{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T18:03:23Z","timestamp":1792605803525,"version":"4.5.0"},"reference-count":132,"publisher":"Springer Science and Business Media LLC","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2011,8]]},"DOI":"10.1007\/s00401-011-0839-6","type":"journal-article","created":{"date-parts":[[2011,5,25]],"date-time":"2011-05-25T00:52:17Z","timestamp":1306284737000},"page":"137-153","source":"Crossref","is-referenced-by-count":408,"title":["Neuropathological background of phenotypical variability in frontotemporal dementia"],"prefix":"10.1007","volume":"122","author":[{"given":"Keith A.","family":"Josephs","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R.","family":"Hodges","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie S.","family":"Snowden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian R.","family":"Mackenzie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Neumann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M.","family":"Mann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis W.","family":"Dickson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2011,5,26]]},"reference":[{"key":"839_CR1","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.bbrc.2006.10.093","volume":"351","author":"T Arai","year":"2006","unstructured":"Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, Mori H, Mann D, Tsuchiya K, Yoshida M, Hashizume Y, Oda T (2006) TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 351:602\u2013611","journal-title":"Biochem Biophys Res Commun"},{"key":"839_CR2","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1002\/ana.10793","volume":"55","author":"T Arai","year":"2004","unstructured":"Arai T, Ikeda K, Akiyama H, Nonaka T, Hasegawa M, Ishiguro K, Iritani S, Tsuchiya K, Iseki E, Yagishita S, Oda T, Mochizuki A (2004) Identification of amino-terminally cleaved tau fragments that distinguish progressive supranuclear palsy from corticobasal degeneration. Ann Neurol 55:72\u201379","journal-title":"Ann Neurol"},{"key":"839_CR3","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1007\/s00702-009-0350-6","volume":"117","author":"RA Armstrong","year":"2010","unstructured":"Armstrong RA, Ellis W, Hamilton RL, Mackenzie IR, Hedreen J, Gearing M, Montine T, Vonsattel JP, Head E, Lieberman AP, Cairns NJ (2010) Neuropathological heterogeneity in frontotemporal lobar degeneration with TDP-43 proteinopathy: a quantitative study of 94 cases using principal components analysis. J Neural Transm 117:227\u2013239","journal-title":"J Neural Transm"},{"key":"839_CR4","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1136\/jnnp.2008.157974","volume":"81","author":"TH Bak","year":"2010","unstructured":"Bak TH, Crawford LM, Berrios G, Hodges JR (2010) Behavioural symptoms in progressive supranuclear palsy and frontotemporal dementia. J Neurol Neurosurg Psychiatry 81:1057\u20131059","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"839_CR5","doi-asserted-by":"crossref","first-page":"916","DOI":"10.1038\/nature05016","volume":"442","author":"M Baker","year":"2006","unstructured":"Baker M, Mackenzie IR, Pickering-Brown SM, Gass J, Rademakers R, Lindholm C, Snowden J, Adamson J, Sadovnick AD, Rollinson S, Cannon A, Dwosh E, Neary D, Melquist S, Richardson A, Dickson D, Berger Z, Eriksen J, Robinson T, Zehr C, Dickey CA, Crook R, McGowan E, Mann D, Boeve B, Feldman H, Hutton M (2006) Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature 442:916\u2013919","journal-title":"Nature"},{"key":"839_CR6","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1002\/ana.21612","volume":"65","author":"L Benajiba","year":"2009","unstructured":"Benajiba L, Le Ber I, Camuzat A, Lacoste M, Thomas-Anterion C, Couratier P, Legallic S, Salachas F, Hannequin D, Decousus M, Lacomblez L, Guedj E, Golfier V, Camu W, Dubois B, Campion D, Meininger V, Brice A (2009) TARDBP mutations in motoneuron disease with frontotemporal lobar degeneration. Ann Neurol 65:470\u2013473","journal-title":"Ann Neurol"},{"key":"839_CR7","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1093\/jnen\/60.4.328","volume":"60","author":"EH Bigio","year":"2001","unstructured":"Bigio EH, Lipton AM, Yen SH, Hutton ML, Baker M, Nacharaju P, White CL 3rd, Davies P, Lin W, Dickson DW (2001) Frontal lobe dementia with novel tauopathy: sporadic multiple system tauopathy with dementia. J Neuropathol Exp Neurol 60:328\u2013341","journal-title":"J Neuropathol Exp Neurol"},{"issue":"Suppl 5","key":"839_CR8","doi-asserted-by":"crossref","first-page":"S15","DOI":"10.1002\/ana.10570","volume":"54","author":"BF Boeve","year":"2003","unstructured":"Boeve BF, Lang AE, Litvan I (2003) Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54(Suppl 5):S15\u2013S19","journal-title":"Ann Neurol"},{"key":"839_CR9","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1212\/WNL.53.4.795","volume":"53","author":"BF Boeve","year":"1999","unstructured":"Boeve BF, Maraganore DM, Parisi JE, Ahlskog JE, Graff-Radford N, Caselli RJ, Dickson DW, Kokmen E, Petersen RC (1999) Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 53:795\u2013800","journal-title":"Neurology"},{"key":"839_CR10","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1017\/S1041610209008862","volume":"21","author":"B Borroni","year":"2009","unstructured":"Borroni B, Alberici A, Agosti C, Cosseddu M, Padovani A (2009) Pattern of behavioral disturbances in corticobasal degeneration syndrome and progressive supranuclear palsy. Int Psychogeriatr 21:463\u2013468","journal-title":"Int Psychogeriatr"},{"key":"839_CR11","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/0304-3940(87)90204-7","volume":"76","author":"H Braak","year":"1987","unstructured":"Braak H, Braak E (1987) Argyrophilic grains: characteristic pathology of cerebral cortex in cases of adult onset dementia without Alzheimer changes. Neurosci Lett 76:124\u2013127","journal-title":"Neurosci Lett"},{"key":"839_CR12","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1111\/j.1365-2990.1989.tb01146.x","volume":"15","author":"H Braak","year":"1989","unstructured":"Braak H, Braak E (1989) Cortical and subcortical argyrophilic grains characterize a disease associated with adult onset dementia. Neuropathol Appl Neurobiol 15:13\u201326","journal-title":"Neuropathol Appl Neurobiol"},{"key":"839_CR13","doi-asserted-by":"crossref","first-page":"2645","DOI":"10.1093\/brain\/awh591","volume":"128","author":"IF Bronner","year":"2005","unstructured":"Bronner IF, ter Meulen BC, Azmani A, Severijnen LA, Willemsen R, Kamphorst W, Ravid R, Heutink P, van Swieten JC (2005) Hereditary Pick\u2019s disease with the G272V tau mutation shows predominant three-repeat tau pathology. Brain 128:2645\u20132653","journal-title":"Brain"},{"key":"839_CR14","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1136\/jnnp.57.4.416","volume":"57","author":"A Brun","year":"1994","unstructured":"Brun A (1994) Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 57:416\u2013418","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"839_CR15","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1007\/s00401-007-0237-2","volume":"114","author":"NJ Cairns","year":"2007","unstructured":"Cairns NJ, Bigio EH, Mackenzie IR, Neumann M, Lee VM, Hatanpaa KJ, White CL 3rd, Schneider JA, Grinberg LT, Halliday G, Duyckaerts C, Lowe JS, Holm IE, Tolnay M, Okamoto K, Yokoo H, Murayama S, Woulfe J, Munoz DG, Dickson DW, Ince PG, Trojanowski JQ, Mann DM (2007) Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol 114:5\u201322","journal-title":"Acta Neuropathol"},{"key":"839_CR16","doi-asserted-by":"crossref","first-page":"1376","DOI":"10.1212\/01.WNL.0000139809.16817.DD","volume":"63","author":"NJ Cairns","year":"2004","unstructured":"Cairns NJ, Grossman M, Arnold SE, Burn DJ, Jaros E, Perry RH, Duyckaerts C, Stankoff B, Pillon B, Skullerud K, Cruz-Sanchez FF, Bigio EH, Mackenzie IR, Gearing M, Juncos JL, Glass JD, Yokoo H, Nakazato Y, Mosaheb S, Thorpe JR, Uryu K, Lee VM, Trojanowski JQ (2004) Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease. Neurology 63:1376\u20131384","journal-title":"Neurology"},{"key":"839_CR17","doi-asserted-by":"crossref","first-page":"227","DOI":"10.2353\/ajpath.2007.070182","volume":"171","author":"NJ Cairns","year":"2007","unstructured":"Cairns NJ, Neumann M, Bigio EH, Holm IE, Troost D, Hatanpaa KJ, Foong C, White CL 3rd, Schneider JA, Kretzschmar HA, Carter D, Taylor-Reinwald L, Paulsmeyer K, Strider J, Gitcho M, Goate AM, Morris JC, Mishra M, Kwong LK, Stieber A, Xu Y, Forman MS, Trojanowski JQ, Lee VM, Mackenzie IR (2007) TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions. Am J Pathol 171:227\u2013240","journal-title":"Am J Pathol"},{"key":"839_CR18","doi-asserted-by":"crossref","first-page":"2153","DOI":"10.1016\/S0002-9440(10)63773-X","volume":"164","author":"NJ Cairns","year":"2004","unstructured":"Cairns NJ, Zhukareva V, Uryu K, Zhang B, Bigio E, Mackenzie IR, Gearing M, Duyckaerts C, Yokoo H, Nakazato Y, Jaros E, Perry RH, Lee VM, Trojanowski JQ (2004) Alpha-internexin is present in the pathological inclusions of neuronal intermediate filament inclusion disease. Am J Pathol 164:2153\u20132161","journal-title":"Am J Pathol"},{"key":"839_CR19","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1002\/ana.410330210","volume":"33","author":"RJ Caselli","year":"1993","unstructured":"Caselli RJ, Windebank AJ, Petersen RC, Komori T, Parisi JE, Okazaki H, Kokmen E, Iverson R, Dinapoli RP, Graff-Radford NR et al (1993) Rapidly progressive aphasic dementia and motor neuron disease. Ann Neurol 33:200\u2013207","journal-title":"Ann Neurol"},{"key":"839_CR20","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1002\/1531-8249(199909)46:3<325::AID-ANA8>3.0.CO;2-V","volume":"46","author":"CB Chambers","year":"1999","unstructured":"Chambers CB, Lee JM, Troncoso JC, Reich S, Muma NA (1999) Overexpression of four-repeat tau mRNA isoforms in progressive supranuclear palsy but not in Alzheimer\u2019s disease. Ann Neurol 46:325\u2013332","journal-title":"Ann Neurol"},{"key":"839_CR21","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1002\/ana.92","volume":"49","author":"D Chan","year":"2001","unstructured":"Chan D, Fox NC, Scahill RI, Crum WR, Whitwell JL, Leschziner G, Rossor AM, Stevens JM, Cipolotti L, Rossor MN (2001) Patterns of temporal lobe atrophy in semantic dementia and Alzheimer\u2019s disease. Ann Neurol 49:433\u2013442","journal-title":"Ann Neurol"},{"key":"839_CR22","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1097\/WNN.0b013e3181864a09","volume":"21","author":"DO Claassen","year":"2008","unstructured":"Claassen DO, Parisi JE, Giannini C, Boeve BF, Dickson DW, Josephs KA (2008) Frontotemporal dementia mimicking dementia with Lewy bodies. Cogn Behav Neurol 21:157\u2013163","journal-title":"Cogn Behav Neurol"},{"key":"839_CR23","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1001\/archneur.1995.00540340103019","volume":"52","author":"PN Cooper","year":"1995","unstructured":"Cooper PN, Jackson M, Lennox G, Lowe J, Mann DM (1995) Tau, ubiquitin, and alpha B-crystallin immunohistochemistry define the principal causes of degenerative frontotemporal dementia. Arch Neurol 52:1011\u20131015","journal-title":"Arch Neurol"},{"key":"839_CR24","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1038\/nature05017","volume":"442","author":"M Cruts","year":"2006","unstructured":"Cruts M, Gijselinck I, van der Zee J, Engelborghs S, Wils H, Pirici D, Rademakers R, Vandenberghe R, Dermaut B, Martin JJ, van Duijn C, Peeters K, Sciot R, Santens P, De Pooter T, Mattheijssens M, Van den Broeck M, Cuijt I, Vennekens K, De Deyn PP, Kumar-Singh S, Van Broeckhoven C (2006) Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature 442:920\u2013924","journal-title":"Nature"},{"key":"839_CR25","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1007\/s00401-006-0189-y","volume":"113","author":"Y Davidson","year":"2007","unstructured":"Davidson Y, Kelley T, Mackenzie IR, Pickering-Brown S, Du Plessis D, Neary D, Snowden JS, Mann DM (2007) Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43. Acta Neuropathol 113:521\u2013533","journal-title":"Acta Neuropathol"},{"key":"839_CR26","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1212\/WNL.0b013e3181c7198e","volume":"74","author":"V Deramecourt","year":"2010","unstructured":"Deramecourt V, Lebert F, Debachy B, Mackowiak-Cordoliani MA, Bombois S, Kerdraon O, Buee L, Maurage CA, Pasquier F (2010) Prediction of pathology in primary progressive language and speech disorders. Neurology 74:42\u201349","journal-title":"Neurology"},{"issue":"Suppl 2","key":"839_CR27","doi-asserted-by":"crossref","first-page":"II6","DOI":"10.1007\/BF03161076","volume":"246","author":"DW Dickson","year":"1999","unstructured":"Dickson DW (1999) Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration. J Neurol 246(Suppl 2):II6\u2013II15","journal-title":"J Neurol"},{"key":"839_CR28","doi-asserted-by":"crossref","first-page":"S16","DOI":"10.1212\/WNL.56.suppl_4.S16","volume":"56","author":"DW Dickson","year":"2001","unstructured":"Dickson DW (2001) Neuropathology of Pick\u2019s disease. Neurology 56:S16\u2013S20","journal-title":"Neurology"},{"key":"839_CR29","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1111\/j.1750-3639.1998.tb00158.x","volume":"8","author":"DW Dickson","year":"1998","unstructured":"Dickson DW (1998) Pick\u2019s disease: a modern approach. Brain Pathol 8:339\u2013354","journal-title":"Brain Pathol"},{"key":"839_CR30","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1093\/jnen\/61.11.935","volume":"61","author":"DW Dickson","year":"2002","unstructured":"Dickson DW, Bergeron C, Chin SS, Duyckaerts C, Horoupian D, Ikeda K, Jellinger K, Lantos PL, Lippa CF, Mirra SS, Tabaton M, Vonsattel JP, Wakabayashi K, Litvan I (2002) Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 61:935\u2013946","journal-title":"J Neuropathol Exp Neurol"},{"key":"839_CR31","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1002\/ana.20873","volume":"59","author":"MS Forman","year":"2006","unstructured":"Forman MS, Farmer J, Johnson JK, Clark CM, Arnold SE, Coslett HB, Chatterjee A, Hurtig HI, Karlawish JH, Rosen HJ, Van Deerlin V, Lee VM, Miller BL, Trojanowski JQ, Grossman M (2006) Frontotemporal dementia: clinicopathological correlations. Ann Neurol 59:952\u2013962","journal-title":"Ann Neurol"},{"key":"839_CR32","first-page":"86","volume-title":"Neurodegeneration: the molecular pathology of dementia and movement disorders","author":"B Ghetti","year":"2003","unstructured":"Ghetti B, Hutton M, Wszolek Z (2003) Frontotemporal dementia and parkinsonism linked to chromosome 17 associated with tau gene mutations (FTDP-17t). In: Dickson D (ed) Neurodegeneration: the molecular pathology of dementia and movement disorders. ISN Neuropath Press, Basel, pp 86\u2013102"},{"issue":"Pt 5","key":"839_CR33","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1093\/brain\/112.5.1171","volume":"112","author":"WR Gibb","year":"1989","unstructured":"Gibb WR, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112(Pt 5):1171\u20131192","journal-title":"Brain"},{"key":"839_CR34","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1212\/WNL.0b013e31821103e6","volume":"76","author":"ML Gorno-Tempini","year":"2011","unstructured":"Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, Ogar JM, Rohrer JD, Black S, Boeve BF, Manes F, Dronkers NF, Vandenberghe R, Rascovsky K, Patterson K, Miller BL, Knopman DS, Hodges JR, Mesulam MM, Grossman M (2011) Classification of primary progressive aphasia and its variants. Neurology 76:1006\u20131014","journal-title":"Neurology"},{"key":"839_CR35","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1001\/archneur.64.10.1449","volume":"64","author":"M Grossman","year":"2007","unstructured":"Grossman M, Wood EM, Moore P, Neumann M, Kwong L, Forman MS, Clark CM, McCluskey LF, Miller BL, Lee VM, Trojanowski JQ (2007) TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Arch Neurol 64:1449\u20131454","journal-title":"Arch Neurol"},{"key":"839_CR36","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1212\/01.WNL.0000034763.54161.1F","volume":"59","author":"S Gydesen","year":"2002","unstructured":"Gydesen S, Brown JM, Brun A, Chakrabarti L, Gade A, Johannsen P, Rossor M, Thusgaard T, Grove A, Yancopoulou D, Spillantini MG, Fisher EM, Collinge J, Sorensen SA (2002) Chromosome 3 linked frontotemporal dementia (FTD-3). Neurology 59:1585\u20131594","journal-title":"Neurology"},{"key":"839_CR37","doi-asserted-by":"crossref","first-page":"2015","DOI":"10.1212\/WNL.44.11.2015","volume":"44","author":"JJ Hauw","year":"1994","unstructured":"Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, Lantos PL, McKee A, Tabaton M, Litvan I (1994) Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 44:2015\u20132019","journal-title":"Neurology"},{"key":"839_CR38","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1002\/ana.20203","volume":"56","author":"JR Hodges","year":"2004","unstructured":"Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH, Kril JJ, Halliday GM (2004) Clinicopathological correlates in frontotemporal dementia. Ann Neurol 56:399\u2013406","journal-title":"Ann Neurol"},{"key":"839_CR39","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1093\/brain\/awp248","volume":"133","author":"JR Hodges","year":"2010","unstructured":"Hodges JR, Mitchell J, Dawson K, Spillantini MG, Xuereb JH, McMonagle P, Nestor PJ, Patterson K (2010) Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain 133:300\u2013306","journal-title":"Brain"},{"key":"839_CR40","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1007\/s00401-009-0593-1","volume":"118","author":"IE Holm","year":"2009","unstructured":"Holm IE, Isaacs AM, Mackenzie IR (2009) Absence of FUS-immunoreactive pathology in frontotemporal dementia linked to chromosome 3 (FTD-3) caused by mutation in the CHMP2B gene. Acta Neuropathol 118:719\u2013720","journal-title":"Acta Neuropathol"},{"key":"839_CR41","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1002\/mds.22574","volume":"24","author":"WT Hu","year":"2009","unstructured":"Hu WT, Rippon GW, Boeve BF, Knopman DS, Petersen RC, Parisi JE, Josephs KA (2009) Alzheimer\u2019s disease and corticobasal degeneration presenting as corticobasal syndrome. Mov Disord 24:1375\u20131379","journal-title":"Mov Disord"},{"key":"839_CR42","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1001\/archneurol.2009.253","volume":"66","author":"WT Hu","year":"2009","unstructured":"Hu WT, Seelaar H, Josephs KA, Knopman DS, Boeve BF, Sorenson EJ, McCluskey L, Elman L, Schelhaas HJ, Parisi JE, Kuesters B, Lee VM, Trojanowski JQ, Petersen RC, van Swieten JC, Grossman M (2009) Survival profiles of patients with frontotemporal dementia and motor neuron disease. Arch Neurol 66:1359\u20131364","journal-title":"Arch Neurol"},{"key":"839_CR43","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1038\/31508","volume":"393","author":"M Hutton","year":"1998","unstructured":"Hutton M, Lendon CL, Rizzu P, Baker M, Froelich S, Houlden H, Pickering-Brown S, Chakraverty S, Isaacs A, Grover A, Hackett J, Adamson J, Lincoln S, Dickson D, Davies P, Petersen RC, Stevens M, de Graaff E, Wauters E, van Baren J, Hillebrand M, Joosse M, Kwon JM, Nowotny P, Che LK, Norton J, Morris JC, Reed LA, Trojanowski J, Basun H, Lannfelt L, Neystat M, Fahn S, Dark F, Tannenberg T, Dodd PR, Hayward N, Kwok JB, Schofield PR, Andreadis A, Snowden J, Craufurd D, Neary D, Owen F, Oostra BA, Hardy J, Goate A, van Swieten J, Mann D, Lynch T, Heutink P (1998) Association of missense and 5\u2019-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 393:702\u2013705","journal-title":"Nature"},{"key":"839_CR44","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1111\/j.1440-1789.2005.00598.x","volume":"25","author":"K Ishihara","year":"2005","unstructured":"Ishihara K, Araki S, Ihori N, Shiota J, Kawamura M, Yoshida M, Hashizume Y, Nakano I (2005) Argyrophilic grain disease presenting with frontotemporal dementia: a neuropsychological and pathological study of an autopsied case with presenile onset. Neuropathology 25:165\u2013170","journal-title":"Neuropathology"},{"key":"839_CR45","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1111\/j.1440-1789.2009.01003.x","volume":"29","author":"Y Iwasaki","year":"2009","unstructured":"Iwasaki Y, Ito M, Mori K, Deguchi A, Nagaoka M, Yoshida M, Hashizume Y (2009) An autopsy case of diffuse neurofibrillary tangles with calcification: early stage pathologic findings. Neuropathology 29:697\u2013703","journal-title":"Neuropathology"},{"key":"839_CR46","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1006\/neur.1996.0046","volume":"5","author":"M Jackson","year":"1996","unstructured":"Jackson M, Lennox G, Lowe J (1996) Motor neurone disease-inclusion dementia. Neurodegeneration 5:339\u2013350","journal-title":"Neurodegeneration"},{"key":"839_CR47","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1001\/archneur.62.6.925","volume":"62","author":"JK Johnson","year":"2005","unstructured":"Johnson JK, Diehl J, Mendez MF, Neuhaus J, Shapira JS, Forman M, Chute DJ, Roberson ED, Pace-Savitsky C, Neumann M, Chow TW, Rosen HJ, Forstl H, Kurz A, Miller BL (2005) Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol 62:925\u2013930","journal-title":"Arch Neurol"},{"key":"839_CR48","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1002\/ana.21426","volume":"64","author":"KA Josephs","year":"2008","unstructured":"Josephs KA (2008) Frontotemporal dementia and related disorders: deciphering the enigma. Ann Neurol 64:4\u201314","journal-title":"Ann Neurol"},{"key":"839_CR49","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1097\/nen.0b013e31803020cf","volume":"66","author":"KA Josephs","year":"2007","unstructured":"Josephs KA, Ahmed Z, Katsuse O, Parisi JF, Boeve BF, Knopman DS, Petersen RC, Davies P, Duara R, Graff-Radford NR, Uitti RJ, Rademakers R, Adamson J, Baker M, Hutton ML, Dickson DW (2007) Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. J Neuropathol Exp Neurol 66:142\u2013151","journal-title":"J Neuropathol Exp Neurol"},{"key":"839_CR50","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1097\/WCO.0b013e3283168ddd","volume":"21","author":"KA Josephs","year":"2008","unstructured":"Josephs KA, Duffy JR (2008) Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy. Curr Opin Neurol 21:688\u2013692","journal-title":"Curr Opin Neurol"},{"key":"839_CR51","doi-asserted-by":"crossref","first-page":"1385","DOI":"10.1093\/brain\/awl078","volume":"129","author":"KA Josephs","year":"2006","unstructured":"Josephs KA, Duffy JR, Strand EA, Whitwell JL, Layton KF, Parisi JE, Hauser MF, Witte RJ, Boeve BF, Knopman DS, Dickson DW, Jack CR Jr, Petersen RC (2006) Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 129:1385\u20131398","journal-title":"Brain"},{"key":"839_CR52","doi-asserted-by":"crossref","first-page":"2291","DOI":"10.1093\/brain\/awg231","volume":"126","author":"KA Josephs","year":"2003","unstructured":"Josephs KA, Holton JL, Rossor MN, Braendgaard H, Ozawa T, Fox NC, Petersen RC, Pearl GS, Ganguly M, Rosa P, Laursen H, Parisi JE, Waldemar G, Quinn NP, Dickson DW, Revesz T (2003) Neurofilament inclusion body disease: a new proteinopathy? Brain 126:2291\u20132303","journal-title":"Brain"},{"key":"839_CR53","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1097\/01.jnen.0000218446.38158.61","volume":"65","author":"KA Josephs","year":"2006","unstructured":"Josephs KA, Katsuse O, Beccano-Kelly DA, Lin WL, Uitti RJ, Fujino Y, Boeve BF, Hutton ML, Baker MC, Dickson DW (2006) Atypical progressive supranuclear palsy with corticospinal tract degeneration. J Neuropathol Exp Neurol 65:396\u2013405","journal-title":"J Neuropathol Exp Neurol"},{"key":"839_CR54","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1007\/s00401-008-0397-8","volume":"116","author":"KA Josephs","year":"2008","unstructured":"Josephs KA, Lin WL, Ahmed Z, Stroh DA, Graff-Radford NR, Dickson DW (2008) Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions. Acta Neuropathol 116:159\u2013167","journal-title":"Acta Neuropathol"},{"key":"839_CR55","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1001\/archneur.63.4.506","volume":"63","author":"KA Josephs","year":"2006","unstructured":"Josephs KA, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Dickson DW (2006) Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease. Arch Neurol 63:506\u2013512","journal-title":"Arch Neurol"},{"key":"839_CR56","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1212\/01.wnl.0000191307.69661.c3","volume":"66","author":"KA Josephs","year":"2006","unstructured":"Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR, Parisi JE, Dickson DW (2006) Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 66:41\u201348","journal-title":"Neurology"},{"key":"839_CR57","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s00401-009-0547-7","volume":"118","author":"KA Josephs","year":"2009","unstructured":"Josephs KA, Stroh A, Dugger B, Dickson DW (2009) Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes. Acta Neuropathol 118:349\u2013358","journal-title":"Acta Neuropathol"},{"key":"839_CR58","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1007\/s00401-004-0974-4","volume":"109","author":"KA Josephs","year":"2005","unstructured":"Josephs KA, Uchikado H, McComb RD, Bashir R, Wszolek Z, Swanson J, Matsumoto J, Shaw G, Dickson DW (2005) Extending the clinicopathological spectrum of neurofilament inclusion disease. Acta Neuropathol 109:427\u2013432","journal-title":"Acta Neuropathol"},{"key":"839_CR59","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/j.neurobiolaging.2006.09.019","volume":"29","author":"KA Josephs","year":"2008","unstructured":"Josephs KA, Whitwell JL, Dickson DW, Boeve BF, Knopman DS, Petersen RC, Parisi JE, Jack CR Jr (2008) Voxel-based morphometry in autopsy proven PSP and CBD. Neurobiol Aging 29:280\u2013289","journal-title":"Neurobiol Aging"},{"key":"839_CR60","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1002\/mds.23471","volume":"26","author":"KA Josephs","year":"2011","unstructured":"Josephs KA, Whitwell JL, Eggers SD, Senjem ML, Jack CR Jr (2011) Gray matter correlates of behavioral severity in progressive supranuclear palsy. Mov Disord 26:493\u2013498","journal-title":"Mov Disord"},{"key":"839_CR61","doi-asserted-by":"crossref","first-page":"1859","DOI":"10.1016\/j.neurobiolaging.2007.04.027","volume":"29","author":"KA Josephs","year":"2008","unstructured":"Josephs KA, Whitwell JL, Jack CR Jr (2008) Anatomic correlates of stereotypies in frontotemporal lobar degeneration. Neurobiol Aging 29:1859\u20131863","journal-title":"Neurobiol Aging"},{"key":"839_CR62","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1212\/WNL.0b013e3181bf9945","volume":"73","author":"KA Josephs","year":"2009","unstructured":"Josephs KA, Whitwell JL, Knopman DS, Boeve BF, Vemuri P, Senjem ML, Parisi JE, Ivnik RJ, Dickson DW, Petersen RC, Jack CR Jr (2009) Two distinct subtypes of right temporal variant frontotemporal dementia. Neurology 73:1443\u20131450","journal-title":"Neurology"},{"key":"839_CR63","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1111\/j.1468-1331.2010.02975.x","volume":"17","author":"KA Josephs","year":"2010","unstructured":"Josephs KA, Whitwell JL, Parisi JE, Petersen RC, Boeve BF, Jack CR Jr, Dickson DW (2010) Caudate atrophy on MRI is a characteristic feature of FTLD-FUS. Eur J Neurol 17:969\u2013975","journal-title":"Eur J Neurol"},{"key":"839_CR64","doi-asserted-by":"crossref","first-page":"1628","DOI":"10.1212\/01.wnl.0000334756.18558.92","volume":"71","author":"KA Josephs","year":"2008","unstructured":"Josephs KA, Whitwell JL, Vemuri P, Senjem ML, Boeve BF, Knopman DS, Smith GE, Ivnik RJ, Petersen RC, Jack CR Jr (2008) The anatomic correlate of prosopagnosia in semantic dementia. Neurology 71:1628\u20131633","journal-title":"Neurology"},{"key":"839_CR65","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1038\/ng.132","volume":"40","author":"E Kabashi","year":"2008","unstructured":"Kabashi E, Valdmanis PN, Dion P, Spiegelman D, McConkey BJ, Van de Velde C, Bouchard JP, Lacomblez L, Pochigaeva K, Salachas F, Pradat PF, Camu W, Meininger V, Dupre N, Rouleau GA (2008) TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat Genet 40:572\u2013574","journal-title":"Nat Genet"},{"key":"839_CR66","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.neurobiolaging.2007.08.022","volume":"30","author":"BJ Kelley","year":"2009","unstructured":"Kelley BJ, Haidar W, Boeve BF, Baker M, Graff-Radford NR, Krefft T, Frank AR, Jack CR Jr, Shiung M, Knopman DS, Josephs KA, Parashos SA, Rademakers R, Hutton M, Pickering-Brown S, Adamson J, Kuntz KM, Dickson DW, Parisi JE, Smith GE, Ivnik RJ, Petersen RC (2009) Prominent phenotypic variability associated with mutations in progranulin. Neurobiol Aging 30:739\u2013751","journal-title":"Neurobiol Aging"},{"key":"839_CR67","doi-asserted-by":"crossref","first-page":"1996","DOI":"10.1093\/brain\/awh598","volume":"128","author":"A Kertesz","year":"2005","unstructured":"Kertesz A, McMonagle P, Blair M, Davidson W, Munoz DG (2005) The evolution and pathology of frontotemporal dementia. Brain 128:1996\u20132005","journal-title":"Brain"},{"key":"839_CR68","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1002\/ana.20700","volume":"59","author":"JA Knibb","year":"2006","unstructured":"Knibb JA, Xuereb JH, Patterson K, Hodges JR (2006) Clinical and pathological characterization of progressive aphasia. Ann Neurol 59:156\u2013165","journal-title":"Ann Neurol"},{"key":"839_CR69","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1002\/ana.20425","volume":"57","author":"DS Knopman","year":"2005","unstructured":"Knopman DS, Boeve BF, Parisi JE, Dickson DW, Smith GE, Ivnik RJ, Josephs KA, Petersen RC (2005) Antemortem diagnosis of frontotemporal lobar degeneration. Ann Neurol 57:480\u2013488","journal-title":"Ann Neurol"},{"key":"839_CR70","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1212\/01.WNL.0000106827.39764.7E","volume":"62","author":"DS Knopman","year":"2004","unstructured":"Knopman DS, Petersen RC, Edland SD, Cha RH, Rocca WA (2004) The incidence of frontotemporal lobar degeneration in Rochester, Minnesota, 1990 through 1994. Neurology 62:506\u2013508","journal-title":"Neurology"},{"key":"839_CR71","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1136\/jnnp.57.5.594","volume":"57","author":"K Kosaka","year":"1994","unstructured":"Kosaka K (1994) Diffuse neurofibrillary tangles with calcification: a new presenile dementia. J Neurol Neurosurg Psychiatry 57:594\u2013596","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"839_CR72","first-page":"18","volume":"75","author":"K Kosaka","year":"1973","unstructured":"Kosaka K, Shibayama H, Kobayashi H, Hoshino T, Iwase S (1973) An autopsy case of unclassifiable presenile dementia. Seishin Shinkeigaku Zasshi 75:18\u201334","journal-title":"Seishin Shinkeigaku Zasshi"},{"key":"839_CR73","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1097\/NEN.0b013e318187a80f","volume":"67","author":"GG Kovacs","year":"2008","unstructured":"Kovacs GG, Majtenyi K, Spina S, Murrell JR, Gelpi E, Hoftberger R, Fraser G, Crowther RA, Goedert M, Budka H, Ghetti B (2008) White matter tauopathy with globular glial inclusions: a distinct sporadic frontotemporal lobar degeneration. J Neuropathol Exp Neurol 67:963\u2013975","journal-title":"J Neuropathol Exp Neurol"},{"key":"839_CR74","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1007\/BF00307636","volume":"80","author":"H Kusaka","year":"1990","unstructured":"Kusaka H, Matsumoto S, Imai T (1990) An adult-onset case of sporadic motor neuron disease with basophilic inclusions. Acta Neuropathol 80:660\u2013665","journal-title":"Acta Neuropathol"},{"key":"839_CR75","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1126\/science.1166066","volume":"323","author":"TJ Kwiatkowski Jr","year":"2009","unstructured":"Kwiatkowski TJ Jr, Bosco DA, Leclerc AL, Tamrazian E, Vanderburg CR, Russ C, Davis A, Gilchrist J, Kasarskis EJ, Munsat T, Valdmanis P, Rouleau GA, Hosler BA, Cortelli P, de Jong PJ, Yoshinaga Y, Haines JL, Pericak-Vance MA, Yan J, Ticozzi N, Siddique T, McKenna-Yasek D, Sapp PC, Horvitz HR, Landers JE, Brown RH Jr (2009) Mutations in the FUS\/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science 323:1205\u20131208","journal-title":"Science"},{"key":"839_CR76","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1001\/archneurol.2010.146","volume":"67","author":"P Lillo","year":"2010","unstructured":"Lillo P, Garcin B, Hornberger M, Bak TH, Hodges JR (2010) Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis. Arch Neurol 67:826\u2013830","journal-title":"Arch Neurol"},{"key":"839_CR77","doi-asserted-by":"crossref","first-page":"2045","DOI":"10.1093\/brain\/awq123","volume":"133","author":"H Ling","year":"2010","unstructured":"Ling H, O\u2019Sullivan SS, Holton JL, Revesz T, Massey LA, Williams DR, Paviour DC, Lees AJ (2010) Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain 133:2045\u20132057","journal-title":"Brain"},{"key":"839_CR78","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1212\/WNL.47.1.1","volume":"47","author":"I Litvan","year":"1996","unstructured":"Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, Goetz CG, Golbe LI, Grafman J, Growdon JH, Hallett M, Jankovic J, Quinn NP, Tolosa E, Zee DS (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47:1\u20139","journal-title":"Neurology"},{"key":"839_CR79","first-page":"342","volume-title":"Neurodegeneration: the molecular pathology of dementia and movement disorders","author":"JS Lowe","year":"2003","unstructured":"Lowe JS, Rossor MN (2003) Frontotemporal lobar degeneration. In: Dickson DW (ed) Neurodegeneration: the molecular pathology of dementia and movement disorders. ISN Neuropath Press, Basel, pp 342\u2013348"},{"key":"839_CR80","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1007\/s00401-006-0138-9","volume":"112","author":"IR Mackenzie","year":"2006","unstructured":"Mackenzie IR, Baborie A, Pickering-Brown S, Du Plessis D, Jaros E, Perry RH, Neary D, Snowden JS, Mann DM (2006) Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype. Acta Neuropathol 112:539\u2013549","journal-title":"Acta Neuropathol"},{"key":"839_CR81","doi-asserted-by":"crossref","first-page":"3081","DOI":"10.1093\/brain\/awl271","volume":"129","author":"IR Mackenzie","year":"2006","unstructured":"Mackenzie IR, Baker M, Pickering-Brown S, Hsiung GY, Lindholm C, Dwosh E, Gass J, Cannon A, Rademakers R, Hutton M, Feldman HH (2006) The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene. Brain 129:3081\u20133090","journal-title":"Brain"},{"key":"839_CR82","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1093\/brain\/awn061","volume":"131","author":"IR Mackenzie","year":"2008","unstructured":"Mackenzie IR, Foti D, Woulfe J, Hurwitz TA (2008) Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions. Brain 131:1282\u20131293","journal-title":"Brain"},{"key":"839_CR83","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1007\/s00401-010-0764-0","volume":"121","author":"IR Mackenzie","year":"2011","unstructured":"Mackenzie IR, Munoz DG, Kusaka H, Yokota O, Ishihara K, Roeber S, Kretzschmar HA, Cairns NJ, Neumann M (2011) Distinct pathological subtypes of FTLD-FUS. Acta Neuropathol 121:207\u2013218","journal-title":"Acta Neuropathol"},{"key":"839_CR84","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00401-009-0612-2","volume":"119","author":"IR Mackenzie","year":"2010","unstructured":"Mackenzie IR, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J, Kovacs GG, Ghetti B, Halliday G, Holm IE, Ince PG, Kamphorst W, Revesz T, Rozemuller AJ, Kumar-Singh S, Akiyama H, Baborie A, Spina S, Dickson DW, Trojanowski JQ, Mann DM (2010) Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol 119:1\u20134","journal-title":"Acta Neuropathol"},{"key":"839_CR85","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1007\/s00401-008-0460-5","volume":"117","author":"IR Mackenzie","year":"2009","unstructured":"Mackenzie IR, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J, Kovacs GG, Ghetti B, Halliday G, Holm IE, Ince PG, Kamphorst W, Revesz T, Rozemuller AJ, Kumar-Singh S, Akiyama H, Baborie A, Spina S, Dickson DW, Trojanowski JQ, Mann DM (2009) Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol 117:15\u201318","journal-title":"Acta Neuropathol"},{"key":"839_CR86","doi-asserted-by":"crossref","first-page":"3115","DOI":"10.1093\/brain\/awl276","volume":"129","author":"M Masellis","year":"2006","unstructured":"Masellis M, Momeni P, Meschino W, Heffner R Jr, Elder J, Sato C, Liang Y, St George-Hyslop P, Hardy J, Bilbao J, Black S, Rogaeva E (2006) Novel splicing mutation in the progranulin gene causing familial corticobasal syndrome. Brain 129:3115\u20133123","journal-title":"Brain"},{"key":"839_CR87","doi-asserted-by":"crossref","first-page":"1803","DOI":"10.1001\/archneur.58.11.1803","volume":"58","author":"GM McKhann","year":"2001","unstructured":"McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ (2001) Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick\u2019s Disease. Arch Neurol 58:1803\u20131809","journal-title":"Arch Neurol"},{"key":"839_CR88","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1001\/archneur.64.1.43","volume":"64","author":"M Mesulam","year":"2007","unstructured":"Mesulam M, Johnson N, Krefft TA, Gass JM, Cannon AD, Adamson JL, Bigio EH, Weintraub S, Dickson DW, Hutton ML, Graff-Radford NR (2007) Progranulin mutations in primary progressive aphasia: the PPA1 and PPA3 families. Arch Neurol 64:43\u201347","journal-title":"Arch Neurol"},{"key":"839_CR89","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1002\/ana.21388","volume":"63","author":"M Mesulam","year":"2008","unstructured":"Mesulam M, Wicklund A, Johnson N, Rogalski E, Leger GC, Rademaker A, Weintraub S, Bigio EH (2008) Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 63:709\u2013719","journal-title":"Ann Neurol"},{"key":"839_CR90","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1001\/archneurol.2007.37","volume":"65","author":"L Molina-Porcel","year":"2008","unstructured":"Molina-Porcel L, Llado A, Rey MJ, Molinuevo JL, Martinez-Lage M, Esteve FX, Ferrer I, Tolosa E, Blesa R (2008) Clinical and pathological heterogeneity of neuronal intermediate filament inclusion disease. Arch Neurol 65:272\u2013275","journal-title":"Arch Neurol"},{"key":"839_CR91","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1002\/1531-8249(200001)47:1<36::AID-ANA8>3.0.CO;2-L","volume":"47","author":"CJ Mummery","year":"2000","unstructured":"Mummery CJ, Patterson K, Price CJ, Ashburner J, Frackowiak RS, Hodges JR (2000) A voxel-based morphometry study of semantic dementia: relationship between temporal lobe atrophy and semantic memory. Ann Neurol 47:36\u201345","journal-title":"Ann Neurol"},{"key":"839_CR92","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1007\/s00401-009-0598-9","volume":"118","author":"DG Munoz","year":"2009","unstructured":"Munoz DG, Neumann M, Kusaka H, Yokota O, Ishihara K, Terada S, Kuroda S, Mackenzie IR (2009) FUS pathology in basophilic inclusion body disease. Acta Neuropathol 118:617\u2013627","journal-title":"Acta Neuropathol"},{"key":"839_CR93","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1177\/1533317507312270","volume":"22","author":"S Nanda","year":"2007","unstructured":"Nanda S, Bhatt SP, Pamula J, Woodruff WW, Fowler M, Miller D (2007) Diffuse neurofibrillary tangles with calcification (DNTC): Kosaka-Shibayama disease in America. Am J Alzheimers Dis Other Dement 22:535\u2013537","journal-title":"Am J Alzheimers Dis Other Dement"},{"key":"839_CR94","doi-asserted-by":"crossref","first-page":"1546","DOI":"10.1212\/WNL.51.6.1546","volume":"51","author":"D Neary","year":"1998","unstructured":"Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546\u20131554","journal-title":"Neurology"},{"key":"839_CR95","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1097\/nen.0b013e31803020b9","volume":"66","author":"M Neumann","year":"2007","unstructured":"Neumann M, Mackenzie IR, Cairns NJ, Boyer PJ, Markesbery WR, Smith CD, Taylor JP, Kretzschmar HA, Kimonis VE, Forman MS (2007) TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol 66:152\u2013157","journal-title":"J Neuropathol Exp Neurol"},{"key":"839_CR96","doi-asserted-by":"crossref","first-page":"2922","DOI":"10.1093\/brain\/awp214","volume":"132","author":"M Neumann","year":"2009","unstructured":"Neumann M, Rademakers R, Roeber S, Baker M, Kretzschmar HA, Mackenzie IR (2009) A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain 132:2922\u20132931","journal-title":"Brain"},{"key":"839_CR97","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1007\/s00401-009-0581-5","volume":"118","author":"M Neumann","year":"2009","unstructured":"Neumann M, Roeber S, Kretzschmar HA, Rademakers R, Baker M, Mackenzie IR (2009) Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol 118:605\u2013616","journal-title":"Acta Neuropathol"},{"key":"839_CR98","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1126\/science.1134108","volume":"314","author":"M Neumann","year":"2006","unstructured":"Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT, Bruce J, Schuck T, Grossman M, Clark CM, McCluskey LF, Miller BL, Masliah E, Mackenzie IR, Feldman H, Feiden W, Kretzschmar HA, Trojanowski JQ, Lee VM (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130\u2013133","journal-title":"Science"},{"key":"839_CR99","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1080\/13554790500263529","volume":"11","author":"J Ogar","year":"2005","unstructured":"Ogar J, Slama H, Dronkers N, Amici S, Gorno-Tempini ML (2005) Apraxia of speech: an overview. Neurocase 11:427\u2013432","journal-title":"Neurocase"},{"key":"839_CR100","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1093\/brain\/awf069","volume":"125","author":"SM Pickering-Brown","year":"2002","unstructured":"Pickering-Brown SM, Richardson AM, Snowden JS, McDonagh AM, Burns A, Braude W, Baker M, Liu WK, Yen SH, Hardy J, Hutton M, Davies Y, Allsop D, Craufurd D, Neary D, Mann DM (2002) Inherited frontotemporal dementia in nine British families associated with intronic mutations in the tau gene. Brain 125:732\u2013751","journal-title":"Brain"},{"key":"839_CR101","doi-asserted-by":"crossref","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","journal-title":"Brain"},{"key":"839_CR102","doi-asserted-by":"crossref","first-page":"1615","DOI":"10.1212\/WNL.58.11.1615","volume":"58","author":"E Ratnavalli","year":"2002","unstructured":"Ratnavalli E, Brayne C, Dawson K, Hodges JR (2002) The prevalence of frontotemporal dementia. Neurology 58:1615\u20131621","journal-title":"Neurology"},{"key":"839_CR103","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/s00401-008-0395-x","volume":"116","author":"S Roeber","year":"2008","unstructured":"Roeber S, Mackenzie IR, Kretzschmar HA, Neumann M (2008) TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD. Acta Neuropathol 116:147\u2013157","journal-title":"Acta Neuropathol"},{"key":"839_CR104","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1016\/j.neuroimage.2009.12.088","volume":"53","author":"JD Rohrer","year":"2010","unstructured":"Rohrer JD, Ridgway GR, Modat M, Ourselin S, Mead S, Fox NC, Rossor MN, Warren JD (2010) Distinct profiles of brain atrophy in frontotemporal lobar degeneration caused by progranulin and tau mutations. Neuroimage 53:1070\u20131076","journal-title":"Neuroimage"},{"key":"839_CR105","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.2353\/ajpath.2006.060438","volume":"169","author":"DM Sampathu","year":"2006","unstructured":"Sampathu DM, Neumann M, Kwong LK, Chou TT, Micsenyi M, Truax A, Bruce J, Grossman M, Trojanowski JQ, Lee VM (2006) Pathological heterogeneity of frontotemporal lobar degeneration with ubiquitin-positive inclusions delineated by ubiquitin immunohistochemistry and novel monoclonal antibodies. Am J Pathol 169:1343\u20131352","journal-title":"Am J Pathol"},{"key":"839_CR106","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1007\/s00415-009-5404-z","volume":"257","author":"H Seelaar","year":"2010","unstructured":"Seelaar H, Klijnsma KY, de Koning I, van der Lugt A, Chiu WZ, Azmani A, Rozemuller AJ, van Swieten JC (2010) Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration. J Neurol 257:747\u2013753","journal-title":"J Neurol"},{"key":"839_CR107","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1212\/01.WNL.0000158425.46019.5C","volume":"64","author":"WW Seeley","year":"2005","unstructured":"Seeley WW, Bauer AM, Miller BL, Gorno-Tempini ML, Kramer JH, Weiner M, Rosen HJ (2005) The natural history of temporal variant frontotemporal dementia. Neurology 64:1384\u20131390","journal-title":"Neurology"},{"key":"839_CR108","doi-asserted-by":"crossref","first-page":"1593","DOI":"10.1002\/mds.22558","volume":"24","author":"BP Shelley","year":"2009","unstructured":"Shelley BP, Hodges JR, Kipps CM, Xuereb JH, Bak TH (2009) Is the pathology of corticobasal syndrome predictable in life? Mov Disord 24:1593\u20131599","journal-title":"Mov Disord"},{"key":"839_CR109","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1038\/ng1609","volume":"37","author":"G Skibinski","year":"2005","unstructured":"Skibinski G, Parkinson NJ, Brown JM, Chakrabarti L, Lloyd SL, Hummerich H, Nielsen JE, Hodges JR, Spillantini MG, Thusgaard T, Brandner S, Brun A, Rossor MN, Gade A, Johannsen P, Sorensen SA, Gydesen S, Fisher EM, Collinge J (2005) Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nat Genet 37:806\u2013808","journal-title":"Nat Genet"},{"key":"839_CR110","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1155\/1989\/124043","volume":"2","author":"J Snowden","year":"1989","unstructured":"Snowden J, Goulding P, Neary D (1989) Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 2:167\u2013182","journal-title":"Behav Neurol"},{"key":"839_CR111","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/s00401-007-0236-3","volume":"114","author":"J Snowden","year":"2007","unstructured":"Snowden J, Neary D, Mann D (2007) Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 114:31\u201338","journal-title":"Acta Neuropathol"},{"key":"839_CR112","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1136\/jnnp.70.3.323","volume":"70","author":"JS Snowden","year":"2001","unstructured":"Snowden JS, Bathgate D, Varma A, Blackshaw A, Gibbons ZC, Neary D (2001) Distinct behavioural profiles in frontotemporal dementia and semantic dementia. J Neurol Neurosurg Psychiatry 70:323\u2013332","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"839_CR113","doi-asserted-by":"crossref","unstructured":"Snowden JS, Hu Q, Rollinson S, Halliwell N, Robinson A, Davidson YS, Momeni P, Baborie A, Griffiths TD, Jaros E, Perry RH, Richardson A, Pickering-Brown SM, Neary D, Mann DM (2011) The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene. Acta neuropathologica. doi: 10.1007\/s00401-011-0816-0","DOI":"10.1007\/s00401-011-0816-0"},{"key":"839_CR114","doi-asserted-by":"crossref","first-page":"3091","DOI":"10.1093\/brain\/awl267","volume":"129","author":"JS Snowden","year":"2006","unstructured":"Snowden JS, Pickering-Brown SM, Mackenzie IR, Richardson AM, Varma A, Neary D, Mann DM (2006) Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia. Brain 129:3091\u20133102","journal-title":"Brain"},{"key":"839_CR115","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1111\/j.1750-3639.1998.tb00162.x","volume":"8","author":"MG Spillantini","year":"1998","unstructured":"Spillantini MG, Bird TD, Ghetti B (1998) Frontotemporal dementia and Parkinsonism linked to chromosome 17: a new group of tauopathies. Brain Pathol 8:387\u2013402","journal-title":"Brain Pathol"},{"key":"839_CR116","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1097\/nen.0b013e3181567873","volume":"66","author":"S Spina","year":"2007","unstructured":"Spina S, Murrell JR, Huey ED, Wassermann EM, Pietrini P, Grafman J, Ghetti B (2007) Corticobasal syndrome associated with the A9D progranulin mutation. J Neuropathol Exp Neurol 66:892\u2013900","journal-title":"J Neuropathol Exp Neurol"},{"key":"839_CR117","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1126\/science.1154584","volume":"319","author":"J Sreedharan","year":"2008","unstructured":"Sreedharan J, Blair IP, Tripathi VB, Hu X, Vance C, Rogelj B, Ackerley S, Durnall JC, Williams KL, Buratti E, Baralle F, de Belleroche J, Mitchell JD, Leigh PN, Al-Chalabi A, Miller CC, Nicholson G, Shaw CE (2008) TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 319:1668\u20131672","journal-title":"Science"},{"key":"839_CR118","doi-asserted-by":"crossref","first-page":"1196","DOI":"10.1212\/01.WNL.0000091868.28557.B8","volume":"61","author":"SA Thompson","year":"2003","unstructured":"Thompson SA, Patterson K, Hodges JR (2003) Left\/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications. Neurology 61:1196\u20131203","journal-title":"Neurology"},{"key":"839_CR119","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1093\/jnen\/61.6.547","volume":"61","author":"T Togo","year":"2002","unstructured":"Togo T, Sahara N, Yen SH, Cookson N, Ishizawa T, Hutton M, de Silva R, Lees A, Dickson DW (2002) Argyrophilic grain disease is a sporadic 4-repeat tauopathy. J Neuropathol Exp Neurol 61:547\u2013556","journal-title":"J Neuropathol Exp Neurol"},{"key":"839_CR120","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1007\/s00401-004-0947-7","volume":"109","author":"T Uchihara","year":"2005","unstructured":"Uchihara T, Shibuya K, Nakamura A, Yagishita S (2005) Silver stains distinguish tau-positive structures in corticobasal degeneration\/progressive supranuclear palsy and in Alzheimer\u2019s disease\u2013comparison between Gallyas and Campbell-Switzer methods. Acta Neuropathol 109:299\u2013305","journal-title":"Acta Neuropathol"},{"key":"839_CR121","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1007\/s00401-005-0988-6","volume":"109","author":"T Uchihara","year":"2005","unstructured":"Uchihara T, Tsuchiya K, Nakamura A, Akiyama H (2005) Silver staining profiles distinguish Pick bodies from neurofibrillary tangles of Alzheimer type: comparison between Gallyas and Campbell-Switzer methods. Acta Neuropathol 109:483\u2013489","journal-title":"Acta Neuropathol"},{"key":"839_CR122","doi-asserted-by":"crossref","first-page":"1658","DOI":"10.1212\/01.WNL.0000160328.17975.9C","volume":"64","author":"H Uchikado","year":"2005","unstructured":"Uchikado H, Shaw G, Wang DS, Dickson DW (2005) Screening for neurofilament inclusion disease using alpha-internexin immunohistochemistry. Neurology 64:1658\u20131659","journal-title":"Neurology"},{"key":"839_CR123","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/s00401-010-0698-6","volume":"120","author":"H Urwin","year":"2010","unstructured":"Urwin H, Josephs KA, Rohrer JD, Mackenzie IR, Neumann M, Authier A, Seelaar H, Van Swieten JC, Brown JM, Johannsen P, Nielsen JE, Holm IE, Dickson DW, Rademakers R, Graff-Radford NR, Parisi JE, Petersen RC, Hatanpaa KJ, White CL 3rd, Weiner MF, Geser F, Van Deerlin VM, Trojanowski JQ, Miller BL, Seeley WW, van der Zee J, Kumar-Singh S, Engelborghs S, De Deyn PP, Van Broeckhoven C, Bigio EH, Deng HX, Halliday GM, Kril JJ, Munoz DG, Mann DM, Pickering-Brown SM, Doodeman V, Adamson G, Ghazi-Noori S, Fisher EM, Holton JL, Revesz T, Rossor MN, Collinge J, Mead S, Isaacs AM (2010) FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol 120:33\u201341","journal-title":"Acta Neuropathol"},{"key":"839_CR124","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1093\/hmg\/ddm309","volume":"17","author":"J Zee van der","year":"2008","unstructured":"van der Zee J, Urwin H, Engelborghs S, Bruyland M, Vandenberghe R, Dermaut B, De Pooter T, Peeters K, Santens P, De Deyn PP, Fisher EM, Collinge J, Isaacs AM, Van Broeckhoven C (2008) CHMP2B C-truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro. Hum Mol Genet 17:313\u2013322","journal-title":"Hum Mol Genet"},{"key":"839_CR125","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/j.1750-3639.2007.00052.x","volume":"17","author":"J Swieten van","year":"2007","unstructured":"van Swieten J, Spillantini MG (2007) Hereditary frontotemporal dementia caused by Tau gene mutations. Brain Pathol 17:63\u201373","journal-title":"Brain Pathol"},{"key":"839_CR126","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1126\/science.1165942","volume":"323","author":"C Vance","year":"2009","unstructured":"Vance C, Rogelj B, Hortobagyi T, De Vos KJ, Nishimura AL, Sreedharan J, Hu X, Smith B, Ruddy D, Wright P, Ganesalingam J, Williams KL, Tripathi V, Al-Saraj S, Al-Chalabi A, Leigh PN, Blair IP, Nicholson G, de Belleroche J, Gallo JM, Miller CC, Shaw CE (2009) Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 323:1208\u20131211","journal-title":"Science"},{"key":"839_CR127","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1080\/14640747508400525","volume":"27","author":"EK Warrington","year":"1975","unstructured":"Warrington EK (1975) Selective impairment of semantic memory. Q J Exp Psychol 27:635\u2013657","journal-title":"Q J Exp Psychol"},{"key":"839_CR128","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/ng1332","volume":"36","author":"GD Watts","year":"2004","unstructured":"Watts GD, Wymer J, Kovach MJ, Mehta SG, Mumm S, Darvish D, Pestronk A, Whyte MP, Kimonis VE (2004) Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nat Genet 36:377\u2013381","journal-title":"Nat Genet"},{"key":"839_CR129","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1212\/WNL.0b013e3181feb2e8","volume":"75","author":"JL Whitwell","year":"2010","unstructured":"Whitwell JL, Jack CR Jr, Boeve BF, Parisi JE, Ahlskog JE, Drubach DA, Senjem ML, Knopman DS, Petersen RC, Dickson DW, Josephs KA (2010) Imaging correlates of pathology in corticobasal syndrome. Neurology 75:1879\u20131887","journal-title":"Neurology"},{"key":"839_CR130","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1212\/01.wnl.0000343851.46573.67","volume":"72","author":"JL Whitwell","year":"2009","unstructured":"Whitwell JL, Jack CR Jr, Boeve BF, Senjem ML, Baker M, Rademakers R, Ivnik RJ, Knopman DS, Wszolek ZK, Petersen RC, Josephs KA (2009) Voxel-based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN. Neurology 72:813\u2013820","journal-title":"Neurology"},{"key":"839_CR131","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1093\/brain\/awh488","volume":"128","author":"DR Williams","year":"2005","unstructured":"Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ (2005) Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson\u2019s syndrome and PSP-parkinsonism. Brain 128:1247\u20131258","journal-title":"Brain"},{"key":"839_CR132","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1212\/WNL.0b013e3181f07e0c","volume":"75","author":"J Yan","year":"2010","unstructured":"Yan J, Deng HX, Siddique N, Fecto F, Chen W, Yang Y, Liu E, Donkervoort S, Zheng JG, Shi Y, Ahmeti KB, Brooks B, Engel WK, Siddique T (2010) Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS\/dementia. Neurology 75:807\u2013814","journal-title":"Neurology"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.springerlink.com\/index\/pdf\/10.1007\/s00401-011-0839-6","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,23]],"date-time":"2021-11-23T21:08:11Z","timestamp":1637701691000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00401-011-0839-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,5,26]]},"references-count":132,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2011,8]]}},"alternative-id":["839"],"URL":"https:\/\/doi.org\/10.1007\/s00401-011-0839-6","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,5,26]]}}}