复发型视神经脊髓炎的临床特征与视功能分析

Clinical features and visual function of recurrent neuromyelitis optica

【摘要】目的观察复发型视神经脊髓炎（NMO）患者的临床特征及视功能改变。方法回顾性病例系列研究。临床确诊为复发型NMO的34例患者纳入研究。其中，男性2例，女性32例，男女性别比例为1：16；平均首次发病年龄（35．03±14．56）岁；平均复发次数（4．24±2．45）次。所有患者视神经炎（ON）复发次数为2～12次。其中，复发2次者10例15只眼，复发≥3次者24例37只眼。患者行视力、直接检眼镜、裂隙灯显微镜联合前置镜、视野及视觉诱发电位（VEP）检查。观察患者的临床表现，分析ON首次发病患者与ON复发患者的视神经、视野及VEP异常率。以视神经萎缩、视盘水肿为视神经异常；视野敏感度普遍下降，中心及旁中心暗点、环形暗点，半视野缺损，管状视野，视野向心性缩小为视野异常；P，。波潜伏期延长和（或）振幅下降为VEP异常。同时采用间接免疫荧光法对患者行血清NMO-免疫球蛋白G（IgG）检测，观察其血清NMO—IgG阳性情况。结果所有患者均表现为ON和脊髓炎反复发作。ON主要表现为视力下降；脊髓炎主要表现为感觉障碍、运动障碍及膀胱直肠功能障碍。视力检查发现，ON首次发病、ON复发2次及ON复发≥3次患者的致盲率分别为41．67％、33．33％、64．86％。ON复发2次者致盲率较ON首次发病者升高，但差异无统计学意义（χ2=0．270，P=0．603）；ON复发≥3次者致盲率较ON复发2次者明显升高，差异有统计学意义（χ2=4．300，P=0．038）。眼底检查发现，随复发次数增多，视神经异常率增高，差异有统计学意义（χ2=6．750，P=0．034）。视野检查发现，ON首次发病者与ON复发者的视野异常率比较，差异无统计学意义（χ2=0．660，P=0．718）。VEP检查发现，ON复发者较ON首次发病者VEP异常率明显增高，差异有统计学意义（χ2=6．990，P=0．030）。血清NMO-IgG检测发现，ON首次发病者与ON复发者的NMO-IgG阳性率比较，差异无统计学意义（χ2=1．510，P=0．470）；NMO—IgG阳性患者双眼盲发生率较NMO-IgG阴性患者高，差异有统计学意义（χ2=5．063，P=0．027）。结论NMO患者的临床特征为ON和脊髓炎反复发作；以视力下降、感觉障碍、运动障碍及膀胱直肠功能障碍为主要临床表现。随着ON复发次数增多，视神经异常率及VEP异常率均增高。血清NMO-IgG阳性的复发型NMO患者双眼盲发牛率较阴件者高．

Objective To observe the clinical features and visual function of recurrent neuromyelitis optica （NMO）. Methods Thirty-four patients with NMO were enrolled in this retrospective case series study. The patients included two males and 32 females. The average first onset age was （35.03＋ 14.56） years old and the average recurrent rate were （4.24＋2.45） times. The recurrent rate of optic neuritis （ON） ranged from two to 12 times. The recurrent rate of ON was two times in 15 eyes of 10 patients, ~three times in 37 eyes of 24 patients. Vision acuity, direct ophthalmoscope, {undus pre-set lens examination, visual field and visual evoked potential （VEP） were evaluated. Clinical features were observed. The abnormal rate of optic nerve including optic edema and atrophy; abnormal rate of visual field including decreasing retinal sensitivity, central and paracentral scotoma, ring scotoma, half field defects, tunnel visual field, visual field centrality constriction; abnormal rate of VEP including Prolonged latent phase and/or decreasing amplitude of P100 wave from patients of first episode or recurrence was analyzed. Serum NMO- IgG was detected from 28 patients by indirect immunofluorescence technique to observe its positive rate. Results All patients were characterized by repeated episodes of ON and myelitis. The main clinical feature of ON was visual loss, and the main clinical features of myelitis included sensory disability, dyskinesia and vesicorectal disorder. Blindness rate was 41.67% after the first attack of ON, 33.33% after two relapses, and 64.86% after three relapses. The difference of blindness rate between first attack and two episodes was not significant （χ2 = 0. 270, P = 0. 603）. However, the blindness rate in patients having three episodes was significantly higher than those having two episodes （χ2 = 4. 300, P= 0. 038）. With recurrence rate increasing, the abnormal rate of the optic nerve （χ2 =6. 750, P=0. 034）and VEP（χ2=6. 990, P= 0. 030）increased. But the abnormal rate of visual field did not increase along with recurrent rate （χ2= 0. 660, P=0. 718）. Seropositive rate of NMO-IgG did not differ significantly between patients with first attack ON and that with recurrent ON （χ2= 1. 510, P = 0. 470）. But the seropositive patients had significantly higher bilateral blindness rate than seronegative patients （χ2 = 5. 063, P= 0. 027）. Conclusions NMO are characterized by recurrent ON and myelitis. Visual loss, sensory disability, dyskinesia and vesicorectal disorder are the main clinical features. With recurrence rate increasing, the blindness rate, abnormalities the optic nerve and the abnormity rate of VEP increase. Seropositive recurrent NMO patients have hi^her bilateral blindness rate than serone^ative oatients.