VARIANT CJD (vCJD) or nvCJD

New Variant Creutzfeldt Jakob Disease nvCJD, was linked to young people and BSE in the U.K., aka mad cow disease...

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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Thursday, May 22, 2014

Accelerated, spleen-based titration of variant CJD infectivity in transgenic mice expressing human prion protein with sensitivity comparable to survival time bioassay

Accelerated, spleen-based titration of variant CJD infectivity in transgenic mice expressing human prion protein with sensitivity comparable to survival time bioassay

 

Sophie Halliez1, Fabienne Reine1, Laetitia Herzog1, Emilie Jaumain1, Stéphane Haïk2,3,4, Human Rezaei1, Jean-Luc Vilotte5, Hubert Laude1 and Vincent Béringue1*

 

+ Author Affiliations 1INRA (institut National de la Recherche Agronomique), UR892, Virologie Immunologie Moléculaires, F-78350 Jouy-en-Josas, France 2INSERM, Equipe maladie d'Alzheimer et maladies à Prions, CRicm, UMRS 975, CNRS UMR7225, UPMC. R., Hôpital de la Salpêtrière, F-75013, Paris, France. 3InVS, Centre national de référence des Agents Transmissibles Non Conventionnels, Hôpital de la Salpêtrière, F-75013, Paris, France. 4AP-HP, Laboratoire de Neuropathologie, Hôpital de la Salpêtrière, F-75013, Paris, France 5INRA, UMR1313, Génétique Animale et Biologie Intégrative, F-78350 Jouy-en-Josas, France

 

ABSTRACT

 

The dietary exposure of the human population to the prions responsible for the bovine spongiform encephalopathy (BSE) epizooty has led to the emergence of variant Creutzfeldt-Jakob disease (vCJD). This fatal, untreatable neurodegenerative disorder is a growing public health concern because the prevalence of the infection seems much greater than the disease incidence and because secondary transmission of vCJD by blood transfusion or use of blood products has occurred. A current limitation in variant CJD risk assessment purposes is the lack of quantitative information on the infectivity of contaminated tissues. To these aims, we tested the potential of a transgenic mouse line overexpressing human PrP, which was previously reported to propagate vCJD prions. Endpoint titration of vCJD infectivity in different tissues was evaluated by two different methods: i) the “classical” bioassay, based on the appearance of clinical symptoms and the detection of pathological prion protein in tissues of the inoculated mouse, ii) a shortened bioassay based on the detection of the protein in the mouse spleen at defined time points. Both methods proved equally sensitive in quantifying infectivity, even after very low dose inoculation of infected material, but the time time-schedule was shortened from ∼2.5-year to ∼1-year with the spleen bioassay. Compared to the ‘gold-standard’ RIII model routinely used for endpoint titration of vCJD/BSE prions, either method improved the sensitivity by >2 orders of magnitude and allowed re-evaluating the infectious titre of spleen from a vCJD individual at disease end stage to >1000-fold higher values.

 

Importance Here, we provide key re-evaluation of the infectious titre of variant CJD brain and spleen tissues. The highly sensitive, accelerated spleen-based assay should thus constitute a key advance for variant CJD epidemiological and risk assessment purposes and should greatly facilitate future titration studies, aimed at validating decontamination procedures for example. The overlooked notion that the lymphoid tissue exhibits higher capacity than the brain to replicate prions even after low dose infection raises new questions about the molecular and/or cellular determinant(s) involved, a key issue regarding potent silent carriers of variant CJD in the lymphoid tissue.

 

 FOOTNOTES ↵* Corresponding author: vincent.beringue@jouy.inra.fr Copyright © 2014, American Society for Microbiology. All Rights Reserved.

 


 

CANADA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss

 

PLEASE NOTE, type determination pending Creutzfeldt Jakob Disease (tdpCJD) in Canada is also on a steady increase.

 

please see ;

 

> 3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.

 

CJD Deaths Reported by CJDSS1, 1994-20122

 

CJD Deaths Reported by CJDSS1, 1994-20122

 

As of May 31, 2012

 


 


 

SEE DECEMBER 2012 CANADA

 


 

USA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss

 

National Prion Disease Pathology Surveillance Center

 

Cases Examined1

 

(May 18, 2012)

 

5 Includes 14 cases in which the diagnosis is pending, and 18 inconclusive cases;

 

6 Includes 17 (16 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded. The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).

 

Rev 5/18/2012

 


 

> 6 Includes

 

> 17 (16 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded.

 

> The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).

 

WELL, it seems the USA mad cow strains in humans classified as type determination pending tdpCJD, VPSPr, sFFI, and sCJD) have steadily increased over the years, and the same old song and dance continues with sporadic CJD cases $$$

 


 

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

 

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***

 


 

Sunday, October 13, 2013

 

*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

 


 

Tuesday, April 01, 2014

 

Questions linger in U.S. CJD cases 2005, and still do in 2014

 


 

Sunday, March 09, 2014

 

A Creutzfeldt-Jakob Disease (CJD) Lookback Study: Assessing the Risk of Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease

 

FDA TSEAC CIRCUS AND TRAVELING ROAD SHOW FOR THE TSE PRION DISEASES

 


 

Sunday, April 06, 2014

 

SPORADIC CJD and the potential for zoonotic transmission there from, either directly or indirectly via friendly fire iatrogenic mode, evidence to date

 


 

Wednesday, December 11, 2013

 

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

 


 

Friday, April 25, 2014

 

Accuracy of administrative diagnostic data for pathologically confirmed cases of Creutzfeldt-Jakob disease in Massachusetts, 2000-2008

 

Article in Press

 


 

Monday, May 19, 2014

 

Variant CJD: 18 years of research and surveillance

 


 

 

TSS

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