Rapid communications
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
volume 13
issue 15
publication date April 2008
How to cite this article, click here.
Indexed in MedLine as: Euro Surveill 2008;13(15) Published online April 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
J de Pedro Cuesta ([email protected]" onclick="window.open(this.href);return false
, Instituto de Salud Carlos III, Department of Applied Epidemiology, National Centre of Epidemiology, Madrid, Spain
--------------------------------------------------------------------------------
In 2005, the first case of variant Creutzfeldt-Jakob disease (vCJD) was reported in Spain, in a woman born in 1978 with clinical onset of symptoms in 2004 [1]. She subsequently died in 2005.
Recently, two more laboratory-confirmed vCJD cases were reported to the Spanish CJD state registry. In February 2006, a woman born in 1957 developed progressive cognitive deterioration, and died in December 2007 with suspected sporadic CJD (typical EEG in October 2007) MM at codon 129 and no mutations in PRPN gene. A man born in 1967 had onset in May 2007 with psychiatric symptoms, and after several months developed progressive cognitive decline with dementia, typical MRI, MM at codon 129, no mutations in PRPN gene. He died in February 2008. Post-mortem, neuropathology with histochemistry confirmed vCJD in both cases. No clear specific dietary habits, blood donations or reception were recorded. Neither case appears to have visited the United Kingdom before 2004.
The latest two cases were resident in the same region of the country, Castilla y Leon, but no link between them was established.
--------------------------------------------------------------------------------
References
Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(8):E050804.1. Available from: http://www.eurosurveillance.org/ew/2005/050804.asp#1
Citation style for this article: . Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008. Euro Surveill 2008;13(15). Available online: http://www.eurosurveillance.org/edition ... 0410_3.asp
*Acquired in UK
** Acquired in Saudi Arabia
*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.
**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1
from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36
type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the
year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from
which brain tissue or blood in the case of familial disease were
submitted.
-- Inconclusive: Cases in which the samples were not sufficient to
make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was
adequate to establish the presence but not the type; in all cases,
vCJD could be excluded.
--
Terry S. Singeltary Sr. <[email protected]>
[In submitting these data, Terry S. Singeltary Sr. draws attention to
the steady increase in the "type unknown" category, which, according
to their definition, comprises cases in which vCJD could be excluded.
The total of 26 cases for the current year (2007) is disturbing,
possibly symptomatic of the circulation of novel agents.
Characterization of these agents should be given a high priority. -
Mod.CP]
http://www.promedmail.org/pls/askus/f?p ... 1010,39963
There is a growing number of human CJD cases, and they were presented
last week in San Francisco by Luigi Gambatti(?) from his CJD
surveillance collection.
He estimates that it may be up to 14 or 15 persons which display
selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.pdf
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
[email protected]" onclick="window.open(this.href);return false;
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S.
under-counts CJD, Schonberger conceded that the current surveillance
system has errors but stated that most of the errors will be confined
to the older population.
http://www.thepathologicalprotein.com/
doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost
my mom to hvCJD (Heidenhain variant CJD) and have been searching for
answers ever since. What I have found is that we have not been told
the truth. CWD in deer and elk is a small portion of a much bigger
problem." ...
http://www.thelancet.com/journals/lanin ... 1/fulltext
http://download.thelancet.com/pdfs/jour ... 007151.pdf
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/http://www.neu ... /2/176#535
APHIS-2006-0041-0006 TSE advisory committee for the meeting December
15, 2006
http://www.regulations.gov/fdmspublic/C ... tType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of
Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments ... 0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments ... 5IFA-2.pdf
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as
well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
http://cjdusa.blogspot.com/
Creutzfeldt Jakob Disease
http://creutzfeldt-jakob-disease.blogspot.com/
Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel
PrPSc ype in a Young British Woman
http://creutzfeldt-jakob-disease.blogsp ... otein.html
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
ANIMAL HEALTH REPORT 2006 (BSE h-BASE EVENT IN ALABAMA, Scrapie, and CWD)
http://animalhealthreport2006.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Friday, January 11, 2008
CJD HUMAN TSE REPORT UK, USA, CANADA, and Mexico JANUARY 2008
http://cjdmadcowbaseoct2007.blogspot.co ... a-and.html
Friday, February 8, 2008
Creutzfeldt Jakob Disease Delaware UPDATE
http://cjdmadcowbaseoct2007.blogspot.co ... aware.html
CREUTZFELDT JAKOB DISEASE TEXAS
http://cjdtexas.blogspot.com/
Friday, January 25, 2008
January 2008 Update on Feed Enforcement Activities to Limit the Spread
of BSE
http://madcowspontaneousnot.blogspot.co ... ement.html
http://madcowspontaneousnot.blogspot.com/
BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA
http://madcowtesting.blogspot.com/
NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007
http://nor-98.blogspot.com/
http://scrapie-usa.blogspot.com/
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L,
and or Italian L-BASE
http://bse-atypical.blogspot.com/2008/0 ... c-bse.html
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/
SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS
http://cjdmadcowbaseoct2007.blogspot.co ... ounds.html
SPECIFIED RISK MATERIALS
http://madcowspontaneousnot.blogspot.co ... s-srm.html
TSS
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
volume 13
issue 15
publication date April 2008
How to cite this article, click here.
Indexed in MedLine as: Euro Surveill 2008;13(15) Published online April 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
J de Pedro Cuesta ([email protected]" onclick="window.open(this.href);return false
, Instituto de Salud Carlos III, Department of Applied Epidemiology, National Centre of Epidemiology, Madrid, Spain--------------------------------------------------------------------------------
In 2005, the first case of variant Creutzfeldt-Jakob disease (vCJD) was reported in Spain, in a woman born in 1978 with clinical onset of symptoms in 2004 [1]. She subsequently died in 2005.
Recently, two more laboratory-confirmed vCJD cases were reported to the Spanish CJD state registry. In February 2006, a woman born in 1957 developed progressive cognitive deterioration, and died in December 2007 with suspected sporadic CJD (typical EEG in October 2007) MM at codon 129 and no mutations in PRPN gene. A man born in 1967 had onset in May 2007 with psychiatric symptoms, and after several months developed progressive cognitive decline with dementia, typical MRI, MM at codon 129, no mutations in PRPN gene. He died in February 2008. Post-mortem, neuropathology with histochemistry confirmed vCJD in both cases. No clear specific dietary habits, blood donations or reception were recorded. Neither case appears to have visited the United Kingdom before 2004.
The latest two cases were resident in the same region of the country, Castilla y Leon, but no link between them was established.
--------------------------------------------------------------------------------
References
Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(8):E050804.1. Available from: http://www.eurosurveillance.org/ew/2005/050804.asp#1
Citation style for this article: . Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008. Euro Surveill 2008;13(15). Available online: http://www.eurosurveillance.org/edition ... 0410_3.asp
*Acquired in UK
** Acquired in Saudi Arabia
*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.
**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1
from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36
type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the
year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from
which brain tissue or blood in the case of familial disease were
submitted.
-- Inconclusive: Cases in which the samples were not sufficient to
make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was
adequate to establish the presence but not the type; in all cases,
vCJD could be excluded.
--
Terry S. Singeltary Sr. <[email protected]>
[In submitting these data, Terry S. Singeltary Sr. draws attention to
the steady increase in the "type unknown" category, which, according
to their definition, comprises cases in which vCJD could be excluded.
The total of 26 cases for the current year (2007) is disturbing,
possibly symptomatic of the circulation of novel agents.
Characterization of these agents should be given a high priority. -
Mod.CP]
http://www.promedmail.org/pls/askus/f?p ... 1010,39963
There is a growing number of human CJD cases, and they were presented
last week in San Francisco by Luigi Gambatti(?) from his CJD
surveillance collection.
He estimates that it may be up to 14 or 15 persons which display
selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/ ... 4240t1.pdf
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
[email protected]" onclick="window.open(this.href);return false;
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S.
under-counts CJD, Schonberger conceded that the current surveillance
system has errors but stated that most of the errors will be confined
to the older population.
http://www.thepathologicalprotein.com/
doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost
my mom to hvCJD (Heidenhain variant CJD) and have been searching for
answers ever since. What I have found is that we have not been told
the truth. CWD in deer and elk is a small portion of a much bigger
problem." ...
http://www.thelancet.com/journals/lanin ... 1/fulltext
http://download.thelancet.com/pdfs/jour ... 007151.pdf
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/http://www.neu ... /2/176#535
APHIS-2006-0041-0006 TSE advisory committee for the meeting December
15, 2006
http://www.regulations.gov/fdmspublic/C ... tType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of
Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments ... 0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments ... 5IFA-2.pdf
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as
well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
http://cjdusa.blogspot.com/
Creutzfeldt Jakob Disease
http://creutzfeldt-jakob-disease.blogspot.com/
Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel
PrPSc ype in a Young British Woman
http://creutzfeldt-jakob-disease.blogsp ... otein.html
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
ANIMAL HEALTH REPORT 2006 (BSE h-BASE EVENT IN ALABAMA, Scrapie, and CWD)
http://animalhealthreport2006.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Friday, January 11, 2008
CJD HUMAN TSE REPORT UK, USA, CANADA, and Mexico JANUARY 2008
http://cjdmadcowbaseoct2007.blogspot.co ... a-and.html
Friday, February 8, 2008
Creutzfeldt Jakob Disease Delaware UPDATE
http://cjdmadcowbaseoct2007.blogspot.co ... aware.html
CREUTZFELDT JAKOB DISEASE TEXAS
http://cjdtexas.blogspot.com/
Friday, January 25, 2008
January 2008 Update on Feed Enforcement Activities to Limit the Spread
of BSE
http://madcowspontaneousnot.blogspot.co ... ement.html
http://madcowspontaneousnot.blogspot.com/
BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA
http://madcowtesting.blogspot.com/
NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007
http://nor-98.blogspot.com/
http://scrapie-usa.blogspot.com/
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L,
and or Italian L-BASE
http://bse-atypical.blogspot.com/2008/0 ... c-bse.html
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/
SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS
http://cjdmadcowbaseoct2007.blogspot.co ... ounds.html
SPECIFIED RISK MATERIALS
http://madcowspontaneousnot.blogspot.co ... s-srm.html
TSS
