Original Investigation | March 03, 2014
Population Screening for Variant Creutzfeldt-Jakob Disease Using a Novel
Blood Test Diagnostic Accuracy and Feasibility Study
ONLINE FIRST Graham S. Jackson, PhD1; Jesse Burk-Rafel, MSc1,2; Julie Ann
Edgeworth, PhD1; Anita Sicilia, MSc1; Sabah Abdilahi, BSc1; Justine Korteweg,
BSc1; Jonathan Mackey, BSc1; Claire Thomas, BSc1; Guosu Wang, BSc1; Jonathan M.
Schott, MD3; Catherine Mummery, MB, BS3; Patrick F. Chinnery, MD4; Simon Mead,
BM, BCh1,5; John Collinge, FRS1,5 [+] Author Affiliations JAMA Neurol. Published
online March 03, 2014. doi:10.1001/jamaneurol.2013.6001 Text Size: A A A Article
Figures Tables References
ABSTRACT | METHODS | RESULTS | DISCUSSION | ARTICLE INFORMATION |
REFERENCES
Importance
Our study indicates a prototype blood-based variant Creutzfeldt-Jakob
disease (vCJD) assay has sufficient sensitivity and specificity to justify a
large study comparing vCJD prevalence in the United Kingdom with a bovine
spongiform encephalopathy–unexposed population. In a clinical diagnostic
capacity, the assay’s likelihood ratios dramatically change an individual’s
pretest disease odds to posttest probabilities and can confirm vCJD
infection.
Objectives To determine the diagnostic accuracy of a prototype blood test
for vCJD and hence its suitability for clinical use and for screening
prion-exposed populations.
Design, Setting, and Participants Retrospective, cross-sectional diagnostic
study of blood samples from national blood collection and prion disease centers
in the United States and United Kingdom. Anonymized samples were representative
of the US blood donor population (n = 5000), healthy UK donors (n = 200),
patients with nonprion neurodegenerative diseases (n = 352), patients in whom a
prion disease diagnosis was likely (n = 105), and patients with confirmed vCJD
(n = 10).
Main Outcome and Measure Presence of vCJD infection determined by a
prototype test (now in clinical diagnostic use) that captures, enriches, and
detects disease-associated prion protein from whole blood using stainless steel
powder.
Results The assay’s specificity among the presumed negative American donor
samples was 100% (95% CI, 99.93%-100%) and was confirmed in a healthy UK cohort
(100% specificity; 95% CI, 98.2%-100%). Of potentially cross-reactive blood
samples from patients with nonprion neurodegenerative diseases, no samples
tested positive (100% specificity; 95% CI, 98.9%-100%). Among National Prion
Clinic referrals in whom a prion disease diagnosis was likely, 2 patients with
sporadic CJD tested positive (98.1% specificity; 95% CI, 93.3%-99.8%). Finally,
we reconfirmed but could not refine our previous sensitivity estimate in a small
blind panel of samples from unaffected individuals and patients with vCJD (70%
sensitivity; 95% CI, 34.8%-93.3%).
Conclusions and Relevance In conjunction with the assay’s established high
sensitivity (71.4%; 95% CI, 47.8%-88.7%), the extremely high specificity
supports using the assay to screen for vCJD infection in prion-exposed
populations. Additionally, the lack of cross-reactivity and false positives in a
range of nonprion neurodegenerative diseases supports the use of the assay in
patient diagnosis.
Friday, February 14, 2014
Creutzfeldt-Jakob disease (CJD) biannual update (February 2014), with
briefing on novel human prion disease National CJD Research and Surveillance
Unit NCJDRSU
Monday, January 13, 2014
Prions in Variably Protease-Sensitive Prionopathy: An Update Pathogens 2013
Pathogens 2013, 2, 457-471; doi:10.3390/pathogens2030457
Friday, January 10, 2014
*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial
type prion disease, what it ???
Friday, January 10, 2014
*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial
type prion disease, what it ???
Monday, February 24, 2014
Sporadic Fatal Insomnia in an Adolescent
Thursday, February 20, 2014
*** Unnecessary precautions BSE MAD COW DISEASE Dr. William James FSIS VS
Dr. Linda Detwiler 2014
WHAT about the sporadic CJD TSE proteins ?
WE now know that some cases of sporadic CJD are linked to atypical BSE and
atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all
it’s sub-types $$$
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
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
CJD QUESTIONNAIRE USA
CJD VOICE
layperson
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
flounder9@verizon.net
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