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001     169347
005     20240112171828.0
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037 _ _ |a DZNE-2023-00122
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100 1 _ |a Hermann, Peter
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245 _ _ |a Application of real-time quaking-induced conversion in Creutzfeldt-Jakob disease surveillance.
260 _ _ |a Berlin
|c 2023
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520 _ _ |a 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.
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542 _ _ |i 2023-01-10
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650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Creutzfeldt-Jakob Syndrome: diagnosis
|2 MeSH
650 _ 2 |a Creutzfeldt-Jakob Syndrome: epidemiology
|2 MeSH
650 _ 2 |a Prospective Studies
|2 MeSH
650 _ 2 |a Pandemics
|2 MeSH
650 _ 2 |a Sensitivity and Specificity
|2 MeSH
650 _ 2 |a COVID-19
|2 MeSH
650 _ 7 |a Creutzfeldt–Jakob disease
|2 Other
650 _ 7 |a Creutzfeldt–Jakob disease
|2 Other
650 _ 7 |a Diagnosis
|2 Other
650 _ 7 |a Epidemiology
|2 Other
650 _ 7 |a Prion
|2 Other
650 _ 7 |a RT-QuIC
|2 Other
700 1 _ |a Schmitz, Matthias
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700 1 _ |a Cramm, Maria
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700 1 _ |a Goebel, Stefan
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700 1 _ |a Bunck, Timothy
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700 1 _ |a Schütte-Schmidt, Julia
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700 1 _ |a Schulz-Schaeffer, Walter
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700 1 _ |a Stadelmann, Christine
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700 1 _ |a Matschke, Jakob
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700 1 _ |a Glatzel, Markus
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700 1 _ |a Zerr, Inga
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Marc 21