{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T08:14:20Z","timestamp":1783498460017,"version":"3.55.0"},"reference-count":55,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Revisions of the McDonald diagnostic criteria have improved the sensitivity of multiple sclerosis (MS) diagnosis by incorporating MRI-based dissemination in space and time. However, some inflammatory demyelinating lesions fulfill dissemination criteria while showing radiological features atypical for MS, even in the absence of anti-AQP4 and anti-MOG antibodies. The long-term radiological course of these atypical idiopathic inflammatory demyelinating lesions (AIIDLs) remains unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed 39 Japanese patients with AIIDLs, classified into multiple spotty lesions (MSLs), disseminated encephalomyelitis-like lesions (DEMLs), leukoencephalopathy-like lesions (LELs), and tumefactive lesions (TLs), and compared them with 97 patients with radiologically typical MS. Longitudinal MRI analyses evaluated lesion morphology, distribution, volume, and central vein sign (CVS) positivity, together with clinical outcomes and treatment responses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Over a mean follow-up of 11\u00a0years, 38 of 39 AIIDL cases (97.4%) retained radiologically atypical features despite clinical relapses and fulfillment of dissemination criteria. MSLs and TLs lacked canonical MS features, such as periventricular lesions and T1 hypointense black holes. Only one case with LELs evolved into typical MS. CVS positivity exceeding 40% was observed in two MSLs cases but lesions remained atypical in distribution. Lesion volumes differed significantly among subgroups, with smaller volumes in MSLs and larger volumes in LELs compared with typical MS. Corticosteroids, immunosuppressants, and B cell depletion therapies appeared to be associated with clinical stabilization in several AIIDL subtypes, whereas conventional MS disease-modifying therapies were less consistently effective.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In this cohort, most AIIDLs retained radiologically atypical brain MRI features over long-term follow-up despite recurrent inflammatory demyelinating events. These findings suggest that dissemination-based criteria alone may not fully capture the radiological heterogeneity of AQP4-IgG- and MOG-IgG-negative demyelinating disorders. Longitudinal lesion-based MRI phenotyping may provide complementary information for diagnostic reassessment although external validation and integration with optic nerve, spinal cord, and biological markers are required.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00415-026-13951-6","type":"journal-article","created":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T18:09:19Z","timestamp":1782929359000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Beyond dissemination criteria: lesion-based longitudinal MRI of atypical demyelinating lesions at the boundary of multiple sclerosis"],"prefix":"10.1007","volume":"273","author":[{"given":"Yuriko","family":"Aratake","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenji","family":"Yoshinaga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinji","family":"Ashida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chihiro","family":"Fujii","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mio","family":"Hamatani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimitoshi","family":"Kimura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirofumi","family":"Ochi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryosuke","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7048-9401","authenticated-orcid":false,"given":"Takayuki","family":"Kondo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,1]]},"reference":[{"key":"13951_CR1","doi-asserted-by":"publisher","first-page":"552","DOI":"10.1111\/j.1749-6632.1965.tb20235.x","volume":"122","author":"GA Schumacher","year":"1965","unstructured":"Schumacher GA, Beebe G, Kibler RF, Kurland LT, Kurtzke JF, McDowell F et al (1965) Problems of experimental trials of therapy in multiple sclerosis: report by the panel on the evaluation of experimental trials of therapy in multiple sclerosis. Ann N Y Acad Sci 122:552\u2013568. https:\/\/doi.org\/10.1111\/j.1749-6632.1965.tb20235.x","journal-title":"Ann N Y Acad Sci"},{"issue":"10","key":"13951_CR2","doi-asserted-by":"publisher","first-page":"850","DOI":"10.1016\/S1474-4422(25)00270-4","volume":"24","author":"X Montalban","year":"2024","unstructured":"Montalban X, Lebrun-Fr\u00e9nay C, Oh J, Arrambide G, Moccia M, Pia Amato M et al (2024) Diagnosis of multiple sclerosis: 2024 revisions of the McDonald criteria. Lancet Neurol 24(10):850\u2013865. https:\/\/doi.org\/10.1016\/S1474-4422(25)00270-4","journal-title":"Lancet Neurol"},{"issue":"2","key":"13951_CR3","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1016\/S1474-4422(17)30470-2","volume":"17","author":"AJ Thompson","year":"2018","unstructured":"Thompson AJ, Banwell BL, Barkhof F, Carroll WM, Coetzee T, Comi G et al (2018) Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol 17(2):162\u2013173. https:\/\/doi.org\/10.1016\/S1474-4422(17)30470-2","journal-title":"Lancet Neurol"},{"issue":"3","key":"13951_CR4","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1016\/j.clineuro.2004.02.004","volume":"106","author":"CM Poser","year":"2004","unstructured":"Poser CM, Brinar VV (2004) Diagnostic criteria for multiple sclerosis: an historical review. Clin Neurol Neurosurg 106(3):147\u2013158. https:\/\/doi.org\/10.1016\/j.clineuro.2004.02.004","journal-title":"Clin Neurol Neurosurg"},{"issue":"9","key":"13951_CR5","doi-asserted-by":"publisher","first-page":"567","DOI":"10.1038\/nrneurol.2017.106","volume":"13","author":"AJ Solomon","year":"2017","unstructured":"Solomon AJ, Corboy JR (2017) The tension between early diagnosis and misdiagnosis of multiple sclerosis. Nat Rev Neurol 13(9):567\u2013572. https:\/\/doi.org\/10.1038\/nrneurol.2017.106","journal-title":"Nat Rev Neurol"},{"issue":"24","key":"13951_CR6","doi-asserted-by":"publisher","first-page":"1986","DOI":"10.1212\/WNL.0b013e318259e1b2","volume":"78","author":"AJ Solomon","year":"2012","unstructured":"Solomon AJ, Klein EP, Bourdette D (2012) \u201cUndiagnosing\u201d multiple sclerosis: the challenge of misdiagnosis in MS. Neurology 78(24):1986\u20131991. https:\/\/doi.org\/10.1212\/WNL.0b013e318259e1b2","journal-title":"Neurology"},{"key":"13951_CR7","doi-asserted-by":"publisher","first-page":"466","DOI":"10.3389\/fneur.2019.00466","volume":"10","author":"MI Gait\u00e1n","year":"2019","unstructured":"Gait\u00e1n MI, Correale J (2019) Multiple sclerosis misdiagnosis: a persistent problem to solve. Front Neurol 10:466. https:\/\/doi.org\/10.3389\/fneur.2019.00466","journal-title":"Front Neurol"},{"issue":"Pt 11","key":"13951_CR8","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.1093\/brain\/120.11.2059","volume":"120","author":"F Barkhof","year":"1997","unstructured":"Barkhof F, Filippi M, Miller DH, Scheltens P, Campi A, Polman CH et al (1997) Comparison of MRI criteria at first presentation to predict conversion to clinically definite multiple sclerosis. Brain 120(Pt 11):2059\u20132069. https:\/\/doi.org\/10.1093\/brain\/120.11.2059","journal-title":"Brain"},{"issue":"10","key":"13951_CR9","doi-asserted-by":"publisher","first-page":"841","DOI":"10.1016\/S1474-4422(06)70572-5","volume":"5","author":"A Charil","year":"2006","unstructured":"Charil A, Yousry TA, Rovaris M, Barkhof F, De Stefano N, Fazekas F et al (2006) MRI and the diagnosis of multiple sclerosis: expanding the concept of \u201cno better explanation.\u201d Lancet Neurol 5(10):841\u2013852. https:\/\/doi.org\/10.1016\/S1474-4422(06)70572-5","journal-title":"Lancet Neurol"},{"issue":"7","key":"13951_CR10","doi-asserted-by":"publisher","first-page":"1858","DOI":"10.1093\/brain\/awz144","volume":"142","author":"M Filippi","year":"2019","unstructured":"Filippi M, Preziosa P, Banwell BL, Barkhof F, Ciccarelli O, De Stefano N et al (2019) Assessment of lesions on magnetic resonance imaging in multiple sclerosis: practical guidelines. Brain 142(7):1858\u20131875. https:\/\/doi.org\/10.1093\/brain\/awz144","journal-title":"Brain"},{"issue":"3","key":"13951_CR11","doi-asserted-by":"publisher","first-page":"1286","DOI":"10.1007\/s00415-022-11488-y","volume":"270","author":"M Filippi","year":"2023","unstructured":"Filippi M, Preziosa P, Arnold DL, Barkhof F, Harrison DM, Maggi P et al (2023) Present and future of the diagnostic work-up of multiple sclerosis: the imaging perspective. J Neurol 270(3):1286\u20131299. https:\/\/doi.org\/10.1007\/s00415-022-11488-y","journal-title":"J Neurol"},{"issue":"4","key":"13951_CR12","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1038\/nrneurol.2018.39","volume":"14","author":"R Geraldes","year":"2018","unstructured":"Geraldes R, Ciccarelli O, Barkhof F, De Stefano N, Enzinger C, Filippi M et al (2018) The current role of MRI in differentiating multiple sclerosis from its imaging mimics. Nat Rev Neurol 14(4):213. https:\/\/doi.org\/10.1038\/nrneurol.2018.39","journal-title":"Nat Rev Neurol"},{"issue":"1","key":"13951_CR13","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1007\/s00415-015-7952-8","volume":"263","author":"M Jury\u0144czyk","year":"2016","unstructured":"Jury\u0144czyk M, Weinshenker B, Akman-Demir G, Asgari N, Barnes D, Boggild M et al (2016) Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO\/MS overlap syndromes. J Neurol 263(1):140\u2013149. https:\/\/doi.org\/10.1007\/s00415-015-7952-8","journal-title":"J Neurol"},{"issue":"6","key":"13951_CR14","doi-asserted-by":"publisher","first-page":"480","DOI":"10.1111\/j.1469-8749.2008.03136.x","volume":"51","author":"G Alper","year":"2009","unstructured":"Alper G, Heyman R, Wang L (2009) Multiple sclerosis and acute disseminated encephalomyelitis diagnosed in children after long-term follow-up: comparison of presenting features. Dev Med Child Neurol 51(6):480\u2013486. https:\/\/doi.org\/10.1111\/j.1469-8749.2008.03136.x","journal-title":"Dev Med Child Neurol"},{"key":"13951_CR15","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awac480","author":"R Cortese","year":"2022","unstructured":"Cortese R, Battaglini M, Prados F, Bianchi A, Haider L, Jacob A et al (2022) Clinical and MRI measures to identify non-acute MOG-antibody disease in adults. Brain. https:\/\/doi.org\/10.1093\/brain\/awac480","journal-title":"Brain"},{"issue":"6","key":"13951_CR16","doi-asserted-by":"publisher","first-page":"408","DOI":"10.1016\/j.neurol.2018.03.007","volume":"174","author":"X Ayrignac","year":"2018","unstructured":"Ayrignac X, Carra-Dalli\u00e8re C, Labauge P (2018) Atypical inflammatory demyelinating lesions and atypical multiple sclerosis. Rev Neurol (Paris) 174(6):408\u2013418. https:\/\/doi.org\/10.1016\/j.neurol.2018.03.007","journal-title":"Rev Neurol (Paris)"},{"issue":"6","key":"13951_CR17","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1002\/1531-8249(200006)47:6<707::aid-ana3>3.0.co;2-q","volume":"47","author":"C Lucchinetti","year":"2000","unstructured":"Lucchinetti C, Br\u00fcck W, Parisi J, Scheithauer B, Rodriguez M, Lassmann H (2000) Heterogeneity of multiple sclerosis lesions: implications for the pathogenesis of demyelination. Ann Neurol 47(6):707\u2013717. https:\/\/doi.org\/10.1002\/1531-8249(200006)47:6%3c707::aid-ana3%3e3.0.co;2-q","journal-title":"Ann Neurol"},{"issue":"8","key":"13951_CR18","doi-asserted-by":"publisher","first-page":"2016","DOI":"10.1007\/s00415-013-6918-y","volume":"260","author":"M Wallner-Blazek","year":"2013","unstructured":"Wallner-Blazek M, Rovira A, Fillipp M, Rocca MA, Miller DH, Schmierer K et al (2013) Atypical idiopathic inflammatory demyelinating lesions: prognostic implications and relation to multiple sclerosis. J Neurol 260(8):2016\u20132022. https:\/\/doi.org\/10.1007\/s00415-013-6918-y","journal-title":"J Neurol"},{"issue":"11","key":"13951_CR19","doi-asserted-by":"publisher","first-page":"1996","DOI":"10.1016\/j.ejrad.2013.07.026","volume":"82","author":"C Koelblinger","year":"2013","unstructured":"Koelblinger C, Fruehwald-Pallamar J, Kubin K, Wallner-Blazek M, van den Hauwe L, Macedo L et al (2013) Atypical idiopathic inflammatory demyelinating lesions (IIDL): conventional and diffusion-weighted MR imaging (DWI) findings in 42 cases. Eur J Radiol 82(11):1996\u20132004. https:\/\/doi.org\/10.1016\/j.ejrad.2013.07.026","journal-title":"Eur J Radiol"},{"issue":"1","key":"13951_CR20","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s00415-007-0754-x","volume":"255","author":"A Seewann","year":"2008","unstructured":"Seewann A, Enzinger C, Filippi M, Barkhof F, Rovira A, Gass A et al (2008) MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain\u202f: A review of reported findings. J Neurol 255(1):1\u201310. https:\/\/doi.org\/10.1007\/s00415-007-0754-x","journal-title":"J Neurol"},{"key":"13951_CR21","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1016\/j.msard.2018.12.022","volume":"28","author":"P Codjia","year":"2019","unstructured":"Codjia P, Ayrignac X, Carra-Dalliere C, Cohen M, Charif M, Lippi A et al (2019) Multiple sclerosis with atypical MRI presentation: Results of a nationwide multicenter study in 57 consecutive cases. Mult Scler Relat Disord 28:109\u2013116. https:\/\/doi.org\/10.1016\/j.msard.2018.12.022","journal-title":"Mult Scler Relat Disord"},{"key":"13951_CR22","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2021.749406","volume":"12","author":"S Ashida","year":"2021","unstructured":"Ashida S, Ochi H, Hamatani M, Fujii C, Nishigori R, Kawamura K et al (2021) Radiological and Laboratory Features of Multiple Sclerosis Patients With Immunosuppressive Therapy: A Multicenter Retrospective Study in Japan. Front Neurol 12:749406. https:\/\/doi.org\/10.3389\/fneur.2021.749406","journal-title":"Front Neurol"},{"key":"13951_CR23","doi-asserted-by":"publisher","first-page":"1012857","DOI":"10.3389\/fneur.2022.1012857","volume":"13","author":"S Ashida","year":"2022","unstructured":"Ashida S, Kondo T, Fujii C, Hamatani M, Mizuno T, Ochi H (2022) Association of cerebrospinal inflammatory profile with radiological features in newly diagnosed treatment-na\u00efve patients with multiple sclerosis. Front Neurol 13:1012857. https:\/\/doi.org\/10.3389\/fneur.2022.1012857","journal-title":"Front Neurol"},{"key":"13951_CR24","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000001120","author":"O Al-Louzi","year":"2022","unstructured":"Al-Louzi O, Letchuman V, Manukyan S, Beck ES, Roy S, Ohayon J et al (2022) Central vein sign profile of newly developing lesions in multiple sclerosis: a 3-year longitudinal study. Neurol Neuroimmunol Neuroinflamm. https:\/\/doi.org\/10.1212\/NXI.0000000000001120","journal-title":"Neurol Neuroimmunol Neuroinflamm."},{"issue":"2","key":"13951_CR25","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1177\/1352458517717808","volume":"24","author":"N De Stefano","year":"2018","unstructured":"De Stefano N, Giorgio A, Tintor\u00e9 M, Pia Amato M, Kappos L, Palace J et al (2018) Radiologically isolated syndrome or subclinical multiple sclerosis: MAGNIMS consensus recommendations. Mult Scler 24(2):214\u2013221. https:\/\/doi.org\/10.1177\/1352458517717808","journal-title":"Mult Scler"},{"issue":"1","key":"13951_CR26","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1002\/ana.410330105","volume":"33","author":"JJ Kepes","year":"1993","unstructured":"Kepes JJ (1993) Large focal tumor-like demyelinating lesions of the brain: intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis? A study of 31 patients. Ann Neurol 33(1):18\u201327. https:\/\/doi.org\/10.1002\/ana.410330105","journal-title":"Ann Neurol"},{"issue":"2","key":"13951_CR27","doi-asserted-by":"publisher","first-page":"252","DOI":"10.1111\/j.1468-1331.2010.03114.x","volume":"18","author":"MF Liao","year":"2011","unstructured":"Liao MF, Huang CC, Lyu RK, Chen CM, Chang HS, Chu CC et al (2011) Acute disseminated encephalomyelitis that meets modified McDonald criteria for dissemination in space is associated with a high probability of conversion to multiple sclerosis in Taiwanese patients. Eur J Neurol 18(2):252\u2013259. https:\/\/doi.org\/10.1111\/j.1468-1331.2010.03114.x","journal-title":"Eur J Neurol"},{"issue":"11","key":"13951_CR28","doi-asserted-by":"publisher","first-page":"1444","DOI":"10.1212\/wnl.33.11.1444","volume":"33","author":"JF Kurtzke","year":"1983","unstructured":"Kurtzke JF (1983) Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 33(11):1444\u20131452. https:\/\/doi.org\/10.1212\/wnl.33.11.1444","journal-title":"Neurology"},{"issue":"12","key":"13951_CR29","doi-asserted-by":"publisher","first-page":"714","DOI":"10.1038\/nrneurol.2016.166","volume":"12","author":"P Sati","year":"2016","unstructured":"Sati P, Oh J, Constable RT, Evangelou N, Guttmann CR, Henry RG et al (2016) The central vein sign and its clinical evaluation for the diagnosis of multiple sclerosis: a consensus statement from the North American Imaging in Multiple Sclerosis Cooperative. Nat Rev Neurol 12(12):714\u2013722. https:\/\/doi.org\/10.1038\/nrneurol.2016.166","journal-title":"Nat Rev Neurol"},{"issue":"6","key":"13951_CR30","doi-asserted-by":"publisher","first-page":"750","DOI":"10.1177\/1352458517726383","volume":"24","author":"AJ Solomon","year":"2018","unstructured":"Solomon AJ, Watts R, Ontaneda D, Absinta M, Sati P, Reich DS (2018) Diagnostic performance of central vein sign for multiple sclerosis with a simplified three-lesion algorithm. Mult Scler 24(6):750\u2013757. https:\/\/doi.org\/10.1177\/1352458517726383","journal-title":"Mult Scler"},{"issue":"12","key":"13951_CR31","doi-asserted-by":"publisher","first-page":"1446","DOI":"10.1001\/jamaneurol.2019.2478","volume":"76","author":"T Sinnecker","year":"2019","unstructured":"Sinnecker T, Clarke MA, Meier D, Enzinger C, Calabrese M, De Stefano N et al (2019) Evaluation of the central vein sign as a diagnostic imaging biomarker in multiple sclerosis. JAMA Neurol 76(12):1446\u20131456. https:\/\/doi.org\/10.1001\/jamaneurol.2019.2478","journal-title":"JAMA Neurol"},{"issue":"4","key":"13951_CR32","doi-asserted-by":"publisher","first-page":"394","DOI":"10.1177\/1352458520907910","volume":"26","author":"AJ Solomon","year":"2020","unstructured":"Solomon AJ (2020) Progress towards a diagnostic biomarker for MS: Central vein sign. Mult Scler 26(4):394\u2013396. https:\/\/doi.org\/10.1177\/1352458520907910","journal-title":"Mult Scler"},{"issue":"6","key":"13951_CR33","doi-asserted-by":"publisher","first-page":"479.e9","DOI":"10.1016\/j.crad.2020.01.011","volume":"75","author":"A Bhandari","year":"2020","unstructured":"Bhandari A, Xiang H, Lechner-Scott J, Agzarian M (2020) Central vein sign for multiple sclerosis: A systematic review and meta-analysis. Clin Radiol 75(6):479.e9-e15. https:\/\/doi.org\/10.1016\/j.crad.2020.01.011","journal-title":"Clin Radiol"},{"issue":"3","key":"13951_CR34","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1111\/ane.13553","volume":"145","author":"L Chaaban","year":"2022","unstructured":"Chaaban L, Safwan N, Moussa H, El-Sammak S, Khoury SJ, Hannoun S (2022) Central vein sign: A putative diagnostic marker for multiple sclerosis. Acta Neurol Scand 145(3):279\u2013287. https:\/\/doi.org\/10.1111\/ane.13553","journal-title":"Acta Neurol Scand"},{"key":"13951_CR35","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics10121025","author":"M Castellaro","year":"2020","unstructured":"Castellaro M, Tamanti A, Pisani AI, Pizzini FB, Crescenzo F, Calabrese M (2020) The use of the central vein sign in the diagnosis of multiple sclerosis: a systematic review and meta-analysis. Diagnostics (Basel). https:\/\/doi.org\/10.3390\/diagnostics10121025","journal-title":"Diagnostics (Basel)"},{"issue":"9","key":"13951_CR36","doi-asserted-by":"publisher","first-page":"967","DOI":"10.1016\/S1474-4422(16)30043-6","volume":"15","author":"TA Hardy","year":"2016","unstructured":"Hardy TA, Reddel SW, Barnett MH, Palace J, Lucchinetti CF, Weinshenker BG (2016) Atypical inflammatory demyelinating syndromes of the CNS. Lancet Neurol 15(9):967\u2013981. https:\/\/doi.org\/10.1016\/S1474-4422(16)30043-6","journal-title":"Lancet Neurol"},{"issue":"1","key":"13951_CR37","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1159\/000052095","volume":"45","author":"KY Tsai","year":"2001","unstructured":"Tsai KY, Tsai CP, Liao N (2001) Sj\u00f6gren\u2019s syndrome with central nervous system involvement presenting as multiple sclerosis with failure response to beta-interferon. Eur Neurol 45(1):59\u201360. https:\/\/doi.org\/10.1159\/000052095","journal-title":"Eur Neurol"},{"issue":"4","key":"13951_CR38","doi-asserted-by":"publisher","first-page":"431","DOI":"10.1007\/s00296-009-1175-1","volume":"30","author":"B Keiserman","year":"2010","unstructured":"Keiserman B, da Silva LF, Keiserman MW, von M\u00fchlen CA, Staub HL (2010) Lupoid sclerosis. Rheumatol Int 30(4):431\u2013434. https:\/\/doi.org\/10.1007\/s00296-009-1175-1","journal-title":"Rheumatol Int"},{"key":"13951_CR39","doi-asserted-by":"publisher","DOI":"10.1016\/j.msard.2019.101906","volume":"39","author":"A Borhani-Haghighi","year":"2019","unstructured":"Borhani-Haghighi A, Kardeh B, Banerjee S, Yadollahikhales G, Safari A, Sahraian MA et al (2019) Neuro-Behcet\u2019s disease: An update on diagnosis, differential diagnoses, and treatment. Mult Scler Relat Disord 39:101906. https:\/\/doi.org\/10.1016\/j.msard.2019.101906","journal-title":"Mult Scler Relat Disord"},{"issue":"2","key":"13951_CR40","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1016\/j.ejpn.2006.11.007","volume":"11","author":"Y Mikaeloff","year":"2007","unstructured":"Mikaeloff Y, Caridade G, Husson B, Suissa S, Tardieu M, Society NKSGotFN (2007) Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse. Eur J Paediatr Neurol. 11(2):90\u201395. https:\/\/doi.org\/10.1016\/j.ejpn.2006.11.007","journal-title":"Eur J Paediatr Neurol."},{"issue":"10","key":"13951_CR41","doi-asserted-by":"publisher","first-page":"1308","DOI":"10.1212\/wnl.56.10.1308","volume":"56","author":"JL Hynson","year":"2001","unstructured":"Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ (2001) Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 56(10):1308\u20131312. https:\/\/doi.org\/10.1212\/wnl.56.10.1308","journal-title":"Neurology"},{"issue":"16 Suppl 2","key":"13951_CR42","doi-asserted-by":"publisher","first-page":"S23","DOI":"10.1212\/01.wnl.0000259404.51352.7f","volume":"68","author":"S Tenembaum","year":"2007","unstructured":"Tenembaum S, Chitnis T, Ness J, Hahn JS, Group IPMS (2007) Acute disseminated encephalomyelitis. Neurology 68(16 Suppl 2):S23-36. https:\/\/doi.org\/10.1212\/01.wnl.0000259404.51352.7f","journal-title":"Neurology"},{"issue":"9 Suppl 2","key":"13951_CR43","doi-asserted-by":"publisher","first-page":"S38","DOI":"10.1212\/WNL.0000000000002825","volume":"87","author":"D Pohl","year":"2016","unstructured":"Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S et al (2016) Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. Neurology 87(9 Suppl 2):S38-45. https:\/\/doi.org\/10.1212\/WNL.0000000000002825","journal-title":"Neurology"},{"issue":"2","key":"13951_CR44","doi-asserted-by":"publisher","first-page":"246","DOI":"10.1016\/j.jpeds.2003.10.056","volume":"144","author":"Y Mikaeloff","year":"2004","unstructured":"Mikaeloff Y, Suissa S, Vall\u00e9e L, Lubetzki C, Ponsot G, Confavreux C et al (2004) First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability. J Pediatr 144(2):246\u2013252. https:\/\/doi.org\/10.1016\/j.jpeds.2003.10.056","journal-title":"J Pediatr"},{"key":"13951_CR45","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.870867","volume":"13","author":"K Li","year":"2022","unstructured":"Li K, Li M, Wen L, Wang Q, Ding X, Wang J (2022) Clinical presentation and outcomes of acute disseminated encephalomyelitis in adults worldwide: systematic review and meta-analysis. Front Immunol 13:870867. https:\/\/doi.org\/10.3389\/fimmu.2022.870867","journal-title":"Front Immunol"},{"issue":"10","key":"13951_CR46","doi-asserted-by":"publisher","first-page":"1313","DOI":"10.1212\/wnl.56.10.1313","volume":"56","author":"S Schwarz","year":"2001","unstructured":"Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B (2001) Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. Neurology 56(10):1313\u20131318. https:\/\/doi.org\/10.1212\/wnl.56.10.1313","journal-title":"Neurology"},{"issue":"9","key":"13951_CR47","doi-asserted-by":"publisher","first-page":"2013","DOI":"10.1007\/s00415-015-7694-7","volume":"262","author":"DL Koelman","year":"2015","unstructured":"Koelman DL, Mateen FJ (2015) Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome. J Neurol 262(9):2013\u20132024. https:\/\/doi.org\/10.1007\/s00415-015-7694-7","journal-title":"J Neurol"},{"issue":"6","key":"13951_CR48","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1007\/BF00626098","volume":"38","author":"AP Dagher","year":"1996","unstructured":"Dagher AP, Smirniotopoulos J (1996) Tumefactive demyelinating lesions. Neuroradiology 38(6):560\u2013565. https:\/\/doi.org\/10.1007\/BF00626098","journal-title":"Neuroradiology"},{"issue":"Pt 7","key":"13951_CR49","doi-asserted-by":"publisher","first-page":"1759","DOI":"10.1093\/brain\/awn098","volume":"131","author":"CF Lucchinetti","year":"2008","unstructured":"Lucchinetti CF, Gavrilova RH, Metz I, Parisi JE, Scheithauer BW, Weigand S et al (2008) Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain 131(Pt 7):1759\u20131775. https:\/\/doi.org\/10.1093\/brain\/awn098","journal-title":"Brain"},{"issue":"10","key":"13951_CR50","doi-asserted-by":"publisher","first-page":"1448","DOI":"10.1177\/1352458512438237","volume":"18","author":"A Altintas","year":"2012","unstructured":"Altintas A, Petek B, Isik N, Terzi M, Bolukbasi F, Tavsanli M et al (2012) Clinical and radiological characteristics of tumefactive demyelinating lesions: follow-up study. Mult Scler 18(10):1448\u20131453. https:\/\/doi.org\/10.1177\/1352458512438237","journal-title":"Mult Scler"},{"issue":"7","key":"13951_CR51","doi-asserted-by":"publisher","first-page":"1637","DOI":"10.1007\/s00415-015-7758-8","volume":"262","author":"A Siri","year":"2015","unstructured":"Siri A, Carra-Dalliere C, Ayrignac X, Pelletier J, Audoin B, Pittion-Vouyovitch S et al (2015) Isolated tumefactive demyelinating lesions: diagnosis and long-term evolution of 16 patients in a multicentric study. J Neurol 262(7):1637\u20131645. https:\/\/doi.org\/10.1007\/s00415-015-7758-8","journal-title":"J Neurol"},{"issue":"1","key":"13951_CR52","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1111\/ane.12071","volume":"128","author":"M Nagappa","year":"2013","unstructured":"Nagappa M, Taly AB, Sinha S, Bharath RD, Mahadevan A, Bindu PS et al (2013) Tumefactive demyelination: clinical, imaging and follow-up observations in thirty-nine patients. Acta Neurol Scand 128(1):39\u201347. https:\/\/doi.org\/10.1111\/ane.12071","journal-title":"Acta Neurol Scand"},{"issue":"10","key":"13951_CR53","doi-asserted-by":"publisher","first-page":"1197","DOI":"10.1001\/jamaneurol.2021.2738","volume":"78","author":"T Spelman","year":"2021","unstructured":"Spelman T, Magyari M, Piehl F, Svenningsson A, Rasmussen PV, Kant M et al (2021) Treatment Escalation vs Immediate Initiation of Highly Effective Treatment for Patients With Relapsing-Remitting Multiple Sclerosis: Data From 2 Different National Strategies. JAMA Neurol 78(10):1197\u20131204. https:\/\/doi.org\/10.1001\/jamaneurol.2021.2738","journal-title":"JAMA Neurol"},{"issue":"6","key":"13951_CR54","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000626","volume":"6","author":"T Yeo","year":"2019","unstructured":"Yeo T, Probert F, Jurynczyk M, Sealey M, Cavey A, Claridge TDW et al (2019) Classifying the antibody-negative NMO syndromes: Clinical, imaging, and metabolomic modeling. Neurol Neuroimmunol Neuroinflamm 6(6):e626. https:\/\/doi.org\/10.1212\/NXI.0000000000000626","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"issue":"1","key":"13951_CR55","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1007\/s00415-021-10619-1","volume":"269","author":"M Jury\u0144czyk","year":"2022","unstructured":"Jury\u0144czyk M, Klimiec-Moskal E, Kong Y, Hurley S, Messina S, Yeo T, Jenkinson M, Leite MI, Palace J (2022) Elucidating distinct clinico-radiologic signatures in the borderlasnd between neuromyelitis optica and multiple sclerosis. J Neurol 269(1):269\u2013279. https:\/\/doi.org\/10.1007\/s00415-021-10619-1","journal-title":"J Neurol"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-026-13951-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-026-13951-6","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-026-13951-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T07:22:07Z","timestamp":1783495327000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-026-13951-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":55,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["13951"],"URL":"https:\/\/doi.org\/10.1007\/s00415-026-13951-6","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]},"assertion":[{"value":"22 March 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 June 2026","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 June 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 July 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare that they have no competing interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}}],"article-number":"438"}}