Abstract：
Susac syndrome (SuS) presents with encephalopathy, visual disturbances, and hearing loss from immune-mediated microvascular occlusion. While acute SuS is well-described, long-term cognitive outcomes with current treatments are underknown. We assessed ten SuS patients treated in accordance with evidence-based guidelines using immunotherapies targeting humoral and cell-mediated pathways. Patients were followed for a median 3.6 years. Initially, cognition inversely correlated with corpus callosum lesions on MRI. All reported cognitive improvement; 5/10 patients had residual deficits in visual attention and executive function. Early, aggressive treatment was associated with good outcomes; extensive early corpus callosum lesions may identify patients at-risk of persistent cognitive deficits.
摘要：
Susac综合征（SuS）表现为脑病，视觉障碍,免疫介导的微血管闭塞引起的听力损失。虽然急性苏斯被描述得很好，目前治疗的长期认知结果还不为人所知.我们评估了10名根据循证指南使用针对体液和细胞介导途径的免疫疗法治疗的SuS患者。患者的随访时间中位数为3.6年。最初，认知与MRI显示的call体病变呈负相关。所有患者均报告认知改善；5/10的患者在视觉注意力和执行功能方面存在残留缺陷。早期，积极的治疗与良好的结局相关;广泛的早期call体病变可能使患者存在持续性认知缺陷的风险.
