Journal Article

DZNE-2023-00122

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Application of real-time quaking-induced conversion in Creutzfeldt-Jakob disease surveillance.

Hermann, P.Extern* ; Schmitz, M.DZNE* ; Cramm, M.Extern* ; Goebel, S.Extern* ; Bunck, T. ; Schütte-Schmidt, J. ; Schulz-Schaeffer, W. ; Stadelmann, C.Extern* ; Matschke, J. ; Glatzel, M. ; Zerr, I. (Last author)DZNE*

2023
Springer Berlin

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Please use a persistent id in citations: doi:10.1007/s00415-022-11549-2

Abstract: Evaluation of the application of CSF real-time quaking-induced conversion in Creutzfeldt-Jakob disease surveillance to investigate test accuracy, influencing factors, and associations with disease incidence.In a prospective surveillance study, CSF real-time quaking-induced conversion was performed in patients with clinical suspicion of prion disease (2014-2022). Clinically or histochemically characterized patients with sporadic Creutzfeldt-Jakob disease (n = 888) and patients with final diagnosis of non-prion disease (n = 371) were included for accuracy and association studies.The overall test sensitivity for sporadic Creutzfeldt-Jakob disease was 90% and the specificity 99%. Lower sensitivity was associated with early disease stage (p = 0.029) and longer survival (p < 0.001). The frequency of false positives was significantly higher in patients with inflammatory CNS diseases (3.7%) than in other diagnoses (0.4%, p = 0.027). The incidence increased from 1.7 per million person-years (2006-2017) to 2.0 after the test was added to diagnostic the criteria (2018-2021).We validated high diagnostic accuracy of CSF real-time quaking-induced conversion but identified inflammatory brain disease as a potential source of (rare) false-positive results, indicating thorough consideration of this condition in the differential diagnosis of Creutzfeldt-Jakob disease. The surveillance improved after amendment of the diagnostic criteria, whereas the incidence showed no suggestive alterations during the COVID-19 pandemic.

Keyword(s): Humans (MeSH) ; Creutzfeldt-Jakob Syndrome: diagnosis (MeSH) ; Creutzfeldt-Jakob Syndrome: epidemiology (MeSH) ; Prospective Studies (MeSH) ; Pandemics (MeSH) ; Sensitivity and Specificity (MeSH) ; COVID-19 (MeSH) ; Creutzfeldt–Jakob disease ; Creutzfeldt–Jakob disease ; Diagnosis ; Epidemiology ; Prion ; RT-QuIC

Note: ISSN 1432-1459 not unique: **2 hits**.

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Contributing Institute(s):

1. Translational Studies and Biomarkers (AG Zerr)
2. Ext UMG Zerr (Ext UMG Zerr)

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

Appears in the scientific report 2023

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Database coverage:
; ; ; BIOSIS Previews ; Clarivate Analytics Master Journal List ; Current Contents - Life Sciences ; Ebsco Academic Search ; IF >= 5 ; JCR ; Nationallizenz ; SCOPUS ; Web of Science Core Collection

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