Leptomeningeal enhancement on post-contrast FLAIR images for early diagnosis of Susac syndrome. (February 2022)

• Record Type: Journal Article
• Authors: Bellanger, Guillaume; Biotti, Damien; Adam, Gilles; Darcourt, Jean; Roques, Margaux; Patsoura, Sofia; Savatovsky, Julien; Obadia, Michael; Menjot de Champfleur, Nicolas; Charif, Mahmoud; Labauge, Pierre; Cotton, Francois; Durand Dubief, Françoise; Tourdias, Thomas; Dulau, Cecile; Kremer, Stéphane; De Sèze, Jérôme; Ciron, Jonathan; Varenne, Fanny; Viguier, Alain; Lerebours, Fleur; Larrue, Vincent; Cognard, Christophe; Bonneville, Fabrice
• Is Part Of: Multiple sclerosis. Volume 28:Number 2(2022)
• Page Start: 189
• Page End: 197
• Publication Date: 2022-02
• Subjects: Multiples sclerosis -- Susac syndrome -- post-contrast FLAIR -- leptomeningeal enhancement; Central nervous system -- Diseases -- Periodicals; Myelin sheath -- Diseases -- Periodicals; Inflammation -- Periodicals; Multiple sclerosis -- Periodicals; Central Nervous System Diseases -- Periodicals; Demyelinating Diseases -- Periodicals; Inflammation -- Periodicals; Multiple Sclerosis -- Periodicals; Système nerveux central -- Maladies -- Périodiques; Gaine de myéline -- Maladies -- Périodiques; Inflammation (Pathologie) -- Périodiques; Sclérose en plaques -- Périodiques; Electronic journals; 616.834005
• DOI: 10.1177/13524585211012349
• Languages: English
• ISSNs: 1352-4585

Abstract

Background: Leptomeningeal enhancement (LME) is a key feature of Susac syndrome (SuS) but is only occasionally depicted on post-contrast T1-weighted images (T1-WI). Objective: As post-contrast fluid-attenuated inversion recovery (FLAIR) may be more sensitive, our aim was to assess LME in SuS on this sequence. Methods: From 2010 to 2020, 20 patients with definite SuS diagnosis were retrospectively enrolled in this multicentre study. Two radiologists independently assessed the number of LME on post-contrast FLAIR and T1-WI acquisitions performed before any treatment. A chi-square test was used to compare both sequences and the interrater agreement was calculated. Results: Thirty-five magnetic resonance imagings (MRIs) were performed before treatment, including 19 post-contrast FLAIR images in 17 patients and 25 post-contrast T1-WI in 19 patients. In terms of patients, LME was observed on all post-contrast FLAIR, contrary to post-contrast T1-WI (17/17 (100%) vs. 15/19 (79%), p < 0.05). In terms of sequences, LME was observed on all post-contrast FLAIR, contrary to post-contrast T1-WI (19/19 (100%) vs. 16/25 (64%), p < 0.005). LME was disseminated at both supratentorial (19/19) and infratentorial (18/19) levels on post-contrast FLAIR, contrary to post-contrast T1-WI (3/25 and 9/25, respectively). Interrater agreement was excellent for post-contrast FLAIR (κ = 0.95) but only moderate for post-contrast T1-WI (κ = 0.61). Conclusion: LME was always observed and easily visible on post-contrast FLAIR images prior to SuS treatment. In association with other MRI features, it is highly indicative of SuS.