Gaudemer Augustin, Henry-Feugeas Marie-Cécile, Peyre Marion, Kachaner Alexandra, Klein Isabelle, Khalil Antoine, Papo Thomas, Sacré Karim
Department of Radiology, Hospital Bichat-Claude Bernard, Assistance Publique Hôpitaux de Paris, Université Paris Cité, Paris, France.
Department of Internal Medicine, Hospital Bichat-Claude Bernard, Assistance Publique Hôpitaux de Paris, Université Paris Cité, Paris, France.
Eur J Neurol. 2025 Jan;32(1):e70002. doi: 10.1111/ene.70002.
Susac syndrome (SuS) is a rare immune-mediated microangiopathy with potential disabling evolution. We aimed to analyze brain microstructural damage through diffusion tensor imaging (DTI) in SuS and determine its association with poor outcomes.
CarESS study is a prospective multicenter national cohort study of patients with SuS. Patients included at the principal investigator's center with at least two available brain magnetic resonance imaging (MRI) with DTI were analyzed. Mean diffusivity (MD) and fractional anisotropy (FA) were measured in fibers crossing three regions of interest (ROIs): the corpus callosum as a whole, the genu of the corpus callosum, and the splenium of the corpus callosum. The primary outcome was work resumption.
Twenty-two patients (36 (25;42) years, 16 (73%) females) were studied. The triad (i.e., brain, eye, and ear involvement) was complete in 21 (95%) patients. All but one patients received steroids alone or in combination with immunosuppressive drugs (n = 11) and/or IVIg (n = 7). Over a median follow-up of 6 (5;8) years, 15 (68%) patients went back to work. FA and MD were longitudinally measured in 123 DTI MRI accounting for a median of 5.6 [4.2; 7] MRI per patient. Microstructural damages in the corpus callosum as a whole, the genu of the corpus callosum, and the splenium of the corpus callosum increased during follow-up and were significantly associated with the inability to return to work.
Brain DTI identified microstructural damage in fibers crossing the corpus callosum that are associated with long-term disability in SuS.
ClinicalTrials.gov portal identifier: NCT01481662 (https://clinicaltrials.gov/ct2/show/NCT01481662?term=caress&draw=2&rank=5).
Susac综合征（SuS）是一种罕见的免疫介导性微血管病，病情可能会逐渐发展至致残。我们旨在通过扩散张量成像（DTI）分析SuS患者的脑微结构损伤，并确定其与不良预后的关联。
CarESS研究是一项针对SuS患者的前瞻性多中心全国队列研究。对主要研究者所在中心纳入的、至少有两次可用的脑磁共振成像（MRI）及DTI检查结果的患者进行分析。在穿过三个感兴趣区域（ROI）的纤维中测量平均扩散率（MD）和各向异性分数（FA），这三个区域分别为：整个胼胝体、胼胝体膝部和胼胝体压部。主要结局为恢复工作情况。
共研究了22例患者（年龄36（25；42）岁，16例（73%）为女性）。21例（95%）患者出现了三联征（即脑、眼和耳受累）。除1例患者外，所有患者均单独使用或联合使用了类固醇与免疫抑制药物（n = 11）和/或静脉注射免疫球蛋白（IVIg，n = 7）。在中位随访期6（5；8）年时，15例（68%）患者恢复了工作。在123次DTI MRI检查中纵向测量了FA和MD，每位患者的MRI检查中位数为5.6[4.2；7]次。在随访期间，整个胼胝体、胼胝体膝部和胼胝体压部的微结构损伤均有所增加，且与无法恢复工作显著相关。
脑DTI可识别穿过胼胝体的纤维中的微结构损伤，这些损伤与SuS患者的长期残疾有关。
ClinicalTrials.gov平台标识符：NCT01481662（https://clinicaltrials.gov/ct2/show/NCT01481662?term=caress&draw=2&rank=5）
