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Central intra-lesional iron deposits as a possible novel imaging marker at 7 Tesla MRI in Susac Syndrome - an exploratory study

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Item Type:Article
Title:Central intra-lesional iron deposits as a possible novel imaging marker at 7 Tesla MRI in Susac Syndrome - an exploratory study
Creators Name:Strunk, D. and Sinnecker, T. and Kleffner, I. and Doerr, J. and Ringelstein, M. and Gross, C.C. and Deuschl, C. and Maderwald, S. and Quick, H.H. and Yamac, E. and Wrede, K.H. and Kraemer, M.
Abstract:BACKGROUND: 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. METHODS: 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 ("iron dots"). Seven T MRI datasets from the same institute, comprising 75 patients with, among others, MS, served as controls. RESULTS: The "iron dot" 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. CONCLUSION: We are the first to demonstrate this type of "iron dot" 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.
Keywords:Susac Syndrome, 7 Tesla MRI, Central Vein Sign, T1 Hypointense Lesions, “Iron Dot” Lesions, Imaging Marker
Source:BMC Medical Imaging
Publisher:BioMed Central
Page Range:4
Date:2 January 2024
Official Publication:https://doi.org/10.1186/s12880-023-01171-7
PubMed:View item in PubMed

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