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Charting the progression and heterogeneity of TDP-43 accumulation is necessary to better characterize TDP-43 proteinopathies, but current TDP-43 staging systems are heuristic and assume each syndrome is homogeneous. Here, we use data-driven disease progression modelling to derive a fine-grained empirical staging system for the classification and differentiation of frontotemporal lobar degeneration due to TDP-43 (FTLD-TDP, n = 126), amyotrophic lateral sclerosis (ALS, n = 141) and limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC) with and without Alzheimer\u2019s disease (n = 304).<\/jats:p>\n                  <jats:p>The data-driven staging of ALS and FTLD-TDP complement and extend previously described human-defined staging schema for ALS and behavioural variant frontotemporal dementia. In LATE-NC individuals, progression along data-driven stages was positively associated with age, but negatively associated with age in individuals with FTLD-TDP. Using only regional TDP-43 severity, our data driven model distinguished individuals diagnosed with ALS, FTLD-TDP or LATE-NC with a cross-validated accuracy of 85.9%, with misclassifications associated with mixed pathological diagnosis, age and genetic mutations. Adding age and SuStaIn stage to this model increased accuracy to 92.3%. Our model differentiates LATE-NC from FTLD-TDP, though some overlap was observed between late-stage LATE-NC and early-stage FTLD-TDP. We further tested for the presence of subtypes with distinct regional TDP-43 progression patterns within each diagnostic group, identifying two distinct cortical-predominant and brainstem-predominant subtypes within FTLD-TDP and a further two subcortical-predominant and corticolimbic-predominant subtypes within ALS. The FTLD-TDP subtypes exhibited differing proportions of TDP-43 type, while there was a trend for age differing between ALS subtypes. Interestingly, a negative relationship between age and SuStaIn stage was seen in the brainstem\/subcortical-predominant subtype of each proteinopathy. No subtypes were observed for the LATE-NC group, despite aggregating individuals with and without Alzheimer\u2019s disease and a larger sample size for this group.<\/jats:p>\n                  <jats:p>Overall, we provide an empirical pathological TDP-43 staging system for ALS, FTLD-TDP and LATE-NC, which yielded accurate classification. We further demonstrate that there is substantial heterogeneity amongst ALS and FTLD-TDP progression patterns that warrants further investigation in larger cross-cohort studies.<\/jats:p>","DOI":"10.1093\/brain\/awad145","type":"journal-article","created":{"date-parts":[[2023,5,8]],"date-time":"2023-05-08T00:00:56Z","timestamp":1683504056000},"page":"2975-2988","source":"Crossref","is-referenced-by-count":75,"title":["Data-driven neuropathological staging and subtyping of TDP-43 proteinopathies"],"prefix":"10.1093","volume":"146","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7772-781X","authenticated-orcid":false,"given":"Alexandra L","family":"Young","sequence":"first","affiliation":[{"name":"Department of Neuroimaging, Institute of Psychiatry, Psychology and Neuroscience, King\u2019s College London , London, SE5 8AF , UK"},{"name":"Centre for Medical Image Computing, Department of Computer Science, University College London , London, WC1V 6LJ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6394-9940","authenticated-orcid":false,"given":"Jacob W","family":"Vogel","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences, SciLifeLab, Lund University , SE-222 42 Lund , Sweden"},{"name":"Clinical Memory Research Unit, Lund University , SE-222 42 Lund , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John L","family":"Robinson","sequence":"additional","affiliation":[{"name":"Penn Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine , Philadelphia, PA 19104 , USA"},{"name":"Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine , Philadelphia, PA 19104 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corey T","family":"McMillan","sequence":"additional","affiliation":[{"name":"Penn Frontotemporal Degeneration Center, Department of Neurology, Perelman School of Medicine, University of Pennsylvania , Philadelphia, PA 19104 , USA"},{"name":"Department of Neurology, University of Pennsylvania School of Medicine , Philadelphia, PA 19104 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rik","family":"Ossenkoppele","sequence":"additional","affiliation":[{"name":"Clinical Memory Research Unit, Lund University , SE-222 42 Lund , Sweden"},{"name":"Alzheimer Center Amsterdam, Neurology, Vrije Universiteit Amsterdam, Amsterdam UMC location VUmc , 1081 HZ Amsterdam , The Netherlands"},{"name":"Amsterdam Neuroscience, Neurodegeneration , 1081 HV Amsterdam , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A","family":"Wolk","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania School of Medicine , Philadelphia, PA 19104 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5599-5098","authenticated-orcid":false,"given":"David J","family":"Irwin","sequence":"additional","affiliation":[{"name":"Penn Frontotemporal Degeneration Center, Department of Neurology, Perelman School of Medicine, University of Pennsylvania , Philadelphia, PA 19104 , USA"},{"name":"Department of Neurology, University of Pennsylvania School of Medicine , Philadelphia, PA 19104 , USA"},{"name":"Digital Neuropathology Laboratory, Department of Neurology, Perelman School of Medicine, University of Pennsylvania , Philadelphia, PA 19104 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Elman","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania School of Medicine , Philadelphia, PA 19104 , 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