{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,3]],"date-time":"2025-08-03T04:34:28Z","timestamp":1754195668760},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T00:00:00Z","timestamp":1704153600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T00:00:00Z","timestamp":1704153600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Universit\u00e4tsklinikum D\u00fcsseldorf. Anstalt \u00f6ffentlichen Rechts"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Med Imaging"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Susac syndrome (SuS) is a rare autoimmune disease that leads to hearing impairment, visual field deficits, and encephalopathy due to an occlusion of precapillary arterioles in the brain, retina, and inner ear. Given the potentially disastrous outcome and difficulties in distinguishing SuS from its differential diagnoses, such as multiple sclerosis (MS), our exploratory study aimed at identifying potential new SuS-specific neuroimaging markers.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Seven patients with a definite diagnosis of SuS underwent magnetic resonance imaging (MRI) at 7 Tesla (7T), including T2* weighted and quantitative susceptibility mapping (QSM) sequences. T2 weighted hyperintense lesions were analyzed with regard to number, volume, localization, central vein sign, T1 hypointensity, and focal iron deposits in the center of SuS lesions (\u201ciron dots\u201d). Seven T MRI datasets from the same institute, comprising 75 patients with, among others, MS, served as controls.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>The \u201ciron dot\u201d sign was present in 71.4% (5\/7) of the SuS patients, compared to 0% in our control cohort. Thus, sensitivity was 71.4% and specificity 100%. A central vein sign was only incidentally detected.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>We are the first to demonstrate this type of \u201ciron dot\u201d lesions on highly resolving 7T T2*w and QSM images in vivo as a promising neuroimaging marker of SuS, corroborating previous histopathological ex vivo findings.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12880-023-01171-7","type":"journal-article","created":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T12:03:40Z","timestamp":1704197020000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Central intra-lesional iron deposits as a possible novel imaging marker at 7 Tesla MRI in Susac Syndrome - an exploratory study"],"prefix":"10.1186","volume":"24","author":[{"given":"Daniel","family":"Strunk","sequence":"first","affiliation":[]},{"given":"Tim","family":"Sinnecker","sequence":"additional","affiliation":[]},{"given":"Ilka","family":"Kleffner","sequence":"additional","affiliation":[]},{"given":"Jan","family":"Doerr","sequence":"additional","affiliation":[]},{"given":"Marius","family":"Ringelstein","sequence":"additional","affiliation":[]},{"given":"Catharina C.","family":"Gross","sequence":"additional","affiliation":[]},{"given":"Cornelius","family":"Deuschl","sequence":"additional","affiliation":[]},{"given":"Stefan","family":"Maderwald","sequence":"additional","affiliation":[]},{"given":"Harald H.","family":"Quick","sequence":"additional","affiliation":[]},{"given":"Elif","family":"Yamac","sequence":"additional","affiliation":[]},{"given":"Karsten H.","family":"Wrede","sequence":"additional","affiliation":[]},{"given":"Markus","family":"Kraemer","sequence":"additional","affiliation":[]}],"member":"297","published-online":{"date-parts":[[2024,1,2]]},"reference":[{"key":"1171_CR1","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1212\/wnl.44.4.591","volume":"44","author":"JO Susac","year":"1994","unstructured":"Susac JO. Susac\u2019s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology. 1994;44:591\u20133. https:\/\/doi.org\/10.1212\/wnl.44.4.591.","journal-title":"Neurology"},{"key":"1171_CR2","doi-asserted-by":"publisher","first-page":"5779","DOI":"10.1038\/s41467-019-13593-5","volume":"10","author":"CC Gross","year":"2019","unstructured":"Gross CC, Meyer C, Bhatia U, et al. CD8\u2009+\u2009T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 2019;10:5779. https:\/\/doi.org\/10.1038\/s41467-019-13593-5.","journal-title":"Nat Commun"},{"key":"1171_CR3","doi-asserted-by":"publisher","first-page":"1287","DOI":"10.1136\/jnnp-2016-314295","volume":"87","author":"I Kleffner","year":"2016","unstructured":"Kleffner I, D\u00f6rr J, Ringelstein M, et al. Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016;87:1287\u201395. https:\/\/doi.org\/10.1136\/jnnp-2016-314295.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"1171_CR4","doi-asserted-by":"publisher","first-page":"1783","DOI":"10.1212\/01.wnl.0000103880.29693.48","volume":"61","author":"JO Susac","year":"2003","unstructured":"Susac JO, Murtagh FR, Egan RA, et al. MRI findings in Susac\u2019s syndrome. Neurology. 2003;61:1783\u20137. https:\/\/doi.org\/10.1212\/01.wnl.0000103880.29693.48.","journal-title":"Neurology"},{"key":"1171_CR5","doi-asserted-by":"publisher","first-page":"1867","DOI":"10.1212\/01.wnl.0000280580.95671.01","volume":"70","author":"I Kleffner","year":"2008","unstructured":"Kleffner I, Deppe M, Mohammadi S, et al. Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 2008;70:1867\u20139. https:\/\/doi.org\/10.1212\/01.wnl.0000280580.95671.01.","journal-title":"Neurology"},{"key":"1171_CR6","doi-asserted-by":"publisher","first-page":"92","DOI":"10.1016\/j.jns.2010.08.028","volume":"299","author":"I Kleffner","year":"2010","unstructured":"Kleffner I, Deppe M, Mohammadi S, et al. Neuroimaging in Susac\u2019s syndrome: focus on DTI. J Neurol Sci. 2010;299:92\u20136. https:\/\/doi.org\/10.1016\/j.jns.2010.08.028.","journal-title":"J Neurol Sci"},{"key":"1171_CR7","doi-asserted-by":"publisher","first-page":"1592","DOI":"10.1177\/1352458512441270","volume":"18","author":"J Wuerfel","year":"2012","unstructured":"Wuerfel J, Sinnecker T, Ringelstein EB, et al. Lesion morphology at 7 Tesla MRI differentiates Susac syndrome from multiple sclerosis. Mult Scler Houndmills Basingstoke Engl. 2012;18:1592\u20139. https:\/\/doi.org\/10.1177\/1352458512441270.","journal-title":"Mult Scler Houndmills Basingstoke Engl"},{"key":"1171_CR8","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1177\/1352458519876031","volume":"26","author":"P Maggi","year":"2020","unstructured":"Maggi P, Absinta M, Sati P, et al. The central vein sign in patients with diagnostic red flags for multiple sclerosis: a prospective multicenter 3T study. Mult Scler Houndmills Basingstoke Engl. 2020;26:421\u201332. https:\/\/doi.org\/10.1177\/1352458519876031.","journal-title":"Mult Scler Houndmills Basingstoke Engl"},{"key":"1171_CR9","doi-asserted-by":"publisher","first-page":"699","DOI":"10.3174\/ajnr.A1434","volume":"30","author":"K Kollia","year":"2009","unstructured":"Kollia K, Maderwald S, Putzki N, et al. First clinical study on ultra-high-field MR imaging in patients with multiple sclerosis: comparison of 1.5T and 7T. AJNR Am J Neuroradiol. 2009;30:699\u2013702. https:\/\/doi.org\/10.3174\/ajnr.A1434.","journal-title":"AJNR Am J Neuroradiol"},{"key":"1171_CR10","doi-asserted-by":"publisher","first-page":"900","DOI":"10.1002\/acn3.572","volume":"5","author":"JR Behrens","year":"2018","unstructured":"Behrens JR, Wanner J, Kuchling J, et al. 7 Tesla MRI of Balo\u2019s concentric sclerosis versus multiple sclerosis lesions. Ann Clin Transl Neurol. 2018;5:900\u201312. https:\/\/doi.org\/10.1002\/acn3.572.","journal-title":"Ann Clin Transl Neurol"},{"key":"1171_CR11","doi-asserted-by":"publisher","first-page":"e171","DOI":"10.1212\/NXI.0000000000000171","volume":"2","author":"T Sinnecker","year":"2015","unstructured":"Sinnecker T, Othman J, K\u00fchl M, et al. 7T MRI in natalizumab-associated PML and ongoing MS Disease activity: a case study. Neurol Neuroimmunol Neuroinflammation. 2015;2:e171. https:\/\/doi.org\/10.1212\/NXI.0000000000000171.","journal-title":"Neurol Neuroimmunol Neuroinflammation"},{"key":"1171_CR12","doi-asserted-by":"publisher","first-page":"389","DOI":"10.1007\/s10334-016-0548-1","volume":"29","author":"B Chen","year":"2016","unstructured":"Chen B, Schoemberg T, Kraff O, et al. Cranial fixation plates in cerebral magnetic resonance imaging: a 3 and 7 Tesla in vivo image quality study. Magma N Y N. 2016;29:389\u201398. https:\/\/doi.org\/10.1007\/s10334-016-0548-1.","journal-title":"Magma N Y N"},{"key":"1171_CR13","doi-asserted-by":"publisher","first-page":"636","DOI":"10.3174\/ajnr.A4569","volume":"37","author":"T Matsushige","year":"2016","unstructured":"Matsushige T, Chen B, Ringelstein A, et al. Giant intracranial aneurysms at 7T MRI. AJNR Am J Neuroradiol. 2016;37:636\u201341. https:\/\/doi.org\/10.3174\/ajnr.A4569.","journal-title":"AJNR Am J Neuroradiol"},{"key":"1171_CR14","doi-asserted-by":"publisher","first-page":"2505","DOI":"10.1161\/STROKEAHA.120.030590","volume":"51","author":"J Rodemerk","year":"2020","unstructured":"Rodemerk J, Junker A, Chen B, et al. Pathophysiology of intracranial aneurysms: COX-2 expression, Iron Deposition in Aneurysm Wall, and correlation with magnetic resonance imaging. Stroke. 2020;51:2505\u201313. https:\/\/doi.org\/10.1161\/STROKEAHA.120.030590.","journal-title":"Stroke"},{"key":"1171_CR15","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, et al. 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. 2016;12:714\u201322. https:\/\/doi.org\/10.1038\/nrneurol.2016.166.","journal-title":"Nat Rev Neurol"},{"key":"1171_CR16","doi-asserted-by":"publisher","first-page":"346","DOI":"10.1097\/RLI.0b013e31824600e9","volume":"47","author":"T Kober","year":"2012","unstructured":"Kober T, Granziera C, Ribes D, et al. MP2RAGE multiple sclerosis magnetic resonance imaging at 3 T. Invest Radiol. 2012;47:346\u201352. https:\/\/doi.org\/10.1097\/RLI.0b013e31824600e9.","journal-title":"Invest Radiol"},{"key":"1171_CR17","doi-asserted-by":"publisher","first-page":"1057","DOI":"10.1177\/1352458520943785","volume":"27","author":"F Guisset","year":"2021","unstructured":"Guisset F, Lolli V, Bugli C, et al. The central vein sign in multiple sclerosis patients with vascular comorbidities. Mult Scler Houndmills Basingstoke Engl. 2021;27:1057\u201365. https:\/\/doi.org\/10.1177\/1352458520943785.","journal-title":"Mult Scler Houndmills Basingstoke Engl"},{"key":"1171_CR18","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1186\/s13167-015-0038-y","volume":"6","author":"T Sinnecker","year":"2015","unstructured":"Sinnecker T, Kuchling J, Dusek P, et al. Ultrahigh field MRI in clinical neuroimmunology: a potential contribution to improved diagnostics and personalised Disease management. EPMA J. 2015;6:16. https:\/\/doi.org\/10.1186\/s13167-015-0038-y.","journal-title":"EPMA J"},{"key":"1171_CR19","doi-asserted-by":"publisher","first-page":"1223","DOI":"10.3174\/ajnr.A4729","volume":"37","author":"S Chawla","year":"2016","unstructured":"Chawla S, Kister I, Wuerfel J, et al. Iron and non-iron-related characteristics of multiple sclerosis and Neuromyelitis Optica Lesions at 7T MRI. Am J Neuroradiol. 2016;37:1223\u201330. https:\/\/doi.org\/10.3174\/ajnr.A4729.","journal-title":"Am J Neuroradiol"},{"key":"1171_CR20","doi-asserted-by":"publisher","first-page":"669","DOI":"10.1002\/ana.23959","volume":"74","author":"M Absinta","year":"2013","unstructured":"Absinta M, Sati P, Gait\u00e1n MI, et al. Seven-tesla phase imaging of acute multiple sclerosis lesions: a new window into the inflammatory process. Ann Neurol. 2013;74:669\u201378. https:\/\/doi.org\/10.1002\/ana.23959.","journal-title":"Ann Neurol"},{"key":"1171_CR21","doi-asserted-by":"publisher","first-page":"812","DOI":"10.1001\/archneurol.2010.148","volume":"67","author":"D Pitt","year":"2010","unstructured":"Pitt D, Boster A, Pei W, et al. Imaging cortical lesions in multiple sclerosis with ultra-high-field magnetic resonance imaging. Arch Neurol. 2010;67:812\u20138. https:\/\/doi.org\/10.1001\/archneurol.2010.148.","journal-title":"Arch Neurol"},{"key":"1171_CR22","doi-asserted-by":"publisher","first-page":"877","DOI":"10.1002\/acn3.51338","volume":"8","author":"KM Gillen","year":"2021","unstructured":"Gillen KM, Mubarak M, Park C, et al. QSM is an imaging biomarker for chronic glial activation in multiple sclerosis lesions. Ann Clin Transl Neurol. 2021;8:877\u201386. https:\/\/doi.org\/10.1002\/acn3.51338.","journal-title":"Ann Clin Transl Neurol"},{"key":"1171_CR23","doi-asserted-by":"publisher","first-page":"e994","DOI":"10.1212\/NXI.0000000000000994","volume":"8","author":"N Yahyavi-Firouz-Abadi","year":"2021","unstructured":"Yahyavi-Firouz-Abadi N, Kiczek M, Zeiler SR, Wasserman BA. Imaging features of Susac Syndrome on High-Resolution Intracranial Vessel Wall MRI. Neurol Neuroimmunol Neuroinflammation. 2021;8:e994. https:\/\/doi.org\/10.1212\/NXI.0000000000000994.","journal-title":"Neurol Neuroimmunol Neuroinflammation"},{"key":"1171_CR24","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1007\/s10072-020-04562-8","volume":"42","author":"J Baskin","year":"2021","unstructured":"Baskin J, Hardy TA, Law LY, et al. Black blood MRI: endotheliopathy of Susac syndrome unmasked. Neurol Sci off J Ital Neurol Soc Ital Soc Clin Neurophysiol. 2021;42:325\u20137. https:\/\/doi.org\/10.1007\/s10072-020-04562-8.","journal-title":"Neurol Sci off J Ital Neurol Soc Ital Soc Clin Neurophysiol"},{"key":"1171_CR25","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, et al. Evaluation of the Central Vein Sign as a diagnostic imaging biomarker in multiple sclerosis. JAMA Neurol. 2019;76:1446\u201356. https:\/\/doi.org\/10.1001\/jamaneurol.2019.2478.","journal-title":"JAMA Neurol"},{"key":"1171_CR26","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1002\/ana.25146","volume":"83","author":"P Maggi","year":"2018","unstructured":"Maggi P, Absinta M, Grammatico M, et al. Central vein sign differentiates multiple sclerosis from central nervous system inflammatory vasculopathies. Ann Neurol. 2018;83:283\u201394. https:\/\/doi.org\/10.1002\/ana.25146.","journal-title":"Ann Neurol"},{"key":"1171_CR27","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1007\/s11940-018-0504-7","volume":"20","author":"T Sinnecker","year":"2018","unstructured":"Sinnecker T, Granziera C, Wuerfel J, Schlaeger R. Future brain and Spinal Cord Volumetric Imaging in the clinic for monitoring treatment response in MS. Curr Treat Options Neurol. 2018;20:17. https:\/\/doi.org\/10.1007\/s11940-018-0504-7.","journal-title":"Curr Treat Options Neurol"},{"key":"1171_CR28","doi-asserted-by":"publisher","first-page":"1271","DOI":"10.1016\/j.neuroimage.2009.10.002","volume":"49","author":"JP Marques","year":"2010","unstructured":"Marques JP, Kober T, Krueger G, et al. MP2RAGE, a self bias-field corrected sequence for improved segmentation and T1-mapping at high field. NeuroImage. 2010;49:1271\u201381. https:\/\/doi.org\/10.1016\/j.neuroimage.2009.10.002.","journal-title":"NeuroImage"}],"container-title":["BMC Medical Imaging"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12880-023-01171-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12880-023-01171-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12880-023-01171-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T12:12:14Z","timestamp":1704197534000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcmedimaging.biomedcentral.com\/articles\/10.1186\/s12880-023-01171-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,2]]},"references-count":28,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["1171"],"URL":"https:\/\/doi.org\/10.1186\/s12880-023-01171-7","relation":{},"ISSN":["1471-2342"],"issn-type":[{"value":"1471-2342","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,2]]},"assertion":[{"value":"18 June 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 December 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 January 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was performed in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki Declaration. The ethics committee of University Duesburg Essen approved the study. All study participants, or their legal guardian, provided informed written consent to study enrollment.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"All study participants, or their legal guardian, provided informed written consent to publication of study data and MRI scans.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no conflict of interest related to the current study.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"4"}}