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：视神经板前间隙炎症累及视神经最前端节段。该炎症可单独发生，亦可继发于免疫性疾病或脱髓鞘疾病背景。
病例报告：本文报告2例分别为32岁、41岁的女性患者，均罹患序贯性双侧孤立性板前视神经炎，且表现为非典型临床特征。两例患者均在糖皮质激素治疗无效后，针对第二只受累眼启动血浆置换（PLEX）治疗，最终获得极佳的视力恢复。两名患者均因类似的激素抵抗性发作，导致对侧眼视力丧失。本研究纳入的病例影像学检查未发现伴随脱髓鞘病灶，血清学检测亦未检出水通道蛋白4（Aquaporin-4）、髓鞘少突胶质细胞糖蛋白（MOG）等生物标志物。患者均接受了超过5年的随访，仅剩的单眼视力维持稳定。
结论：对于孤立性、重症进展性且对糖皮质激素治疗抵抗的板前视神经炎症病例，即便针对视神经脊髓炎及其他系统性、神经系统疾病的排查结果无异常，血浆置换（PLEX）仍可发挥有效治疗作用。