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
|
| ||||||||||||
Total animals
affected
|
| ||||||||||||
Outbreak
statistics
|
* Removed from the susceptible population through death, destruction and/or slaughter; |
Epidemiology
Source of the outbreak(s) or
origin of infection
|
|
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
|
|
Measures to be
applied
|
|
Diagnostic test results
Laboratory name and
type
|
National Institute for
Agrarian and Veterinary Research (INIAV) ( National laboratory
)
| ||||||||||||
Tests and
results
|
|
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
|
| ||||||||||||
Nombre total d'animaux
atteints
|
| ||||||||||||
Statistiques sur le
foyer
|
* Soustraits de la population sensible suite à la mort, à l´abattage et/ou à la destruction; |
Epidémiologie
Source du/des foyer(s) ou
origine de l´infection
|
|
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
|
|
Mesures à
appliquer
|
|
Résultats des tests de diagnostics
Nom du laboratoire et
type
|
Institut national de
recherche vétérinaire et agraire (INIAV) ( Laboratoire national
)
| ||||||||||||
Tests et
résultats
|
|
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
|
| ||||||||||||
Número total de animales
afectados
|
| ||||||||||||
Estadística del
foco
|
* Descontados de la población susceptible a raíz de su muerte, destrucción o sacrificio; |
Epidemiología
Fuente del o de los focos u
origen de la infección
|
|
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
|
|
Medidas para
implementar
|
|
Resultados de las pruebas diagnósticas
Nombre y tipo de
laboratorio
|
Instituto nacional de
investigación veterinaria y agraria (INIAV) ( Laboratorio nacional
)
| ||||||||||||
Pruebas y
resultados
|
|
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
http://www.usatradeonline.gov/agworld.nsf/505c55d16b88351a852567010058449b/91142d01964ffee885257297004ac400/$FILE/E47017.PDF
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
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
############
-------- 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
