BACKGROUND AND PURPOSE: Leptomeningeal enhancement can be found in a variety of neurologic diseases such as Susac Syndrome. Our aim was to assess its prevalence and significance of leptomeningeal enhancement in Susac syndrome using 3T postcontrast fluidattenuated inversion recovery MR imaging. MATERIALS AND METHODS: From January 2011 to December 2017, nine consecutive patients with Susac syndrome and a control group of 73 patients with multiple sclerosis or clinically isolated syndrome were included. Two neuroradiologists blinded to the clinical and ophthalmologic data independently reviewed MRIs and assessed leptomeningeal enhancement and parenchymal abnormalities. Follow-up MRIs (5.9 MRIs is the mean number per patient over a median period of 46 months) of patients with Susac syndrome were reviewed and compared with clinical and retinal fluorescein angiographic data evaluated by an independent ophthalmologist. Fisher tests were used to compare the 2 groups, and mixed-effects logistic models were used for analysis of clinical and imaging follow-up of patients with Susac syndrome. RESULTS: Patients with Susac syndrome were significantly more likely to present with leptomeningeal enhancement: 5/9 (56%) versus 6/73 (8%) in the control group (P ϭ .002). They had a significantly higher leptomeningeal enhancement burden with Ն3 lesions in 5/9 patients versus 0/73 (P Ͻ .001). Regions of leptomeningeal enhancement were significantly more likely to be located in the posterior fossa: 5/9 versus 0/73 (P Ͻ .001). Interobserver agreement for leptomeningeal enhancement was good (ϭ 0.79). There was a significant association between clinical relapses and increase of both leptomeningeal enhancement and parenchymal lesion load: OR ϭ 6.15 (P ϭ .01) and OR ϭ 5 (P ϭ .02), respectively. CONCLUSIONS: Leptomeningeal enhancement occurs frequently in Susac syndrome and could be helpful for diagnosis and in predicting clinical relapse. ABBREVIATIONS: CIS ϭ clinically isolated syndrome; CC ϭ corpus callosum; FA ϭ fluorescein angiography; LME ϭ leptomeningeal enhancement; pcFLAIR ϭ postcontrast FLAIR; SuS ϭ Susac syndrome S usac Syndrome (SuS) is a vasculopathy characterized by a triad of neurologic, hearing, and ophthalmologic disorders. 1-3 Fluorescein angiography (FA) typically shows branch retinal ar
Purpose. The recent use of “en-face” enhanced-depth imaging spectral-domain optical coherence tomography (EDI SD-OCT) helps distinguish the retinal layers involved in the physiopathology of multiple evanescent white dot syndrome (MEWDS). Methods. Four patients presenting with MEWDS underwent a comprehensive ocular examination including C-scan (“en-face”) EDI SD-OCT at the initial visit and during follow-up. Results. C-scans combined with the other multimodal imaging enabled the visualization of retinal damage. Acute lesions appeared as diffuse and focal disruptions occurring in the ellipsoid and interdigitation zones. The match between autofluorescence imaging, indocyanine green angiography, and “en-face” OCT helped identify the acute microstructural damages in the outer retina further than the choroid. Follow-up using “en-face” EDI-OCT revealed progressive and complete recovery of the central outer retinal layers. Conclusion. “En-face” EDI SD-OCT identified the site of initial damage in MEWDS as the photoreceptors and the interdigitation layers rather than the choroid. Moreover, “en-face” OCT is helpful in the follow-up of these lesions by being able to show the recovery of the outer retinal layers.
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