46例视神经脊髓炎首次临床脱髓鞘事件分析

摘要

目的：分析视神经脊髓炎（ NMO）临床出现首次脱髓鞘事件时脊髓、视神经、脑损害的表现，探讨NMO首次临床事件的特征。方法纳入2010年1月至2015年6月在神经内科住院的46例NMO病例，收集患者首次发作时的临床表现、脊髓及颅脑、视神经核磁共振检查结果等资料，进行病例对照分析研究。结果（1）46例确诊NMO病例分别以视神经炎（ON，16／46，34．8％）、脊髓炎（25／46，54．3％）、ON合并脊髓炎（3／46，6．5％）、脑部损害（2／46，4．4％）为首次临床脱髓鞘事件。（2）NMO首次临床脱髓鞘事件中：双眼ON 病例的视神经损害及神经功能缺损程度重于单眼 ON 病例（严重视力损害比例为66．7％∶14．3％、EDSS评分为2．0∶1．0，P≤0．05），且双眼ON病例的首次缓解期持续时间短、短期预后差（18．0个月∶40．0个月，完全恢复比例为22．2％∶86．0％，P≤0．05）；脊髓炎事件中长节段脊髓损害多于短节段脊髓损害（68．0％∶32．0％），长节段横贯性脊髓炎（LETM）病例的神经功能缺损重、短期预后差于非LETM病例（EDSS评分为3．0∶1．2，完全恢复百分率为30．0％∶73．0％，P≤0．05），但非LETM病例的脊髓炎后缓解期短（10．1个月∶16．3个月，P≤0．05）、病例复发比例有增大趋势（60．0％∶30．0％）。结论视神经炎及脊髓炎是NMO病例最常见的临床首发脱髓鞘事件，也有个别病例以脑部损害为首发事件。首发事件中双侧ON及LETM病例的神经功能损害严重、短期预后不佳，要警惕非LETM病例发展为NMO的可能性。%Objective To analyze the clinical presentations of spinal cord, optic nerve and brain damages in the first demyelinating event of neuromyelitis optica ( NMO) , and to explore the characteristics of the first episode in NMO.Methods 46 cases were included from January 2010 to June 2015 in the Department of Neurology.The information of clinical presentations of first demyelinating events, spinal cord, brain and optic nerve MRI findings were studied.Results (1) In the 46 cases, 16 cases(34.8%) presented with optic neuritis (ON) as the first clinical demyelinating event, 25 cases(54.3%) were myelitis, 3 cases(6.5%) were ON combined with myelitis and 2 cases(4.4%) were brain damage.(2) In the first clinical demyelinating events in NMO, the optic nerve damages and neurological deficits in bilateral ON cases were much worse than that in unilateral ON cases(severe visual impairment ratio was 66.7% to 14.3%, EDSS score was 2.0 to 1.0), and first remission continued time in bilateral ON cases was shorter than that in unilateral ON cases (18.0 months to 40.0 months, complete recovery ratio was 22.2%to 86.0%, P≤0.05);In myelitis event, the damage of long spinal cord was more than that of short spinal cord (68.0%to 32.0%), the neurological deficits and short-term prognosis in LETM case was much worse than that in non-LETM cases ( EDSS score was 3.0 to 1.2, complete recovery percentage ratio was 30.0%to 73.0%, P≤0.05), but the remission time after myelitis in non-LETM cases was shorter (10.1 months to 16.3 months, P≤0.05), the proportion of recurrent tended to increase (60.0% to 30.0%) .Conclusions Optic neuritis and myelitis are the most common first demyelinating event in NMO cases, while brain damage is the first event in individual case.In the first demyelinating events, severe bilateral ON and LETM are often with worse neurological deficit and poor short-term prognosis, we should be alert to the possibility of non-LETM cases developing to NMO.