SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION

Numero de focos de Encefalopatia Espongiforme Bovine en Espana 2009 - 1010


2009 = 18 cases BSE

http://www.eeb.es/pags/2009.htm


2010 to date = 4 cases of BSE

http://www.eeb.es/pags/espana.htm#2010


BSE SPAIN

Año 2000

http://www.eeb.es/pags/2000.htm


Año 2001

http://www.eeb.es/pags/2000.htm#2001


Año 2002

http://www.eeb.es/pags/2002.htm


Año 2003

http://www.eeb.es/pags/2003.htm


Año 2004

http://www.eeb.es/pags/2004.htm


Año 2005

http://www.eeb.es/pags/2005.htm


Año 2006

http://www.eeb.es/pags/2006.htm


Año 2007

http://www.eeb.es/pags/2007.htm


Año 2008

http://www.eeb.es/pags/2008.htm


Año 2009

http://www.eeb.es/pags/2009.htm


BSE SPAIN 2010 Situacion en Espana Total casos

http://www.eeb.es/pags/espana.htm#2010



Greetings,

PLEASE NOTE HIGH INCIDENT OF SPORADIC CJD AND BSE IN THE ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? another coincident, or BSE related. remember, BSE will propagate as nvCJD and or sporadic CJD in humanized transgenic mice, and please NOTE ALSO, the close proximity of BASQUE COUNTRY SPAIN TO ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? SO, you have a high rate of sporadic CJD cases in an area of BSE and Nor-98 atypical scrapie cases, another spontaneous coincidence, or a related event ?


NOTE THE SPORADIC CJD INCIDENT RATE AT > 2 CASES PER MILLION ;


SEE CJD UPDATE 2010

SEE PAGE 4 ;

Notificaciones* al RNEETH (1993-febrero 2010)

• Por año diagnóstico. Existe 1 caso más sin año diagnóstico

AÑO

CCAA 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 TOTAL

Andalucia 4 6 2 2 5 4 8 11 16 14 9 11 16 15 13 19 11 1 167

Aragon 0 0 1 0 5 4 1 3 4 0 2 0 5 2 2 5 4 1 39

Asturias 2 0 2 0 1 2 3 2 2 0 5 2 1 0 0 0 0 0 22

Baleares 1 2 0 1 1 0 2 0 1 1 1 3 1 0 0 1 4 0 19

Canarias 0 0 0 0 4 4 4 2 2 2 2 2 2 3 3 1 3 0 34

Cantabria 1 2 0 0 2 4 3 3 0 2 0 0 1 0 0 2 0 0 20

Castilla la Mancha 0 1 1 2 2 1 2 2 1 2 0 2 5 3 5 6 7 0 42

Castilla-Leon 2 1 6 2 7 4 4 6 8 5 6 6 7 7 7 12 5 0 95

Cataluna 8 6 3 6 6 15 16 9 13 11 7 19 15 16 13 14 7 0 184

Valencia 1 3 3 7 5 12 11 10 12 13 17 10 8 8 19 16 9 2 166

Extremadura 0 0 0 1 1 2 2 0 0 3 1 0 2 7 5 3 1 0 28

Galicia 1 1 3 0 0 11 3 5 8 7 1 7 6 5 3 2 0 0 63

Madrid 4 4 5 7 5 7 9 10 16 7 12 8 13 16 8 8 13 0 152

Murcia 1 0 0 0 1 0 1 0 2 3 2 2 3 1 6 2 3 0 27

Navarra 1 0 0 2 1 0 2 2 1 0 0 0 4 2 0 0 0 0 15

Pais Vasco 2 3 1 6 4 8 5 8 8 9 7 6 11 10 11 8 4 1 112

La Rioja 2 0 0 0 1 1 0 0 1 0 1 0 1 0 1 0 0 0 8

TOTAL 30 29 27 36 51 79 76 73 95 79 73 78 101 95 96 99 71 5 1193

• Por año diagnóstico. Existe 1 caso más sin año diagnóstico

REGISTRO ESPAÑOL DE EETH

1993-febrero 2010

ECJ = 957

vECJ = 5

IFL = 44

GSS = 2

Esporadico = 905

Familiar = 46

Yatrogenico = 6

* Existen 2 casos mas con diagnóstico pendiente de clasificación

ALSO, ON PAGE 14, NOTE THE HIGH INCIDENCE IN THIS SAME AREA ;

Número de casos de Insomnio Familiar Letal por CA. 1993-febrero 2010

HIGH INCIENCE RATE AT 18 CASES


please see full text ;


REGISTRO NACIONAL DE ENCEFALOPATÍAS ESPONGIFORMES TRANSMISIBLES HUMANAS C.N.E y Servicios de Vigilancia Epidemiológica de CCAA (Situación a 15 de febrero de 2010)


http://www.isciii.es/htdocs/pdf/DatosRegistroCreutzfeldJacob2.pdf



The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653

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

Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1

1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to:

John Collinge, E-mail: j.collinge@prion.ucl.ac.uk

Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002


--------------------------------------------------------------------------------


Abstract

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.

Keywords:BSE, Creutzfeldt–Jakob disease, prion, transgenic


http://www.nature.com/emboj/journal/v21/n23/abs/7594869a.html


Monday, May 19, 2008

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

http://bseinquiry.blogspot.com/


Sunday, August 10, 2008

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html


Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks

BMC Veterinary Research 2010, 6:17 doi:10.1186/1746-6148-6-17

http://www.biomedcentral.com/content/pdf/1746-6148-6-17.pdf


Sunday, March 28, 2010

Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks

http://nor-98.blogspot.com/2010/03/atypicalnor98-scrapie-in-basque-country.html


Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010

Greetings,

Unusual event if you consider the officials hypothisis that Nor-98 atypical scrapie is a spontaneous event. seems there was a great deal of spontaneous mutations for this time period ;-)...TSS

http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html


Thursday, March 11, 2010

CANADA TYPICAL AND ATYPICAL SCRAPIE REPORT TO MARCH 2010

http://nor-98.blogspot.com/2010/03/canada-typical-and-atypical-scrapie.html


Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html


Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types

hmmm, this is getting interesting now...

Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,

see also ;

All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

http://cjdusa.blogspot.com/2009/09/co-existence-of-scrapie-prion-protein.html


see full text ;

Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types

http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html


TSS