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@ARTICLE{Hermann:169347,
      author       = {Hermann, Peter and Schmitz, Matthias and Cramm, Maria and
                      Goebel, Stefan and Bunck, Timothy and Schütte-Schmidt,
                      Julia and Schulz-Schaeffer, Walter and Stadelmann, Christine
                      and Matschke, Jakob and Glatzel, Markus and Zerr, Inga},
      title        = {{A}pplication of real-time quaking-induced conversion in
                      {C}reutzfeldt-{J}akob disease surveillance.},
      journal      = {Journal of neurology},
      volume       = {270},
      number       = {4},
      issn         = {0340-5354},
      address      = {Berlin},
      publisher    = {Springer},
      reportid     = {DZNE-2023-00122},
      pages        = {2149-2161},
      year         = {2023},
      note         = {ISSN 1432-1459 not unique: **2 hits**.},
      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.},
      keywords     = {Humans / Creutzfeldt-Jakob Syndrome: diagnosis /
                      Creutzfeldt-Jakob Syndrome: epidemiology / Prospective
                      Studies / Pandemics / Sensitivity and Specificity / COVID-19
                      / Creutzfeldt–Jakob disease (Other) / Creutzfeldt–Jakob
                      disease (Other) / Diagnosis (Other) / Epidemiology (Other) /
                      Prion (Other) / RT-QuIC (Other)},
      cin          = {AG Zerr / Ext UMG Zerr},
      ddc          = {610},
      cid          = {I:(DE-2719)1440011-1 / I:(DE-2719)5000037},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:36624183},
      pmc          = {pmc:PMC9829526},
      doi          = {10.1007/s00415-022-11549-2},
      url          = {https://pub.dzne.de/record/169347},
}