{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T17:35:42Z","timestamp":1772818542917,"version":"3.50.1"},"reference-count":43,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2010,2,7]],"date-time":"2010-02-07T00:00:00Z","timestamp":1265500800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2010,7]]},"DOI":"10.1007\/s00401-010-0649-2","type":"journal-article","created":{"date-parts":[[2010,2,6]],"date-time":"2010-02-06T02:12:55Z","timestamp":1265422375000},"page":"21-32","source":"Crossref","is-referenced-by-count":50,"title":["Sporadic four-repeat tauopathy with frontotemporal lobar degeneration, Parkinsonism, and motor neuron disease: a distinct clinicopathological and biochemical disease entity"],"prefix":"10.1007","volume":"120","author":[{"given":"Yong-Juan","family":"Fu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasushi","family":"Nishihira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigetoshi","family":"Kuroda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuko","family":"Toyoshima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomohiko","family":"Ishihara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makoto","family":"Shinozaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akinori","family":"Miyashita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yue-Shan","family":"Piao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chun-Feng","family":"Tan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Tani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryoko","family":"Koike","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keisuke","family":"Iwanaga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mitsuhiro","family":"Tsujihata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Osamu","family":"Onodera","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryozo","family":"Kuwano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masatoyo","family":"Nishizawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akiyoshi","family":"Kakita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takeshi","family":"Ikeuchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hitoshi","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2010,2,7]]},"reference":[{"key":"649_CR1","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1007\/s004010000283","volume":"101","author":"T Arai","year":"2001","unstructured":"Arai T, Ikeda K, Akiyama H et al (2001) Distinct isoforms of tau aggregated in neurons and glial cells in brains of patients with Pick\u2019s disease, corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 101:167\u2013173","journal-title":"Acta Neuropathol"},{"key":"649_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 et al (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":"649_CR3","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 S-H et al (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":"649_CR4","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":"649_CR5","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 et al (1999) Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 53:795\u2013800","journal-title":"Neurology"},{"key":"649_CR6","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1111\/j.1750-3639.1999.tb00550.x","volume":"9","author":"L Bu\u00e9e","year":"1999","unstructured":"Bu\u00e9e L, Delacourte A (1999) Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick\u2019s disease. Brain Pathol 9:681\u2013693","journal-title":"Brain Pathol"},{"key":"649_CR7","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 IRA et al (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":"649_CR8","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1046\/j.1365-2990.2003.00463.x","volume":"29","author":"R Silva de","year":"2003","unstructured":"de Silva R, Lashley T, Gibb G et al (2003) Pathological inclusion bodies in tauopathies contain distinct complements of tau with three or four microtubule-binding repeat domains as demonstrated by new specific monoclonal antibodies. Neuropathol Appl Neurobiol 29:288\u2013302","journal-title":"Neuropathol Appl Neurobiol"},{"key":"649_CR9","doi-asserted-by":"crossref","unstructured":"Dickson DW (1999) Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration. J Neurol 246(Suppl 2):II6\u2013II15","DOI":"10.1007\/BF03161076"},{"key":"649_CR10","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 et al (2002) Office of rare diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 61:935\u2013946","journal-title":"J Neuropathol Exp Neurol"},{"key":"649_CR11","first-page":"115","volume-title":"Neurodegeneration: the molecular pathology of dementia and movement disorders","author":"D Dickson","year":"2003","unstructured":"Dickson D, Litvan I (2003) Corticobasal degeneration. In: Dickson DW (ed) Neurodegeneration: the molecular pathology of dementia and movement disorders. ISN Neuropath Press, Basel, pp 115\u2013123"},{"key":"649_CR12","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1111\/j.1750-3639.2007.00054.x","volume":"17","author":"DW Dickson","year":"2007","unstructured":"Dickson DW, Rademarkers R, Hutton ML (2007) Progressive supranuclear palsy: pathology and genetics. Brain Pathol 17:74\u201382","journal-title":"Brain Pathol"},{"key":"649_CR13","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1007\/s00401-003-0756-4","volume":"106","author":"I Ferrer","year":"2003","unstructured":"Ferrer I, Hern\u00e1ndez I, Boada M et al (2003) Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies. Acta Neuropathol 106:419\u2013435","journal-title":"Acta Neuropathol"},{"key":"649_CR14","first-page":"86","volume-title":"Neurodegeneration: the molecular pathology of dementia and movement disorders","author":"B Ghetti","year":"2003","unstructured":"Ghetti B, Hutton ML, Wszolek ZK (2003) Frontotemporal dementia and Parkinsonism linked to chromosome 17 associated with tau gene mutation (FTDP-17T). In: Dickson DW (ed) Neurodegeneration: the molecular pathology of dementia and movement disorders. ISN Neuropath Press, Basel, pp 86\u2013102"},{"key":"649_CR15","first-page":"103","volume-title":"Neurodegeneration: the molecular pathology of dementia and movement disorders","author":"J-J Hauw","year":"2003","unstructured":"Hauw J-J, Agid Y (2003) Progressive supranuclear palsy (PSP) or Steele-Richardson-Olszewski disease. In: Dickson DW (ed) Neurodegeneration: the molecular pathology of dementia and movement disorders. ISN Neuropath Press, Basel, pp 103\u2013114"},{"key":"649_CR16","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 et al (2006) Atypical progressive supranuclear palsy with corticospinal tract degeneration. J Neuropathol Exp Neurol 65:396\u2013405","journal-title":"J Neuropathol Exp Neurol"},{"key":"649_CR17","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1007\/s00401-003-0728-8","volume":"106","author":"O Katsuse","year":"2003","unstructured":"Katsuse O, Iseki E, Arai T et al (2003) 4-Repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 106:251\u2013260","journal-title":"Acta Neuropathol"},{"key":"649_CR18","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1007\/s004010050911","volume":"96","author":"T Komori","year":"1998","unstructured":"Komori T, Arai N, Oda M, Nakayama H et al (1998) Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 96:401\u2013408","journal-title":"Acta Neuropathol"},{"key":"649_CR19","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 et al (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":"649_CR20","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1007\/BF00691887","volume":"45","author":"S Kuroda","year":"1979","unstructured":"Kuroda S, Otsuki S, Tateishi J, Hirano A (1979) Neurofibrillary degeneration in a case of quadriplegia and myoclonic movement. Acta Neuropathol 45:105\u2013109","journal-title":"Acta Neuropathol"},{"key":"649_CR21","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 et al (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":"649_CR22","first-page":"183","volume":"82","author":"I Litvan","year":"2000","unstructured":"Litvan I, Grimes DA, Lang AE (2000) Phenotypes and prognosis: clinicopathologic studies of corticobasal degeneration. Adv Neurol 82:183\u2013196","journal-title":"Adv Neurol"},{"key":"649_CR23","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1007\/s00401-008-0460-5","volume":"117","author":"IRA Mackenzie","year":"2009","unstructured":"Mackenzie IRA, Neumann M, Bigio EH et al (2009) Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol 117:15\u201318","journal-title":"Acta Neuropathol"},{"key":"649_CR24","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00401-009-0612-2","volume":"119","author":"IRA Mackenzie","year":"2010","unstructured":"Mackenzie IRA, Neumann M, Bigio EH et al (2010) Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol 119:1\u20134","journal-title":"Acta Neuropathol"},{"key":"649_CR25","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1007\/s00401-005-1028-2","volume":"110","author":"O Martinaud","year":"2005","unstructured":"Martinaud O, Laquerri\u00e8re A, Guyant-Mar\u00e9chal L et al (2005) Frontotemporal dementia, motor neuron disease and tauopathy: clinical and neuropathological study in a family. Acta Neuropathol 110:84\u201392","journal-title":"Acta Neuropathol"},{"key":"649_CR26","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":"649_CR27","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 IRA (2009) A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain 132:2922\u20132931","journal-title":"Brain"},{"key":"649_CR28","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1007\/s00401-008-0385-z","volume":"116","author":"Y Nishihira","year":"2008","unstructured":"Nishihira Y, Tan CF, Onodera O et al (2008) Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions. Acta Neuropathol 116:169\u2013182","journal-title":"Acta Neuropathol"},{"key":"649_CR29","doi-asserted-by":"crossref","unstructured":"Okazaki K, Fu Y-J, Nishihira Y et al (2009) Alzheimer\u2019s disease: report of tow autopsy cases with a clinical diagnosis of corticobasal degeneration. Neuropathology doi: 10.1111\/j.1440-1789.2009.01062.x","DOI":"10.1111\/j.1440-1789.2009.01062.x"},{"key":"649_CR30","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1007\/s00401-005-1086-5","volume":"110","author":"Y-S Piao","year":"2005","unstructured":"Piao Y-S, Tan C-F, Iwanaga K et al (2005) Sporadic four-repeat tauopathy with frontotemporal degeneration, Parkinsonism and motor neuron disease. Acta Neuropathol 110:600\u2013609","journal-title":"Acta Neuropathol"},{"key":"649_CR31","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1111\/j.1750-3639.2003.tb00002.x","volume":"12","author":"Y-S Piao","year":"2003","unstructured":"Piao Y-S, Wakabayashi K, Kakita A et al (2003) Neuropathology with clinical correlations of sporadic amyotrophic lateral sclerosis: 102 autopsy cases examined between 1962 and 2000. Brain Pathol 12:10\u201322","journal-title":"Brain Pathol"},{"key":"649_CR32","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1007\/s00401-003-0719-9","volume":"106","author":"JM Powers","year":"2003","unstructured":"Powers JM, Bryne NP, Ito M et al (2003) A novel leukoencephalopathy associated with tau deposits primarily in white matter glia. Acta Neuropathol 106:181\u2013187","journal-title":"Acta Neuropathol"},{"key":"649_CR33","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1086\/302256","volume":"64","author":"P Rizzu","year":"1999","unstructured":"Rizzu P, Van Swieten JC, Joosse M et al (1999) High prevalence of mutations in the microtubule-associated protein tau in a population study of frontotemporal dementia in the Netherlands. Am J Hum Genet 64:414\u2013421","journal-title":"Am J Hum Genet"},{"key":"649_CR34","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1007\/s00401-006-0093-5","volume":"112","author":"K Sakai","year":"2006","unstructured":"Sakai K, Piao Y-S, Kikugawa K et al (2006) Corticobasal degeneration with focal, massive tau accumulation in the subcortical white matter astrocytes. Acta Neuropathol 112:341\u2013348","journal-title":"Acta Neuropathol"},{"key":"649_CR35","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":"649_CR36","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1007\/s00401-003-0687-0","volume":"105","author":"C-F Tan","year":"2003","unstructured":"Tan C-F, Kakita A, Piao Y-S et al (2003) Primary lateral sclerosis: a rare upper-motor-predominant form of amyotrophic lateral sclerosis often accompanied by frontotemporal lobar degeneration with ubiquitinated neuronal inclusions? Report of an autopsy case and a review of the literature. Acta Neuropathol 105:615\u2013620","journal-title":"Acta Neuropathol"},{"key":"649_CR37","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1007\/s00401-004-0933-0","volume":"109","author":"C-F Tan","year":"2005","unstructured":"Tan C-F, Piao Y-S, Kakita A et al (2005) Frontotemporal dementia with co-occurrence of astrocytic plaques and tufted astrocytes, and severe degeneration of the cerebral white matter: a variant of corticobasal degeneration? Acta Neuropathol 109:329\u2013338","journal-title":"Acta Neuropathol"},{"key":"649_CR38","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1007\/s00401-007-0200-2","volume":"113","author":"T Uchihara","year":"2007","unstructured":"Uchihara T (2007) Silver diagnosis in neuropathology: principles, practice and revised interpretation. Acta Neuropathol 113:483\u2013499","journal-title":"Acta Neuropathol"},{"key":"649_CR39","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1046\/j.1440-1789.2003.00523.x","volume":"23","author":"T Uchihara","year":"2003","unstructured":"Uchihara T, Ikeda K, Tsuchiya K (2003) Pick body disease and Pick syndrome. Neuropathology 23:318\u2013326","journal-title":"Neuropathology"},{"key":"649_CR40","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":"649_CR41","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1007\/s00401-006-0141-1","volume":"112","author":"O Yokota","year":"2006","unstructured":"Yokota O, Tsuchiya K, Oda T et al (2006) Amyotrophic lateral sclerosis with dementia: an autopsy case showing many Bunina bodies, tau-positive neuronal and astrocytic plaque-like pathologies, and pallido-nigral degeneration. Acta Neuropathol 112:633\u2013645","journal-title":"Acta Neuropathol"},{"key":"649_CR42","doi-asserted-by":"crossref","first-page":"1578","DOI":"10.1212\/01.WNL.0000160116.65034.12","volume":"64","author":"JJ Zarranz","year":"2005","unstructured":"Zarranz JJ, Ferrer I, Lezcano E et al (2005) A novel mutation (K317M) in the MAPT gene causes FTDP and motor neuron disease. Neurology 64:1578\u20131585","journal-title":"Neurology"},{"key":"649_CR43","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1002\/ana.10222","volume":"51","author":"V Zhukareva","year":"2002","unstructured":"Zhukareva V, Mann D, Pickering-Brown S et al (2002) Sporadic Pick\u2019s disease: a tauopathy characterized by a spectrum of pathological \u03c4 isoforms in gray and white matter. Ann Neurol 51:730\u2013739","journal-title":"Ann Neurol"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-010-0649-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00401-010-0649-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-010-0649-2","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,23]],"date-time":"2024-03-23T20:20:22Z","timestamp":1711225222000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00401-010-0649-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,2,7]]},"references-count":43,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2010,7]]}},"alternative-id":["649"],"URL":"https:\/\/doi.org\/10.1007\/s00401-010-0649-2","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,2,7]]}}}