中文摘要：

目的 分析视神经脊髓炎（NMO）首次发作时脊髓、视神经损害的表现,探讨NMO的首发高危脱髓鞘事件.方法 纳入2010年10月-2012年10月在首都医科大学宣武医院神经内科住院并确诊的51例以脊髓炎或视神经炎为首发事件的NMO病例,收集患者首次脱髓鞘事件的临床表现、脊髓及颅脑MRI检查结果等资料,进行分析.结果 （1）26例（51.0％）NMO病例以脊髓炎为首发事件,其中30.8％ （8/26）为长节段横贯性脊髓炎（LETM）,69.2％ （18/26）为非LETM（包括短节段脊髓炎和非横贯性脊髓炎）,非LETM比LETM多见;非LETM为首发事件病例的首次治疗后转归好于LETM为首发事件病例（完全恢复的比例13/18比2/8,P＜0.05）;与LETM病例相比,非LETM病例脊髓炎复发间隔期短、疾病复发频率高（11.1个月比18.6个月;3次/年比1次/年;P均＜0.05）.（2）25例（49.0％）NMO病例以视神经炎为首发事件,24.0％（6/25）为单眼视神经炎,76.0％（19/25）为双眼视神经炎,双眼视神经炎较单眼视神经炎多见;双眼视神经炎病例首次发病的视力损害重于单眼视神经炎病例,首次缓解期持续时间短（P＜0.05）.结论 非LETM及双眼视神经炎是NMO在首次发病时最常见的临床高危脱髓鞘事件.

英文摘要：

Objective To explore the clinical characteristics of the lesions of spinal cord and optic nerve as the onset of neuromyelitis optica （NMO）.Methods A total of fifty-one patients with myelitis or optic neuritis （ON） as the onset of NMO who hospitalized in our Neurology Department during October 2010 to October 2012 were enrolled in the study.Clinical presentations and MRI findings of the spinal cord and brain were studied.Results （1） A total of 26 cases （51.0%） presented with myelitis as the index event,in which 30.8% （8/26） were the longitudinally extensive transverse myelitis （LETM） and 69.2% （18/ 26） were non-LETM （short segmental myelitis or non-transverse myelitis）.Patients with non-LETM as the onset were found to have better prognosis than those with LETM （full recovery ratio was 13/18 vs 2/8,P 〈 0.05）,while shorter recurrence interval of myelitis and higher recurrence frequency of events were shown in patients with non-LETM （11.1 vs 18.6 months,3 times per year vs once per year,with all P 〈 0.05）.（2） A total of 25 cases （49.0%） presented with ON as the index event with 24.0% （6/25） of unilateral ON and 76.0% （19/25） of bilateral ON.Patients with bilateral ON had more severe visual impairment and shorter first remission period than those with unilateral ON （P 〈 0.05）.Conclusion Non-LETM and bilateral ON are the most common index demyelinating events in NMO cases.