Objective: To observe the effect of intravenous methylprednisolone pulse (IVMP) therapy on the recovery of visual acuity and its influencing factors in patients with the first attack of optic neuritis associated with aquaporin-4(AQP4) antibody seropositive neuromyelitis optica. Methods: Retrospective case series study. A total of 165 eyes of 120 patients diagnosed as optic neuritis related to neuromyelitis optica for the first time in the Neuro-ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to December 2017 were selected in this research. All patients had AQP4 antibody seropositivity and clinical manifestations of acute optic neuritis, excluding other diagnoses. All the patients received the treatment of IVMP 500 mg/d or 1 000 mg/d for 3 days, followed by a slowly tapering course of oral glucocorticoids. The post-treatment and pre-treatment visual acuities were compared. Improving four lines or more was considered as effective markedly, improving two or three lines as effective, and improving one line or no change or a decline as no effect. The onset age, visual acuity before treatment and doses in the acute exacerbation were analyzed. The Mann-Whitney U test and Kruskal-Wallis test were used for statistical analyses. Results: Among the 120 patients, there were 17 males and 103 females, with age ranging from 16 to 80 years (median, 44 years). There were 17.6% (29/165) of the eyes with conspicuous therapy, 33.3% (55/165) of the eyes with effective therapy and 49.1% (81/165) of the eyes with ineffective therapy. The effect of IVMP decreased obviously when the age of onset was over 50 years old [41.1%(23/56) vs. 56.0%(61/109), Z=2.645, P=0.008]. Patients with no light perception and light perception before treatment had better therapeutic effect than those with counting fingers-0.3 before treatment [72.2%(26/36), 72.7%(24/33) vs. 30.1%(25/83), Z=2.726, 2.967; P=0.006, 0.003]. Although the efficacy of patients with visual acuity of onset over 0.3 (9/13) was better than patients with counting fingers-0.3, but the difference was not statistically significant (Z=1.743, P=0.081). Different doses, including IVMP 1 000 mg/d and 500 mg/d, had no significant difference in the effect (Z=1.115, P=0.265). Conclusions: IVMP therapy is only valid for a half of eyes with optic neuritis associated with AQP4 antibody seropositive neuromyelitis optica. The effect of IVMP treatment at doses of 500 mg/d and 1 000 mg/d is similar. Furthermore, the visual acuity from finger counting to 0.3 and age of onset over 50 years old have an influence on the treatment effect. (Chin J Ophthalmol, 2019, 55: 180-185).
目的： 观察静脉注射甲泼尼龙冲击（IVMP）治疗对水通道蛋白4（AQP4）抗体阳性视神经脊髓炎相关视神经炎（NMO-ON）首次发病患者视力恢复的效果及其影响因素。 方法： 回顾性系列病例研究。选取2012年9月至2017年12月在首都医科大学附属北京同仁医院神经眼科门诊确诊为NMO-ON的120例（165只眼）首次发病患者。所有患者AQP4抗体检测均为阳性，具有急性视神经炎的临床表现，排除其他诊断。均予500 mg/d或1 000 mg/d的IVMP治疗3 d，口服糖皮质激素序贯减量。治疗后1~2周视力较治疗前提高4行及以上为显效；提高2~3行为有效；变化在1行内或无变化或下降为无效。分析发病时年龄、治疗前视力以及糖皮质激素冲击剂量对NMO-ON急性发作期治疗效果的影响。采用Mann-Whitney U检验、Kruskal-Wallis检验进行统计学分析。 结果： 120例患者中，男性17例，女性103例；发病年龄16~80岁，中位年龄44岁。在发病的165只眼中，治疗显效占17.6%（29/165），治疗有效占33.3%（55/165），治疗无效占49.1%（81/165）。发病年龄>50岁的患者与发病年龄≤50岁患者IVMP治疗效果差异有统计学意义[有效率41.1%（23/56）与56.0%（61/109）比较，Z=2.645，P=0.008]。治疗前视力为无光感和光感患眼的治疗效果优于治疗前视力为指数至0.3的患眼[有效率72.2%（26/36）、72.7%（24/33）与30.1%（25/83）比较，Z=2.726、2.967，P=0.006、0.003]，差异均具有统计学意义；视力大于0.3的患者虽然治疗效果（有效比例9/13）也优于治疗前视力为指数至0.3的患者，但差异无统计学意义（Z=1.743，P=0.081）。IVMP剂量采用500 mg/d的患者与采用1 000 mg/d的患者比较，治疗效果差异无统计学意义（Z=1.115，P=0.265）。 结论： IVMP治疗仅对半数AQP4抗体阳性NMO-ON患眼有效，采用剂量为500 mg/d与1 000 mg/d的IVMP治疗效果相近。IVMP治疗对发病年龄大于50岁的患者和治疗前视力为指数至0.3的患者效果更差。（中华眼科杂志，2019，55：180-185）.
Keywords: Aquaporin 4; Injections, intravenous; Methylprednisolone; Neuromyelitis optica; Optic neuritis.