视神经脊髓炎谱系疾病相关视神经炎是一种累及视神经的脱髓鞘性炎症疾病，视力损伤严重，预后差，复发率高。及时控制急性发作和有效预防复发是治疗的关键。目前治疗主要包括糖皮质激素、血浆置换、免疫吸附、免疫抑制剂、靶向单抗类药物。特别是近年来依库丽单抗、萨特利珠单抗、及依那利珠单抗取得重大进展。该文综述视神经脊髓炎谱系疾病相关视神经炎近年治疗研究进展，期望为临床决策提供有益参考。
Neuromyelitis optica spectrum disorders (NMOSD) is a central nervous system inflammatory demyelinating disease with involvement of the optic nerve and spinal cord, with poor prognosis and high recurrence rate. Timely control of acute attacks and effective prevention of recurrence are the keys to treatment. This article reviews the recent research progress in the treatment of optic neuritis associated with NMOSD , hoping to provide useful references for clinical decision-making.
目的：探讨蛋白A免疫吸附联合糖皮质激素治疗对MOG抗体相关视神经炎（MOG antibody-associated optic neuritis， MOG-ON）患者的临床疗效及安全性。方法：回顾性分析2022年6月—2024年12月在广东三九脑科医院神经内科确诊并接受蛋白A免疫吸附联合糖皮质激素治疗的7例MOG-ON患者。所有患者均接受蛋白A免疫吸附治疗（隔天1次，共5次为1个疗程）并同期联合糖皮质激素治疗。评估治疗前及治疗后3个月、6个月的视力变化、扩展伤残状态量表（expanded disability status scale，EDSS）评分变化及MOG抗体滴度变化，并记录不良反应。结果：治疗后6个月，6/7患者视力较治疗前改善，其中4/7视力改善显著。左眼LogMAR视力值从治疗前的0.20(0.14，0.70)改善至0.10(0.10，0.42)，右眼LogMAR视力值从0.30(0.19，0.47)改善至0.18(0.10，0.21)，EDSS视力评分从2.86±1.68降至1.43±1.51（P < 0.05）。治疗前血清MOG抗体滴度几何平均数为1:52.0（几何标准差GSD = 3.7），治疗后3个月降至1:8.8（GSD = 1.9）（P = 0.027），治疗后6个月降至1:13.0（GSD = 4.1）（P = 0.027）。7例患者共接受35次免疫吸附治疗，未观察到严重不良反应，仅有轻微可控的不良事件。结论：蛋白A免疫吸附联合糖皮质激素治疗能够有效降低血液中MOG抗体水平，改善MOG-ON患者的视力。
Objective: To investigate the clinical efficacy and safety of protein A immunoadsorption combined with glucocorticoid therapy in patients with myelin oligodendrocyte glycoprotein antibody-associated optic neuritis（MOG-ON）. Methods: A retrospective analysis was conducted on 7 patients with MOG-ON who were diagnosed and treated with protein A immunoadsorption combined with glucocorticoid therapy at the Department of Neurology，Guangdong Sanjiu Brain Hospital from June 2022 to December 2024. All patients underwent protein A immunoadsorption therapy （once every other day, with 5 sessions constituting one course） in conjunction with concurrent steroid therapy. Visual acuity changes, EDSS score changes, and MOG antibody titer changes were assessed before treatment, as well as at 3 and 6 months after treatment. Additionally, adverse events were meticulously recorded. Results: At the 6 months post-treatment mark, 6 patients （85.7%） demonstrated an improvement in visual acuity compared to their baseline levels, with 4 patients （57.1%） achieving a significant improvement. The median LogMAR visual acuity values in the left eye improved from 0.20(0.14,0.70) to 0.10(0.10,0.42), and in the right eye, they improved from 0.30(0.19,0.47) to 0.18(0.10,0.21). MeanWhile, the EDSS visual score decreased from 2.86±1.68 to 1.43±1.51（P < 0.05）. The geometric mean serum MOG antibody titer declined from 1:52.0（GSD = 3.7） before treatment to 1:8.8（GSD = 1.9） at 3 months after treatment（P = 0.027）, and further decreased to 1:13.0（GSD = 4.1） at 6 months after treatment（P = 0.027）. A total of 35 immunoadsorption sessions were administered to the 7 patients, and no serious adverse reactions were observed; only minor and manageable adverse events occurred. Conclusion: Protein A immunoadsorption combined with glucocorticoid therapy can effectively lower serum MOG antibody levels and enhance visual outcomes in patients with MOG-ON.
视神经炎(optic neuritis,ON)是指视神经的炎性脱髓鞘病变，是引起中青年人视力下降的主要原因。近年来，髓鞘少突胶质细胞糖蛋白抗体阳性视神经炎(myelin oligodendrocyte glycoprotein antibody-positive ON,MOG-ON)成为神经眼科领域的研究热点，国内外报道不断增加。2021年3月，中华医学会眼科学分会神经眼科学组制定了《中国脱髓鞘性视神经炎诊断和治疗循证指南(2021年)》，将MOG-ON作为新的视神经炎亚型纳入脱髓鞘性视神经炎的诊疗体系，给广大眼科医生提供了新的参考依据。因此，临床医生需要充分认识MOG抗体相关疾病和MOG-ON的临床特征和治疗进展，努力提高其诊断和治疗水平，使此类患者能够得到更多的获益，造福于更多的视神经疾病患者。
Optic neuritis(ON)is an inflammatory demyelinating disease of the optic nerve, which is the main cause of vision loss in young and middle-aged people. In recent years, myelin oligodendrocyte glycoprotein antibody-positive ON(MOG-ON)has become a research hotspot in the field of neuro-ophthalmology, and reports at home and abroad are increasing.In March 2021, the Neuro-ophthalmology Group of Ophthalmology Branch of Chinese Medical Association formulated the Evidence-Based Guidelines for the Diagnosis and Treatment of Demyelinating Optic Neuritis in China(2021) , and included MOG-ON as a new optic neuritis subtype in the diagnosis and treatment system of myelinating optic neuritis providing a new reference for the majority of ophthalmologists. Therefore, clinicians need to fully understand the clinical features and treatment progress of MOG antibody-related diseases and MOG-ON, and strive to improve the level of diagnosis and treatment, so that such patients can get more benefits and benefit more patients with optic nerve diseases.
非器质性视力下降也称为心因性或功能性视力下降，除视力下降外，还可伴有视野缺损，多由于精神心理疾患导致的转换障碍引起，部分患者为诈病以获取利益。本文报道1例6岁的女性患者，主诉双眼反复视力下降1年余，早期被误诊为儿童视神经炎，给予糖皮质激素冲击治疗，治疗后稍有好转。通过本例患者误诊的教训，提醒我们在遇到儿童出现不明原因的视力下降时，在没有明确器质性疾病证据时要想到非器质性视力下降的可能，掌握识别非器质性视力下降的检查方法，不能忽略相对性传入性瞳孔障碍等基础的神经眼科检查。
Non-organic vision loss is also known as psychogenic or functional vision loss. In addition to vision loss, it can also be accompanied by visual field defect. It is mostly caused by conversion obstacles caused by mental and psychological diseases. Some patients cheat to obtain benefits. This paper reports a 6-year-old female patient who complained of repeated visual acuity decline for more than one year. She was misdiagnosed as pediatric optic neuritis in the early stage and was treated with glucocorticoid shock therapy, which her condition improved slightly after treatment. The misdiagnosis of this patient teaches us that when children have unexplained visual acuity decline, we should think of the possibility of non-organic visual acuity decline when there is no clear evidence of organic diseases, master the examination methods to identify non-organic visual acuity decline, and cannot ignore the basic neuro-ophthalmic examination such as relative afferent pupillary defect (RAPD).
视神经脊髓炎相关性视神经炎(neuromyelitis optica spectrum disorder optic neuritis，NMO-ON)是一种常见的视神经炎(optic neuritis，ON)类型。女性非白种人占优势，损伤严重，双侧受累较多，视力预后差。我国有很大部分特发性ON最终诊断为NMO-ON。在相关实验室、光学相干断层扫描(optical coherence tomography，OCT)、磁共振(magnetic resonance imaging，MRI)等技术支持下，目前对NMO-ON的认识有了很大的进步，治疗方式除了皮质类固醇外还有免疫球蛋白、血浆置换及免疫抑制剂等。但提高NMO-ON的诊疗水平还有很长的路，更好地认识NMO-ON有助于更快速的诊断、更规范的治疗、更良好的预后。我们可以联合神经科开展多中心大样本量前瞻性的临床对照研究。
Neuromyelitis optica spectrum disorder- optic neuritis (NMO-ON) is a common type of optic neuritis (ON). This affliction is predominant in female non-Caucasians, with severe injury, more bilateral involvement, and poor visual prognosis. In China, a large proportion of idiopathic ON is ultimately diagnosed as NMO-ON. Our understanding of NMO-ON has made great progress under the technical support, such as the relevant laboratory, optical coherence tomography (OCT), magnetic resonance imaging (MRI). In addition to corticosteroids, immunoglobulin, plasmapheresis and immunosuppressive agents are also available for treatment. However, there is still a long way to improve the diagnosis and treatment level of NMO-ON. A better understanding of NMO-ON contributes to faster diagnosis, more standardized treatment, and better prognosis. We should cooperate with the neurology department to conduct a multi-center, large sample size prospective clinical control study.
