Supplementary Material for: Vision restoration of steroid-refractory prelaminar optic nerve inflammation with plasmapheresis: report of two cases and literature review

Introduction: Inflammation of the prelaminar compartment of optic nerve involves the most anterior segment of the nerve. It can occur in isolation, in the setting of immunological or demyelinating disorders. Case presentation: We present two 32 and 41 year old female patients with sequential bilateral isolated prelaminar optic neuritis with atypical features. In both instances, plasma exchange (PLEX) was initiated for the second affected eye after corticosteroids proved ineffective, resulting in excellent visual recovery. Both patients lost vision in the fellow eye due to a similar attack that was also refractory to steroids. Our cases did not show any associated demyelinating lesions in imaging and did not show biomarkers such as Aquaporin-4 or Myelin Oligodendrocyte Glycoprotein (MOG) antibody in serum workup. They were followed for more than 5 years with stable vision in the only eye. Conclusion: In cases of isolated severe progressive prelaminar optic nerve inflammation refractory to steroid treatment, PLEX can be helpful, even if workup is unremarkable for neuromyelitis optica or other systemic and neurologic conditions.引言：视神经板前间隙（prelaminar compartment of optic nerve）的炎症累及视神经最前端节段。该炎症可单独发作，亦可伴发于免疫性疾病或脱髓鞘疾病背景下。 病例报告：本文报告2例女性患者，年龄分别为32岁与41岁，均罹患双侧先后发作的孤立性非典型板前视神经炎（prelaminar optic neuritis）。两例患者均在糖皮质激素治疗无效后，对第二只受累眼启动血浆置换（plasma exchange, PLEX）治疗，最终获得极佳的视力恢复。两名患者的对侧眼均因类似的、对类固醇难治的发作而丧失视力。本研究病例的影像学检查未发现伴发脱髓鞘病灶，血清学检测也未检出水通道蛋白4（Aquaporin-4）、髓鞘少突胶质细胞糖蛋白（Myelin Oligodendrocyte Glycoprotein, MOG）抗体等生物标志物。对患者进行了5年以上的随访，其仅存的患眼视力保持稳定。 结论：对于孤立性、病情呈进行性加重且糖皮质激素治疗难治的板前段视神经炎症病例，即使视神经脊髓炎（neuromyelitis optica）或其他系统性、神经系统疾病的相关检查结果无异常，血浆置换（PLEX）仍可发挥积极治疗作用。