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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