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@ARTICLE{Vglein:285350,
      author       = {Vöglein, Jonathan and Arzberger, Thomas and Ebner, Irena
                      and Herms, Jochen and Roeber, Sigrun and Ruf, Viktoria and
                      Danek, Adrian and Giese, Armin and Höglinger, Günter U and
                      Levin, Johannes},
      title        = {{A}ccuracy of clinical diagnosis in neurodegenerative
                      diseases - a study of 455 autopsy cases.},
      journal      = {Journal of neurology},
      volume       = {273},
      number       = {2},
      issn         = {0367-004X},
      address      = {[Darmstadt]},
      publisher    = {Steinkopff},
      reportid     = {DZNE-2026-00216},
      pages        = {156},
      year         = {2026},
      abstract     = {Precision of clinical diagnosis in neurodegenerative
                      diseases is critically important for clinical care and study
                      recruitment. This study aimed to investigate the clinical
                      accuracy using gold-standard neuropathological
                      reference.Neuropathological diagnoses from the Neurobiobank
                      München were correlated with real-world clinical diagnoses
                      from hospitals in Germany. Accuracy metrics, including
                      sensitivity, specificity, and area under the curve (AUC) of
                      clinical diagnoses, were calculated.Among nine
                      neuropathologically diagnosed neurodegenerative diseases
                      (Alzheimer's disease, argyrophilic grain disease,
                      corticobasal degeneration, frontotemporal lobar
                      degeneration, Huntington's disease, Lewy body disease, motor
                      neuron disease, multiple system atrophy, and progressive
                      supranuclear palsy) with a total of 455 cases, clinical
                      sensitivity varied widely $(0-100\%)$ whereas specificity
                      was consistently high $(89.5-100\%).$ Accuracy was very good
                      (AUC > 0.9) for Huntington's disease, motor neuron disease,
                      and multiple system atrophy; good (AUC = 0.8-0.9) for
                      Alzheimer's disease, dementia with Lewy bodies/Parkinson's
                      disease, and progressive supranuclear palsy; moderate (AUC =
                      0.7-0.8) for frontotemporal dementia, limited (AUC =
                      0.51-0.7) in the n = 20 cases with corticobasal
                      degeneration, and no discriminatory capacity (AUC = 0.5) in
                      the n = 6 cases with argyrophilic grain disease.Clinical
                      diagnostic accuracy of neurodegenerative diseases varies,
                      with sensitivity as the main limiting factor. Improving
                      diagnostic sensitivity will be essential for early and
                      accurate patient identification, especially as
                      disease-modifying therapies targeting causal proteinopathies
                      become available. Achieving this will depend on the
                      development and clinical implementation of reliable
                      molecular biomarkers that indicate the causal
                      proteinopathies of neurodegenerative diseases.},
      keywords     = {Humans / Neurodegenerative Diseases: diagnosis /
                      Neurodegenerative Diseases: pathology / Male / Female / Aged
                      / Middle Aged / Autopsy / Aged, 80 and over / Sensitivity
                      and Specificity / Adult / Clinical diagnosis (Other) /
                      Clinical–neuropathological correlation (Other) /
                      Diagnostic accuracy (Other) / Neurodegenerative diseases
                      (Other) / Neuropathology (Other)},
      cin          = {Clinical Research (Munich) / AG Levin / AG Herms},
      ddc          = {610},
      cid          = {I:(DE-2719)1111015 / I:(DE-2719)1111016 /
                      I:(DE-2719)1110001},
      pnm          = {353 - Clinical and Health Care Research (POF4-353) / 352 -
                      Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41721140},
      pmc          = {pmc:PMC12923403},
      doi          = {10.1007/s00415-026-13680-w},
      url          = {https://pub.dzne.de/record/285350},
}