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Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance

BACKGROUND: 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. METHODS: In a prospective surveillance study, CSF real-time quaking-induced conv...

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Autores principales: Hermann, Peter, Schmitz, Matthias, Cramm, Maria, Goebel, Stefan, Bunck, Timothy, Schütte-Schmidt, Julia, Schulz-Schaeffer, Walter, Stadelmann, Christine, Matschke, Jakob, Glatzel, Markus, Zerr, Inga
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9829526/
https://www.ncbi.nlm.nih.gov/pubmed/36624183
http://dx.doi.org/10.1007/s00415-022-11549-2
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author Hermann, Peter
Schmitz, Matthias
Cramm, Maria
Goebel, Stefan
Bunck, Timothy
Schütte-Schmidt, Julia
Schulz-Schaeffer, Walter
Stadelmann, Christine
Matschke, Jakob
Glatzel, Markus
Zerr, Inga
author_facet Hermann, Peter
Schmitz, Matthias
Cramm, Maria
Goebel, Stefan
Bunck, Timothy
Schütte-Schmidt, Julia
Schulz-Schaeffer, Walter
Stadelmann, Christine
Matschke, Jakob
Glatzel, Markus
Zerr, Inga
author_sort Hermann, Peter
collection PubMed
description BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00415-022-11549-2.
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spelling pubmed-98295262023-01-10 Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance Hermann, Peter Schmitz, Matthias Cramm, Maria Goebel, Stefan Bunck, Timothy Schütte-Schmidt, Julia Schulz-Schaeffer, Walter Stadelmann, Christine Matschke, Jakob Glatzel, Markus Zerr, Inga J Neurol Original Communication BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00415-022-11549-2. Springer Berlin Heidelberg 2023-01-10 2023 /pmc/articles/PMC9829526/ /pubmed/36624183 http://dx.doi.org/10.1007/s00415-022-11549-2 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Communication
Hermann, Peter
Schmitz, Matthias
Cramm, Maria
Goebel, Stefan
Bunck, Timothy
Schütte-Schmidt, Julia
Schulz-Schaeffer, Walter
Stadelmann, Christine
Matschke, Jakob
Glatzel, Markus
Zerr, Inga
Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance
title Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance
title_full Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance
title_fullStr Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance
title_full_unstemmed Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance
title_short Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance
title_sort application of real-time quaking-induced conversion in creutzfeldt–jakob disease surveillance
topic Original Communication
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9829526/
https://www.ncbi.nlm.nih.gov/pubmed/36624183
http://dx.doi.org/10.1007/s00415-022-11549-2
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