or contaminated surgical instruments; assessment of this risk has been inconclusive or inconsistent based on the results of several large-scale biomarker studies [5]–[14]. Furthermore, the almost exact match between the predicted and observed vCJD cases and the threshold infectious dose estimate has greatly reduced the uncertainty regarding future incidents via the primary transmission route, food intake. However, the results obtained cannot infer the likelihood of secondary transmission from the asymptotic carriers of prion disease.

 

see full text ;

 


 

CJD and Baby foods (the great debate 1999)

 

Subject: Re: Girl, 13, shows CJD symptoms.

 

From: "Terry S. Singeltary Sr."

 

Reply-To: Bovine Spongiform Encephalopathy

 

Date: Wed, 24 Nov 1999 11:35:44 -0600 Content-Type: text/plain Parts/Attachments: text/plain (67 lines)

 


 

Sunday, May 18, 2008

 

MAD COW DISEASE BSE CJD CHILDREN VACCINES

 


 

 Sunday, May 18, 2008

 

BSE Inquiry DRAFT FACTUAL ACCOUNTS DFAs

 


 

 Monday, May 19, 2008

 

*** SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS ***

 


 

“Cases of vCJD peaked in 2000, leading some scientists to speculate that the disease has an incubation period of about a decade. Yet studies of different forms of CJD suggest that the incubation time of vCJD could be much longer, indicating that many people in Britain could be carrying the infection without symptoms.”

 


 

Monday, October 14, 2013

 

Researchers estimate one in 2,000 people in the UK carry variant CJD proteins

 


 

However, I think that the specific confusion there is that people talk about sporadic CJD occurring at 1 per million. That is not your individual risk. Your risk is 1 per million every year. Actually, it is nearer 2 per million per year of the population will develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 1 in 30,000. So that has not really changed. When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.

 


 

Cases of vCJD peaked in 2000, leading some scientists to speculate that the disease has an incubation period of about a decade. Yet studies of different forms of CJD suggest that the incubation time of vCJD could be much longer, indicating that many people in Britain could be carrying the infection without symptoms.

 


 

Friday, February 14, 2014

 

Creutzfeldt-Jakob disease (CJD) biannual update (February 2014), with briefing on novel human prion disease National CJD Research and Surveillance Unit NCJDRSU

 


 

Wednesday, December 11, 2013

 

*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***

 


 

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.

 


 

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...

 


 

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

 

Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. 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 BSE-derived 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.

 


 

-------- 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)

 

____________________________________

 


 

Wednesday, October 09, 2013

 

WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY, £41,078,281 in compensation REVISED

 


 

U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001

 


 

Saturday, April 19, 2014

 

Exploring the zoonotic potential of animal prion diseases: In vivo and in vitro approaches

 


 

TSS

Monday, February 10, 2014

Enhanced Virulence of Sheep-Passaged Bovine Spongiform Encephalopathy Agent Is Revealed by Decreased Polymorphism Barriers in Prion Protein Conversion Studies

Enhanced Virulence of Sheep-Passaged Bovine Spongiform Encephalopathy Agent Is Revealed by Decreased Polymorphism Barriers in Prion Protein Conversion Studies

 

Jan Priema, Jan P. M. Langevelda, Lucien J. M. van Keulena, Fred G. van Zijderveldb, Olivier Andreolettic and Alex Bossersa

 

+ Author Affiliations aDepartment of Infection Biology, Central Veterinary Institute of Wageningen UR, Lelystad, The Netherlands bDepartment of Bacteriology and TSEs, Central Veterinary Institute of Wageningen UR, Lelystad, The Netherlands cUMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, France

 

ABSTRACT

 

Bovine spongiform encephalopathy (BSE) can be efficiently transmitted to small ruminants (sheep and goats) with certain prion protein (PrP) genotypes. Polymorphisms in PrP of both the host and donor influence the transmission efficiency of transmissible spongiform encephalopathies (TSEs) in general. These polymorphisms in PrP also modulate the PrP conversion underlying TSE agent replication. Here we demonstrate that single-round protein misfolding cyclic amplification (PMCA) can be used to assess species and polymorphism barriers at the molecular level. We assessed those within and between the ovine and bovine species in vitro using a variety of natural scrapie and experimentally generated cross-species BSE agents. These BSE agents include ovBSE-ARQ isolates (BSE derived from sheep having the ARQ/ARQ PrP genotype), and two unique BSE-derived variants: BSE passaged in VRQ/VRQ sheep and a cow BSE agent isolate generated by back-transmission of ovBSE-ARQ into its original host. PMCA allowed us to quantitatively determine PrP conversion profiles that correlated with known in vivo transmissibility and susceptibility in the two ruminant species in which strain-specific molecular signatures, like its molecular weight after protease digestion, were maintained. Furthermore, both BSE agent isolates from ARQ and VRQ sheep demonstrated a surprising transmission profile in which efficient transmissions to both sheep and bovine variants was combined. Finally, all data support the notion that ARQ-derived sheep BSE points to a significant increase in virulence compared to all other tested scrapie- and BSE-derived variants reflected by the increased conversion efficiencies of previously inefficient convertible PrP variants (including the so-called “resistant” sheep ARR variant).

 

IMPORTANCE Prion diseases such as scrapie in sheep and goats, BSE in cattle, and Creutzfeldt-Jakob disease (CJD) in humans are fatal neurodegenerative diseases caused by prions. BSE is known to be transmissible to a variety of hosts, including sheep and humans. Based on the typical BSE agent strain signatures and epidemiological data, the occurrence of a novel variant of CJD in humans was linked to BSE occurrence in the United Kingdom. Measures, including genetic selection of sheep toward less susceptible PrP genotypes, have been implemented to lower the risk of BSE transmission into sheep, since the disease could potentially spread into a natural reservoir. In this study, we demonstrated using molecular PrP conversion studies that when BSE is first transmitted through sheep, the host range is modified significantly and the PrP converting potency increased, allowing the ovine BSE to transmit more efficiently than cow BSE into supposedly less susceptible hosts.

 

 FOOTNOTES Received 25 August 2013. Accepted 19 December 2013. Address correspondence to Alex Bossers, alex.bossers@wur.nl.

 

Published ahead of print 26 December 2013

 

Copyright © 2014, American Society for Microbiology. All Rights Reserved.

 

 


 

 

Thursday, December 05, 2013

 

National Scrapie Eradication Program October 2013 Monthly Report Fiscal Year 2014 TSE PRION REPORT

 


 

 

Sunday, November 17, 2013

 

L-BSE in Genetically Susceptible and Resistant Sheep: Changes in Prion Strain or Phenotypic Plasticity of the Disease-Associated Prion Protein?

 


 

 

Saturday, November 02, 2013

 

OREGON DETECTS SCRAPIE

 


 

 

Friday, July 26, 2013

 

Voluntary Scrapie Program USA UPDATE July 26, 2013 increase in FY 2013 is not statistically meaningful due to the sample size

 

Greetings BSE-L members et al,

 

“The increase in FY 2013 is not statistically meaningful due to the sample size.”

 

SO, when a TSE mad cow type disease is NOT detectable with a surveillance system that is set up to test numbers so low you can’t find it, and you complain on that one factor, then USDA inc tells you that those are the number set up by OIE inc, and are sufficient to find a TSE mad cow type disease in said species, and that’s good enough for them, as far as ‘sample size’.

 

BUT yet, when the shoe is on the other foot so to speak, oh well, it’s a different story now, the increase in Scrapie cases for the FY 2013 is NOT meaningful now due to the sample size.

 

yep, that’s what happened out in the Trans Pecos region where I told the TAHC et al to test for CWD ten years ago, I complained about that sample size being totally insufficient, where I knew the CWD positives were waltzing into Texas, well, they let these CWD positives do it for another 10 years before doing anything about it, and when they finally increased the sample size, guess what, they found CWD in TEXAS.

 

same thing happened with mad cow disease. they increased the sample size, found it, scared the hell out of them because of all the atypicals, and immediately shut it down, back to OIE numbers, where you cannot find a TSE prion disease. what a bunch of hypocrites, and what a joke. all of them.

 

I suppose the high number of goat cases in California and Michigan, and the two meat-type twin goats residing in the same herd that tested positive, both that were submitted as clinical suspects, I guess that’s just another happenstance of bad luck, another spontaneous event, ...really?

 

*** I have been trying to bring awareness to the increase in numbers of goat scrapie cases in certain areas for years now from different potential sources, to no avail. how many of them might be BSE? as the USDA inc, the OIE inc, CFIA inc, all try to make typical scrapie a legal trading commodity (they have already done this with the atypical scrapie, a very foolish move, one that risk both humans and animals around the globe to the TSE mad cow type agent). this voluntary scrapie program will not work, in my opinion, the USDA, OIE, CFIA, know this, so the next best thing is just to force feed all of us around the globe the TSE prion mad cow type agent by exempting it all $$$ it’s all gonna catch up sooner or later. just my take. ...

 

Friday, July 26, 2013

 

Voluntary Scrapie Program USA UPDATE July 26, 2013 increase in FY 2013 is not statistically meaningful due to the sample size http://scrapie-usa.blogspot.com/2013/07/voluntary-scrapie-program-usa-update.html

 

Sunday, June 2, 2013

 

Characterisation of an Unusual TSE in a Goat by Transmission in Knock-in Transgenic Mice

 


 

 

 Saturday, July 6, 2013

 

*** Small Ruminant Nor98 Prions Share Biochemical Features with Human Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive Prionopathy

 

Research Article

 


 

 

Thursday, March 29, 2012

 

atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012

 

NIAA Annual Conference April 11-14, 2011San Antonio, Texas

 


 

 

Wednesday, January 18, 2012

 

BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE

 

February 1, 2012

 


 

 

Wednesday, January 18, 2012

 

Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie

 

Journal of Neuropathology & Experimental Neurology:

 

February 2012 - Volume 71 - Issue 2 - p 140–147

 


 

 

Thursday, July 14, 2011

 

Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)

 


 

 

SHEEP AND BSE

 

PERSONAL AND CONFIDENTIAL

 

SHEEP AND BSE

 

A. The experimental transmission of BSE to sheep.

 

Studies have shown that the ''negative'' line NPU flock of Cheviots can be experimentally infected with BSE by intracerebral (ic) or oral challenge (the latter being equivalent to 0.5 gram of a pool of four cow brains from animals confirmed to have BSE).

 


 

 

RB264

 

BSE - TRANSMISSION STUDIES

 


 

 

Wednesday, January 18, 2012

 

Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie

 

Journal of Neuropathology & Experimental Neurology:

 

February 2012 - Volume 71 - Issue 2 - p 140–147

 


 

 

Saturday, December 3, 2011

 

Isolation of Prion with BSE Properties from Farmed Goat Volume 17, Number 12—December 2011

 


 

 

Wednesday, February 16, 2011

 

IN CONFIDENCE SCRAPIE TRANSMISSION TO CHIMPANZEES IN CONFIDENCE

 


 

 

Sunday, December 12, 2010

 

EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010

 


 

 

Sunday, April 18, 2010

 

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

 


 

 
Sunday, February 2, 2014
 
The Presence of Disease-Associated Prion Protein in Skeletal Muscle of Cattle Infected with Classical Bovine Spongiform Encephalopathy
 
NOTE Pathology
 
 
 
 
 

 

TSS