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目的 研究慢性复发性炎性视神经病(CRION)的临床特征和预后.方法 回顾性研究.收集北京同仁医院神经内科自2014年1月1日至2016年10月30日CRION患者27例,对其病程、视功能、发作次数、磁共振成像(MRI)表现、血液学特点等进行临床分析.结果 27例患者中男10例,女17例,年龄17 ～59(35.2±12.0)岁.病程最短40d,最长8年.发作2～9(3.30±1.56)次.发作次数≤2次与＞2次者视力预后差异有统计学意义(P=0.04).有25例患者MRI示视神经信号异常,其中22例为眶内段和(或)管内段信号异常,2例有颅内异常信号.5例患者抗核抗体(ANA)滴度升高.5例患者在激素减量或停药过程中复发但未及时加用激素,最终视力预后差.结论 CRION以中年女性多见;急性期多伴疼痛,病变以视神经眶内段为主;发作次数越多,视力损害越重;如果复发,应及时加用激素或免疫抑制剂以挽救视力.

Objective To explore the clinical characteristics and prognosis of chronic relapsing inflammatory optic neuropathy (CRION).Methods A retrospective analysis was conducted.Clinical features,disease course,prognosis and magnetic resonance imaging (MRI) of patients with CRION who were admitted to Department of Neurology between 2014 and 2016 were reviewed and analyzed.Results Totally,there were 27 patients (10 males and 17 females),with an age range of 17-59 years.The disease duration was between 40 days and 8 years.There were 2 to 9 CRION episodes.The mean frequency of CRION episodes was 3.30 ± 1.56.The outcome of visual acuity showed that the more episodes frequency was,the worse outcome became.There was 25 abnormal optic nerve signals in MRI.And in 22 cases,the abnormal signals were in intraorbital segment or inner pipe section.The antinuclear antibodies (ANA) titers were elevated in 5 patients.Five cases relapsed during reduction or withdrawal of steroids,but steroids was not added in time.The final outcome of these 5 patients was poor.Conclusions CRION was more common in female than male patients.Most patients were companied by pain,and the lesions were more common in the intraorbital segment of optic nerve.The more episode frequency was,the worse prognosis became.If a relapse happened,steroids or other immunosuppressive agents should be used.