{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T01:39:17Z","timestamp":1773797957711,"version":"3.50.1"},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T00:00:00Z","timestamp":1771545600000},"content-version":"vor","delay-in-days":19,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["EXC 2145 SyNergy - ID 390857198"],"award-info":[{"award-number":["EXC 2145 SyNergy - ID 390857198"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Klinikum der Universit\u00e4t M\u00fcnchen"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Neuropathological diagnoses from the Neurobiobank M\u00fcnchen 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among nine neuropathologically diagnosed neurodegenerative diseases (Alzheimer\u2019s disease, argyrophilic grain disease, corticobasal degeneration, frontotemporal lobar degeneration, Huntington\u2019s 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\u2013100%) whereas specificity was consistently high (89.5\u2013100%). Accuracy was very good (AUC\u2009&gt;\u20090.9) for Huntington\u2019s disease, motor neuron disease, and multiple system atrophy; good (AUC\u2009=\u20090.8\u20130.9) for Alzheimer\u2019s disease, dementia with Lewy bodies\/Parkinson\u2019s disease, and progressive supranuclear palsy; moderate (AUC\u2009=\u20090.7\u20130.8) for frontotemporal dementia, limited (AUC\u2009=\u20090.51\u20130.7) in the\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200920 cases with corticobasal degeneration, and no discriminatory capacity (AUC\u2009=\u20090.5) in the\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20096 cases with argyrophilic grain disease.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00415-026-13680-w","type":"journal-article","created":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T17:24:48Z","timestamp":1771608288000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Accuracy of clinical diagnosis in neurodegenerative diseases\u00a0- a study of 455 autopsy cases"],"prefix":"10.1007","volume":"273","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5965-838X","authenticated-orcid":false,"given":"Jonathan","family":"V\u00f6glein","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Arzberger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irena","family":"Ebner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jochen","family":"Herms","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sigrun","family":"Roeber","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Viktoria","family":"Ruf","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adrian","family":"Danek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Armin","family":"Giese","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00fcnter U.","family":"H\u00f6glinger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes","family":"Levin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,2,20]]},"reference":[{"issue":"Suppl","key":"13680_CR1","doi-asserted-by":"publisher","first-page":"S10","DOI":"10.1038\/nm1066","volume":"10","author":"CA Ross","year":"2004","unstructured":"Ross CA, Poirier MA (2004) Protein aggregation and neurodegenerative disease. 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All relationships outside the submitted work.\u00a0Armin Giese reports employment by and being a shareholder of MODAG GmbH. In addition, he is an inventor on patents related diagnosis and treatment of neurodegenerative diseases. Johannes Levin reports speaker fees from Bayer Vital, Biogen, EISAI, TEVA, Zambon, Esteve, Lilly, BIAL, Merck and Roche, consulting fees from Axon Neuroscience, EISAI, Lilly, Alnylam, Roche and Biogen, author fees from Thieme medical publishers and W. Kohlhammer GmbH medical publishers and is inventor in a patent \u201cOral Phenylbutyrate for Treatment of Human 4-Repeat Tauopathies\u201d (PCT\/EP2024\/053388) filed by LMU Munich. In addition, he reports compensation for serving as chief medical officer for MODAG GmbH, is beneficiary of the phantom share program of MODAG GmbH and is inventor in a patent \u201cPharmaceutical Composition Comprising Diphenyldiazole Derivatives and Methods of Use\u201d (EP 22 159 408.8) filed by MODAG GmbH, all activities outside the submitted work.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"All procedures performed in the study involving human participants were in accordance with the ethical standards of the institutional ethics committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}}],"article-number":"156"}}