{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T07:58:31Z","timestamp":1776412711883,"version":"3.51.2"},"reference-count":51,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2020,8,14]],"date-time":"2020-08-14T00:00:00Z","timestamp":1597363200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2020,8,14]],"date-time":"2020-08-14T00:00:00Z","timestamp":1597363200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"funder":[{"name":"NIH\/NIA","award":["R56 AG057191"],"award-info":[{"award-number":["R56 AG057191"]}]},{"name":"NIH\/NIA","award":["U01 AG016976"],"award-info":[{"award-number":["U01 AG016976"]}]},{"name":"NIH\/NIA","award":["R01 NS118584"],"award-info":[{"award-number":["R01 NS118584"]}]},{"name":"NIH\/NIA","award":["R01 AG057187"],"award-info":[{"award-number":["R01 AG057187"]}]},{"name":"NIH\/NIA","award":["R21 AG061551"],"award-info":[{"award-number":["R21 AG061551"]}]},{"name":"NIH\/NIA","award":["R01 AG054060"],"award-info":[{"award-number":["R01 AG054060"]}]},{"name":"NIH\/NIA","award":["the UK-ADC P30 AG028383"],"award-info":[{"award-number":["the UK-ADC P30 AG028383"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2020,11]]},"DOI":"10.1007\/s00401-020-02211-0","type":"journal-article","created":{"date-parts":[[2020,8,14]],"date-time":"2020-08-14T12:55:52Z","timestamp":1597409752000},"page":"659-674","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["Distinct clinicopathologic clusters of persons with TDP-43 proteinopathy"],"prefix":"10.1007","volume":"140","author":[{"given":"Yuriko","family":"Katsumata","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erin L.","family":"Abner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shama","family":"Karanth","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Merilee A.","family":"Teylan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charles N.","family":"Mock","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew D.","family":"Cykowski","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edward B.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin L.","family":"Boehme","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shubhabrata","family":"Mukherjee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John S. K.","family":"Kauwe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard J.","family":"Kryscio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frederick A.","family":"Schmitt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David W.","family":"Fardo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter T.","family":"Nelson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,8,14]]},"reference":[{"key":"2211_CR1","doi-asserted-by":"publisher","first-page":"282","DOI":"10.1001\/jamaneurol.2015.4478","volume":"73","author":"RM Ahmed","year":"2016","unstructured":"Ahmed RM, Irish M, Henning E, Dermody N, Bartley L, Kiernan MC, Piguet O, Farooqi S, Hodges JR (2016) Assessment of eating behavior disturbance and associated neural networks in frontotemporal dementia. JAMA Neurol 73:282\u2013290. https:\/\/doi.org\/10.1001\/jamaneurol.2015.4478","journal-title":"JAMA Neurol"},{"key":"2211_CR2","doi-asserted-by":"publisher","first-page":"1540","DOI":"10.1001\/jamaneurol.2014.1931","volume":"71","author":"RM Ahmed","year":"2014","unstructured":"Ahmed RM, Irish M, Kam J, van Keizerswaard J, Bartley L, Samaras K, Hodges JR, Piguet O (2014) Quantifying the eating abnormalities in frontotemporal dementia. JAMA Neurol 71:1540\u20131546. https:\/\/doi.org\/10.1001\/jamaneurol.2014.1931","journal-title":"JAMA Neurol"},{"key":"2211_CR3","first-page":"146","volume":"64","author":"A Alzheimer","year":"1907","unstructured":"Alzheimer A (1907) \u00dcber eine eigenartige Erkrankung der Hirnrinde. Allgemeine Z Psychiatrie Psychisch-Gerichtliche Med 64:146\u2013148","journal-title":"Allgemeine Z Psychiatrie Psychisch-Gerichtliche Med"},{"key":"2211_CR4","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1002\/ana.21154","volume":"61","author":"C Amador-Ortiz","year":"2007","unstructured":"Amador-Ortiz C, Lin WL, Ahmed Z, Personett D, Davies P, Duara R, Graff-Radford NR, Hutton ML, Dickson DW (2007) TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease. Ann Neurol 61:435\u2013445. https:\/\/doi.org\/10.1002\/ana.21154","journal-title":"Ann Neurol"},{"key":"2211_CR5","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1007\/s00401-014-1358-z","volume":"129","author":"N Aoki","year":"2015","unstructured":"Aoki N, Murray ME, Ogaki K, Fujioka S, Rutherford NJ, Rademakers R, Ross OA, Dickson DW (2015) Hippocampal sclerosis in Lewy body disease is a TDP-43 proteinopathy similar to FTLD-TDP Type A. Acta Neuropathol 129:53\u201364. https:\/\/doi.org\/10.1007\/s00401-014-1358-z","journal-title":"Acta Neuropathol"},{"key":"2211_CR6","doi-asserted-by":"publisher","first-page":"1052","DOI":"10.1001\/archneurol.2011.3323","volume":"69","author":"A Baborie","year":"2012","unstructured":"Baborie A, Griffiths TD, Jaros E, Momeni P, McKeith IG, Burn DJ, Keir G, Larner AJ, Mann DM, Perry R (2012) Frontotemporal dementia in elderly individuals. Arch Neurol 69:1052\u20131060. https:\/\/doi.org\/10.1001\/archneurol.2011.3323","journal-title":"Arch Neurol"},{"key":"2211_CR7","doi-asserted-by":"publisher","first-page":"1","DOI":"10.18637\/jss.v067.i01","volume":"67","author":"D Bates","year":"2015","unstructured":"Bates D, Maechler M, Bolker B, Walker S (2015) Fitting linear mixed-effects models using lme4. J Stat Softw 67:1\u201348","journal-title":"J Stat Softw"},{"key":"2211_CR8","doi-asserted-by":"publisher","first-page":"e1004606","DOI":"10.1371\/journal.pgen.1004606","volume":"10","author":"GW Beecham","year":"2014","unstructured":"Beecham GW, Hamilton K, Naj AC, Martin ER, Huentelman M, Myers AJ, Corneveaux JJ, Hardy J, Vonsattel JP, Younkin SG et al (2014) Genome-wide association meta-analysis of neuropathologic features of Alzheimer's disease and related dementias. PLoS Genet 10:e1004606. https:\/\/doi.org\/10.1371\/journal.pgen.1004606","journal-title":"PLoS Genet"},{"key":"2211_CR9","doi-asserted-by":"publisher","first-page":"717","DOI":"10.1093\/jnen\/nly049","volume":"77","author":"LM Besser","year":"2018","unstructured":"Besser LM, Kukull WA, Teylan MA, Bigio EH, Cairns NJ, Kofler JK, Montine TJ, Schneider JA, Nelson PT (2018) The revised national Alzheimer's coordinating center's neuropathology form-available data and new analyses. J Neuropathol Exp Neurol 77:717\u2013726. https:\/\/doi.org\/10.1093\/jnen\/nly049","journal-title":"J Neuropathol Exp Neurol"},{"key":"2211_CR10","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1007\/BF00305867","volume":"81","author":"H Braak","year":"1991","unstructured":"Braak H, Braak E (1991) Alzheimer's disease affects limbic nuclei of the thalamus. Acta Neuropathol 81:261\u2013268","journal-title":"Acta Neuropathol"},{"key":"2211_CR11","doi-asserted-by":"publisher","first-page":"795","DOI":"10.1007\/s00401-019-02045-5","volume":"138","author":"CP Cali","year":"2019","unstructured":"Cali CP, Patino M, Tai YK, Ho WY, McLean CA, Morris CM, Seeley WW, Miller BL, Gaig C, Vonsattel JPG et al (2019) C9orf72 intermediate repeats are associated with corticobasal degeneration, increased C9orf72 expression and disruption of autophagy. Acta Neuropathol 138:795\u2013811. https:\/\/doi.org\/10.1007\/s00401-019-02045-5","journal-title":"Acta Neuropathol"},{"key":"2211_CR12","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1016\/0197-4580(95)02002-0","volume":"17","author":"S Craft","year":"1996","unstructured":"Craft S, Newcomer J, Kanne S, Dagogo-Jack S, Cryer P, Sheline Y, Luby J, Dagogo-Jack A, Alderson A (1996) Memory improvement following induced hyperinsulinemia in Alzheimer's disease. Neurobiol Aging 17:123\u2013130. https:\/\/doi.org\/10.1016\/0197-4580(95)02002-0","journal-title":"Neurobiol Aging"},{"key":"2211_CR13","doi-asserted-by":"publisher","first-page":"2308","DOI":"10.1212\/wnl.44.12.2308","volume":"44","author":"JL Cummings","year":"1994","unstructured":"Cummings JL, Mega M, Gray K, Rosenberg-Thompson S, Carusi DA, Gornbein J (1994) The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia. Neurology 44:2308\u20132314. https:\/\/doi.org\/10.1212\/wnl.44.12.2308","journal-title":"Neurology"},{"key":"2211_CR14","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.neuron.2011.09.011","volume":"72","author":"M DeJesus-Hernandez","year":"2011","unstructured":"DeJesus-Hernandez M, Mackenzie IR, Boeve BF, Boxer AL, Baker M, Rutherford NJ, Nicholson AM, Finch NA, Flynn H, Adamson J et al (2011) Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72:245\u2013256. https:\/\/doi.org\/10.1016\/j.neuron.2011.09.011","journal-title":"Neuron"},{"key":"2211_CR15","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1159\/000289231","volume":"7","author":"DW Dickson","year":"2010","unstructured":"Dickson DW, Baker M, Rademakers R (2010) Common variant in GRN is a genetic risk factor for hippocampal sclerosis in the elderly. Neuro-degenerat Dis 7:170\u2013174. https:\/\/doi.org\/10.1159\/000289231","journal-title":"Neuro-degenerat Dis"},{"key":"2211_CR16","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1016\/j.parkreldis.2012.10.013","volume":"19","author":"TJ Ferman","year":"2013","unstructured":"Ferman TJ, Arvanitakis Z, Fujishiro H, Duara R, Parfitt F, Purdy M, Waters C, Barker W, Graff-Radford NR, Dickson DW (2013) Pathology and temporal onset of visual hallucinations, misperceptions and family misidentification distinguishes dementia with Lewy bodies from Alzheimer's disease. Parkinsonism Relat Disord 19:227\u2013231. https:\/\/doi.org\/10.1016\/j.parkreldis.2012.10.013","journal-title":"Parkinsonism Relat Disord"},{"key":"2211_CR17","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/0022-3956(75)90026-6","volume":"12","author":"MF Folstein","year":"1975","unstructured":"Folstein MF, Folstein SE, McHugh PR (1975) \"Mini-mental state\". A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189\u2013198","journal-title":"J Psychiatr Res"},{"key":"2211_CR18","doi-asserted-by":"publisher","first-page":"355","DOI":"10.1093\/biostatistics\/kxj011","volume":"7","author":"T Hothorn","year":"2006","unstructured":"Hothorn T, Buhlmann P, Dudoit S, Molinaro A, van der Laan MJ (2006) Survival ensembles. Biostatistics 7:355\u2013373. https:\/\/doi.org\/10.1093\/biostatistics\/kxj011","journal-title":"Biostatistics"},{"key":"2211_CR19","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1136\/jnnp.73.4.371","volume":"73","author":"M Ikeda","year":"2002","unstructured":"Ikeda M, Brown J, Holland AJ, Fukuhara R, Hodges JR (2002) Changes in appetite, food preference, and eating habits in frontotemporal dementia and Alzheimer's disease. J Neurol Neurosurg Psychiatry 73:371\u2013376. https:\/\/doi.org\/10.1136\/jnnp.73.4.371","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"2234","key":"2211_CR20","doi-asserted-by":"publisher","first-page":"e2231","DOI":"10.1016\/j.neurobiolaging.2013.03.003","volume":"34","author":"AR Jones","year":"2013","unstructured":"Jones AR, Woollacott I, Shatunov A, Cooper-Knock J, Buchman V, Sproviero W, Smith B, Scott KM, Balendra R, Abel O et al (2013) Residual association at C9orf72 suggests an alternative amyotrophic lateral sclerosis-causing hexanucleotide repeat. Neurobiol Aging 34(2234):e2231\u20132237. https:\/\/doi.org\/10.1016\/j.neurobiolaging.2013.03.003","journal-title":"Neurobiol Aging"},{"key":"2211_CR21","doi-asserted-by":"publisher","first-page":"e47","DOI":"10.1093\/brain\/awz224","volume":"142","author":"KA Josephs","year":"2019","unstructured":"Josephs KA, Mackenzie I, Frosch MP, Bigio EH, Neumann M, Arai T, Dugger BN, Ghetti B, Grossman M, Hasegawa M et al (2019) Late to the part-y. Brain 142:e47. https:\/\/doi.org\/10.1093\/brain\/awz224","journal-title":"Brain"},{"key":"2211_CR22","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1007\/s00401-013-1211-9","volume":"127","author":"KA Josephs","year":"2014","unstructured":"Josephs KA, Murray ME, Whitwell JL, Parisi JE, Petrucelli L, Jack CR, Petersen RC, Dickson DW (2014) Staging TDP-43 pathology in Alzheimer's disease. Acta Neuropathol 127:441\u2013450. https:\/\/doi.org\/10.1007\/s00401-013-1211-9","journal-title":"Acta Neuropathol"},{"key":"2211_CR23","doi-asserted-by":"publisher","first-page":"571","DOI":"10.1007\/s00401-016-1537-1","volume":"131","author":"KA Josephs","year":"2016","unstructured":"Josephs KA, Murray ME, Whitwell JL, Tosakulwong N, Weigand SD, Petrucelli L, Liesinger AM, Petersen RC, Parisi JE, Dickson DW (2016) Updated TDP-43 in Alzheimer's disease staging scheme. Acta Neuropathol 131:571\u2013585. https:\/\/doi.org\/10.1007\/s00401-016-1537-1","journal-title":"Acta Neuropathol"},{"key":"2211_CR24","doi-asserted-by":"publisher","first-page":"811","DOI":"10.1007\/s00401-014-1269-z","volume":"127","author":"KA Josephs","year":"2014","unstructured":"Josephs KA, Whitwell JL, Weigand SD, Murray ME, Tosakulwong N, Liesinger AM, Petrucelli L, Senjem ML, Knopman DS, Boeve BF et al (2014) TDP-43 is a key player in the clinical features associated with Alzheimer's disease. Acta Neuropathol 127:811\u2013824. https:\/\/doi.org\/10.1007\/s00401-014-1269-z","journal-title":"Acta Neuropathol"},{"key":"2211_CR25","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2020.1741","author":"S Karanth","year":"2020","unstructured":"Karanth S, Nelson PT, Katsumata Y, Kryscio RJ, Schmitt FA, Fardo DW, Cykowski MD, Jicha GA, Van Eldik LJ, Abner EL (2020) Prevalence and clinical phenotype of quadruple misfolded proteins in older adults. JAMA Neurol. https:\/\/doi.org\/10.1001\/jamaneurol.2020.1741","journal-title":"JAMA Neurol"},{"key":"2211_CR26","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1186\/s40478-018-0641-y","volume":"6","author":"Y Katsumata","year":"2018","unstructured":"Katsumata Y, Fardo DW, Kukull WA, Nelson PT (2018) Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer's disease and cerebrovascular disease pathologies. Acta Neuropathol Commun 6:142. https:\/\/doi.org\/10.1186\/s40478-018-0641-y","journal-title":"Acta Neuropathol Commun"},{"key":"2211_CR27","doi-asserted-by":"publisher","first-page":"1888","DOI":"10.1212\/01.wnl.0000188871.74093.12","volume":"65","author":"CA Martins","year":"2005","unstructured":"Martins CA, Oulhaj A, de Jager CA, Williams JH (2005) APOE alleles predict the rate of cognitive decline in Alzheimer disease: a nonlinear model. Neurology 65:1888\u20131893. https:\/\/doi.org\/10.1212\/01.wnl.0000188871.74093.12","journal-title":"Neurology"},{"key":"2211_CR28","volume-title":"Frontiers in ecnometrics","author":"D McFadden","year":"1974","unstructured":"McFadden D (1974) Conditional logit analysis of qualitative choice behaviour. In: Zarembka (ed) Frontiers in ecnometrics. Academic press, Cambridge"},{"key":"2211_CR29","unstructured":"Mclnnes L, Healy J (2018) UMAP: uniform manifold approximation and projection for dimension reduction. arXiv: preprint arXiv:1802.03426"},{"key":"2211_CR30","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1212\/WNL.41.4.479","volume":"41","author":"SS Mirra","year":"1991","unstructured":"Mirra SS, Heyman A, McKeel D, Sumi SM, Crain BJ, Brownlee LM, Vogel FS, Hughes JP, van Belle G, Berg L (1991) The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer's disease. Neurology 41:479\u2013486","journal-title":"Neurology"},{"issue":"209","key":"2211_CR31","doi-asserted-by":"publisher","first-page":"e203","DOI":"10.1016\/j.neurobiolaging.2011.08.005","volume":"33","author":"K Mok","year":"2012","unstructured":"Mok K, Traynor BJ, Schymick J, Tienari PJ, Laaksovirta H, Peuralinna T, Myllykangas L, Chio A, Shatunov A, Boeve BF et al (2012) Chromosome 9 ALS and FTD locus is probably derived from a single founder. Neurobiol Aging 33(209):e203\u2013208. https:\/\/doi.org\/10.1016\/j.neurobiolaging.2011.08.005","journal-title":"Neurobiol Aging"},{"key":"2211_CR32","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1097\/WAD.0000000000000111","volume":"30","author":"SE Monsell","year":"2016","unstructured":"Monsell SE, Dodge HH, Zhou XH, Bu Y, Besser LM, Mock C, Hawes SE, Kukull WA, Weintraub S, Neuropsychology Work Group Advisory to the Clinical Task F (2016) Results from the NACC uniform data set neuropsychological battery crosswalk study. Alzheimer Dis Assoc Disord 30:134\u2013139. https:\/\/doi.org\/10.1097\/WAD.0000000000000111","journal-title":"Alzheimer Dis Assoc Disord"},{"key":"2211_CR33","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s00401-011-0910-3","volume":"123","author":"TJ Montine","year":"2012","unstructured":"Montine TJ, Phelps CH, Beach TG, Bigio EH, Cairns NJ, Dickson DW, Duyckaerts C, Frosch MP, Masliah E, Mirra SS et al (2012) National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease: a practical approach. Acta Neuropathol 123:1\u201311. https:\/\/doi.org\/10.1007\/s00401-011-0910-3","journal-title":"Acta Neuropathol"},{"key":"2211_CR34","doi-asserted-by":"publisher","first-page":"1159","DOI":"10.1212\/WNL.39.9.1159","volume":"39","author":"JC Morris","year":"1989","unstructured":"Morris JC, Heyman A, Mohs RC, Hughes JP, van Belle G, Fillenbaum G, Mellits ED, Clark C (1989) The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part I. Clinical and neuropsychological assessment of Alzheimer's disease. Neurology 39:1159\u20131165","journal-title":"Neurology"},{"key":"2211_CR35","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1007\/s00401-014-1302-2","volume":"128","author":"ME Murray","year":"2014","unstructured":"Murray ME, Cannon A, Graff-Radford NR, Liesinger AM, Rutherford NJ, Ross OA, Duara R, Carrasquillo MM, Rademakers R, Dickson DW (2014) Differential clinicopathologic and genetic features of late-onset amnestic dementias. Acta Neuropathol 128:411\u2013421. https:\/\/doi.org\/10.1007\/s00401-014-1302-2","journal-title":"Acta Neuropathol"},{"key":"2211_CR36","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1186\/s40478-018-0531-3","volume":"6","author":"S Nag","year":"2018","unstructured":"Nag S, Yu L, Boyle PA, Leurgans SE, Bennett DA, Schneider JA (2018) TDP-43 pathology in anterior temporal pole cortex in aging and Alzheimer's disease. Acta Neuropathol Commun 6:33. https:\/\/doi.org\/10.1186\/s40478-018-0531-3","journal-title":"Acta Neuropathol Commun"},{"key":"2211_CR37","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1111\/j.1532-5415.2005.53221.x","volume":"53","author":"ZS Nasreddine","year":"2005","unstructured":"Nasreddine ZS, Phillips NA, Bedirian V, Charbonneau S, Whitehead V, Collin I, Cummings JL, Chertkow H (2005) The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc 53:695\u2013699. https:\/\/doi.org\/10.1111\/j.1532-5415.2005.53221.x","journal-title":"J Am Geriatr Soc"},{"key":"2211_CR38","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1111\/j.1750-3639.2008.00244.x","volume":"20","author":"PT Nelson","year":"2010","unstructured":"Nelson PT, Abner EL, Schmitt FA, Kryscio RJ, Jicha GA, Smith CD, Davis DG, Poduska JW, Patel E, Mendiondo MS et al (2010) Modeling the association between 43 different clinical and pathological variables and the severity of cognitive impairment in a large autopsy cohort of elderly persons. Brain Pathol 20:66\u201379. https:\/\/doi.org\/10.1111\/j.1750-3639.2008.00244.x","journal-title":"Brain Pathol"},{"key":"2211_CR39","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1093\/brain\/awz099","volume":"142","author":"PT Nelson","year":"2019","unstructured":"Nelson PT, Dickson DW, Trojanowski JQ, Jack CR, Boyle PA, Arfanakis K, Rademakers R, Alafuzoff I, Attems J, Brayne C et al (2019) Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working group report. Brain 142:1503\u20131527. https:\/\/doi.org\/10.1093\/brain\/awz099","journal-title":"Brain"},{"key":"2211_CR40","doi-asserted-by":"publisher","first-page":"825","DOI":"10.1007\/s00401-014-1282-2","volume":"127","author":"PT Nelson","year":"2014","unstructured":"Nelson PT, Estus S, Abner EL, Parikh I, Malik M, Neltner JH, Ighodaro E, Wang WX, Wilfred BR, Wang LS et al (2014) ABCC9 gene polymorphism is associated with hippocampal sclerosis of aging pathology. Acta Neuropathol 127:825\u2013843. https:\/\/doi.org\/10.1007\/s00401-014-1282-2","journal-title":"Acta Neuropathol"},{"key":"2211_CR41","doi-asserted-by":"publisher","first-page":"721","DOI":"10.1093\/brain\/awm331","volume":"131","author":"SM Pickering-Brown","year":"2008","unstructured":"Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AM, Neary D, Snowden JS, Mann DM (2008) Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations. Brain 131:721\u2013731. https:\/\/doi.org\/10.1093\/brain\/awm331","journal-title":"Brain"},{"key":"2211_CR42","unstructured":"R Core Team (2020) R: a language and environment for statistical computing. R Foundation for Statistical Computing, Vienna. https:\/\/www.R-roject.org\/"},{"key":"2211_CR43","doi-asserted-by":"publisher","first-page":"3631","DOI":"10.1093\/hmg\/ddn257","volume":"17","author":"R Rademakers","year":"2008","unstructured":"Rademakers R, Eriksen JL, Baker M, Robinson T, Ahmed Z, Lincoln SJ, Finch N, Rutherford NJ, Crook RJ, Josephs KA et al (2008) Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia. Hum Mol Genet 17:3631\u20133642. https:\/\/doi.org\/10.1093\/hmg\/ddn257","journal-title":"Hum Mol Genet"},{"key":"2211_CR44","doi-asserted-by":"publisher","first-page":"717","DOI":"10.1212\/WNL.0b013e318264e3ac","volume":"79","author":"NJ Rutherford","year":"2012","unstructured":"Rutherford NJ, Carrasquillo MM, Li M, Bisceglio G, Menke J, Josephs KA, Parisi JE, Petersen RC, Graff-Radford NR, Younkin SG et al (2012) TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease. Neurology 79:717\u2013718. https:\/\/doi.org\/10.1212\/WNL.0b013e318264e3ac","journal-title":"Neurology"},{"key":"2211_CR45","unstructured":"Signorell A et al (2020) DescTools: tools for descriptive statistics. R package version 0.99.36"},{"key":"2211_CR46","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1186\/1471-2105-9-307","volume":"9","author":"C Strobl","year":"2008","unstructured":"Strobl C, Boulesteix AL, Kneib T, Augustin T, Zeileis A (2008) Conditional variable importance for random forests. BMC Bioinf 9:307. https:\/\/doi.org\/10.1186\/1471-2105-9-307","journal-title":"BMC Bioinf"},{"key":"2211_CR47","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1186\/1471-2105-8-25","volume":"8","author":"C Strobl","year":"2007","unstructured":"Strobl C, Boulesteix AL, Zeileis A, Hothorn T (2007) Bias in random forest variable importance measures: illustrations, sources and a solution. BMC Bioinf 8:25. https:\/\/doi.org\/10.1186\/1471-2105-8-25","journal-title":"BMC Bioinf"},{"key":"2211_CR48","doi-asserted-by":"publisher","first-page":"1791","DOI":"10.1212\/WNL.58.12.1791","volume":"58","author":"DR Thal","year":"2002","unstructured":"Thal DR, Rub U, Orantes M, Braak H (2002) Phases of A beta-deposition in the human brain and its relevance for the development of AD. Neurology 58:1791\u20131800","journal-title":"Neurology"},{"key":"2211_CR49","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1186\/s40478-020-00934-5","volume":"8","author":"SO Tome","year":"2020","unstructured":"Tome SO, Vandenberghe R, Ospitalieri S, Van Schoor E, Tousseyn T, Otto M, von Arnim CAF, Thal DR (2020) Distinct molecular patterns of TDP-43 pathology in Alzheimer's disease: relationship with clinical phenotypes. Acta Neuropathol Commun 8:61. https:\/\/doi.org\/10.1186\/s40478-020-00934-5","journal-title":"Acta Neuropathol Commun"},{"key":"2211_CR50","volume-title":"Wechsler adult intelligence scale-revised","author":"D Wechsler","year":"1987","unstructured":"Wechsler D (1987) Wechsler adult intelligence scale-revised. Psychological Corporation, San Antonio, TX"},{"key":"2211_CR51","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1097\/WAD.0b013e318191c7dd","volume":"23","author":"S Weintraub","year":"2009","unstructured":"Weintraub S, Salmon D, Mercaldo N, Ferris S, Graff-Radford NR, Chui H, Cummings J, DeCarli C, Foster NL, Galasko D et al (2009) The Alzheimer's disease centers' uniform data set (UDS): the neuropsychologic test battery. Alzheimer Dis Assoc Disord 23:91\u2013101. https:\/\/doi.org\/10.1097\/WAD.0b013e318191c7dd","journal-title":"Alzheimer Dis Assoc Disord"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-020-02211-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00401-020-02211-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-020-02211-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,8,13]],"date-time":"2021-08-13T23:29:14Z","timestamp":1628897354000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00401-020-02211-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,14]]},"references-count":51,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,11]]}},"alternative-id":["2211"],"URL":"https:\/\/doi.org\/10.1007\/s00401-020-02211-0","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,8,14]]},"assertion":[{"value":"2 June 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 August 2020","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 August 2020","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 August 2020","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"The authors declare that they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}