From the Neurology Department (S.C., R.D., O.G., C.S., J.A., M.O.).
Neuroradiology Department (A.L.. E.S., J.S., F.C.).
AJNR Am J Neuroradiol. 2019 Jul;40(7):1184-1190. doi: 10.3174/ajnr.A6103. Epub 2019 Jun 27.
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 fluid-attenuated inversion recovery MR imaging.
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.
Patients with Susac syndrome were significantly more likely to present with leptomeningeal enhancement: 5/9 (56%) versus 6/73 (8%) in the control group ( = .002). They had a significantly higher leptomeningeal enhancement burden with ≥3 lesions in 5/9 patients versus 0/73 ( < .001). Regions of leptomeningeal enhancement were significantly more likely to be located in the posterior fossa: 5/9 versus 0/73 ( < .001). Interobserver agreement for leptomeningeal enhancement was good (κ = 0.79). There was a significant association between clinical relapses and increase of both leptomeningeal enhancement and parenchymal lesion load: OR = 6.15 ( = .01) and OR = 5 ( = .02), respectively.
Leptomeningeal enhancement occurs frequently in Susac syndrome and could be helpful for diagnosis and in predicting clinical relapse.
软脑膜强化可见于多种神经系统疾病，如 Susac 综合征。我们的目的是使用 3T 对比后液体衰减反转恢复磁共振成像（FLAIR）评估软脑膜强化在 Susac 综合征中的患病率及其意义。
2011 年 1 月至 2017 年 12 月，连续纳入 9 例 Susac 综合征患者和 73 例多发性硬化症或临床孤立综合征患者作为对照组。2 位神经放射科医生在不了解临床和眼科数据的情况下独立审查 MRI，并评估软脑膜强化和脑实质异常。对 Susac 综合征患者的随访 MRI（每位患者平均 5.9 次 MRI，中位数随访时间为 46 个月）进行评估，并与独立眼科医生评估的临床和视网膜荧光素血管造影数据进行比较。采用 Fisher 检验比较两组，采用混合效应逻辑回归模型分析 Susac 综合征患者的临床和影像学随访结果。
Susac 综合征患者更易出现软脑膜强化：9 例患者中有 5 例（56%），对照组中有 6 例（8%）（ =.002）。5 例患者中软脑膜强化负荷≥3 个病变，明显高于对照组（0/73）（ <.001）。软脑膜强化区域更易位于后颅窝：9 例患者中有 5 例（56%），对照组中有 0 例（0%）（ <.001）。软脑膜强化的观察者间一致性良好（κ=0.79）。临床复发与软脑膜强化和脑实质病变负荷增加均显著相关：比值比（OR）=6.15（ =.01）和 OR=5（ =.02）。
软脑膜强化在 Susac 综合征中较为常见，有助于诊断和预测临床复发。
