Two cases of variant Creutzfeldt-Jakob disease SPAIN 07-08

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flounder

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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



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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.


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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
 

hillsdown

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I think this is just the tip of the iceberg. There are going to be more deaths all over the world.

We need to test everything before it goes into the food chain. It is time for people to demand this as producers and consumers.
 

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