Department of Neurology, Girona Biomedical Research Institute (IDIBGI), Dr. Josep Trueta University Hospital, Avda. França s/n, 17007, Girona, Spain.
Neurol Sci. 2012 Dec;33(6):1407-10. doi: 10.1007/s10072-012-1023-z. Epub 2012 Mar 28.
Susac's syndrome is a rare idiopathic microangiopathy affecting the precapillary arterioles of the brain, retina and cochlea leading to the clinical triad of encephalopathy, retinopathy and hearing loss. The objective of this study is to describe a new case of Susac's syndrome reactivated after a 12-year period with a good response to immunosuppressive therapy. The patient was a 32-year-old woman, complaining of diplopia, right blurred vision, progressive gait disturbance, tinnitus, attention deficit, and slight memory loss. The patient was diagnosed as having Susac's syndrome and discharged with steroid therapy. After a 12-year period of clinical stability she had a relapse. Immunosuppressive therapy resulted in significant clinical and radiological improvement. Early clinical identification of Susac's syndrome is crucial for the initiation of immunosuppressive therapy and differential diagnosis. In our case, the combined use of corticosteroids and azathioprine was key in the relapse management.
舒塞氏综合征是一种罕见的特发性微动脉病，影响大脑、视网膜和耳蜗的小动脉前毛细血管，导致脑病、视网膜病和听力损失三联征。本研究的目的是描述一例舒塞氏综合征新病例，该病例在 12 年后因免疫抑制治疗而再次激活，且治疗反应良好。患者为 32 岁女性，主诉复视、右眼视力模糊、进行性步态障碍、耳鸣、注意力缺陷和轻微记忆力减退。该患者被诊断为舒塞氏综合征，并接受类固醇治疗出院。12 年后，患者病情稳定，但又复发。免疫抑制治疗后，患者的临床和影像学症状明显改善。早期识别舒塞氏综合征对于启动免疫抑制治疗和鉴别诊断至关重要。在我们的病例中，皮质类固醇和硫唑嘌呤的联合使用是复发管理的关键。
