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000285350 0247_ $$2ISSN$$a1432-1459
000285350 037__ $$aDZNE-2026-00216
000285350 041__ $$aEnglish
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000285350 1001_ $$0P:(DE-2719)2811820$$aVöglein, Jonathan$$b0$$eFirst author
000285350 245__ $$aAccuracy of clinical diagnosis in neurodegenerative diseases - a study of 455 autopsy cases.
000285350 260__ $$a[Darmstadt]$$bSteinkopff$$c2026
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000285350 520__ $$aPrecision of clinical diagnosis in neurodegenerative diseases is critically important for clinical care and study recruitment. This study aimed to investigate the clinical accuracy using gold-standard neuropathological reference.Neuropathological diagnoses from the Neurobiobank München were correlated with real-world clinical diagnoses from hospitals in Germany. Accuracy metrics, including sensitivity, specificity, and area under the curve (AUC) of clinical diagnoses, were calculated.Among nine neuropathologically diagnosed neurodegenerative diseases (Alzheimer's disease, argyrophilic grain disease, corticobasal degeneration, frontotemporal lobar degeneration, Huntington's disease, Lewy body disease, motor neuron disease, multiple system atrophy, and progressive supranuclear palsy) with a total of 455 cases, clinical sensitivity varied widely (0-100%) whereas specificity was consistently high (89.5-100%). Accuracy was very good (AUC > 0.9) for Huntington's disease, motor neuron disease, and multiple system atrophy; good (AUC = 0.8-0.9) for Alzheimer's disease, dementia with Lewy bodies/Parkinson's disease, and progressive supranuclear palsy; moderate (AUC = 0.7-0.8) for frontotemporal dementia, limited (AUC = 0.51-0.7) in the n = 20 cases with corticobasal degeneration, and no discriminatory capacity (AUC = 0.5) in the n = 6 cases with argyrophilic grain disease.Clinical diagnostic accuracy of neurodegenerative diseases varies, with sensitivity as the main limiting factor. Improving diagnostic sensitivity will be essential for early and accurate patient identification, especially as disease-modifying therapies targeting causal proteinopathies become available. Achieving this will depend on the development and clinical implementation of reliable molecular biomarkers that indicate the causal proteinopathies of neurodegenerative diseases.
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000285350 650_7 $$2Other$$aClinical diagnosis
000285350 650_7 $$2Other$$aClinical–neuropathological correlation
000285350 650_7 $$2Other$$aDiagnostic accuracy
000285350 650_7 $$2Other$$aNeurodegenerative diseases
000285350 650_7 $$2Other$$aNeuropathology
000285350 650_2 $$2MeSH$$aHumans
000285350 650_2 $$2MeSH$$aNeurodegenerative Diseases: diagnosis
000285350 650_2 $$2MeSH$$aNeurodegenerative Diseases: pathology
000285350 650_2 $$2MeSH$$aMale
000285350 650_2 $$2MeSH$$aFemale
000285350 650_2 $$2MeSH$$aAged
000285350 650_2 $$2MeSH$$aMiddle Aged
000285350 650_2 $$2MeSH$$aAutopsy
000285350 650_2 $$2MeSH$$aAged, 80 and over
000285350 650_2 $$2MeSH$$aSensitivity and Specificity
000285350 650_2 $$2MeSH$$aAdult
000285350 7001_ $$0P:(DE-2719)2811333$$aArzberger, Thomas$$b1$$udzne
000285350 7001_ $$aEbner, Irena$$b2
000285350 7001_ $$0P:(DE-2719)2810441$$aHerms, Jochen$$b3$$udzne
000285350 7001_ $$aRoeber, Sigrun$$b4
000285350 7001_ $$aRuf, Viktoria$$b5
000285350 7001_ $$0P:(DE-2719)2810712$$aDanek, Adrian$$b6$$udzne
000285350 7001_ $$aGiese, Armin$$b7
000285350 7001_ $$0P:(DE-2719)2811373$$aHöglinger, Günter U$$b8$$udzne
000285350 7001_ $$0P:(DE-2719)2811659$$aLevin, Johannes$$b9$$eLast author$$udzne
000285350 773__ $$0PERI:(DE-600)1421299-7$$a10.1007/s00415-026-13680-w$$gVol. 273, no. 2, p. 156$$n2$$p156$$tJournal of neurology$$v273$$x0367-004X$$y2026
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