血清AQP4-Ab在视神经炎患者中的表达

目的：探讨血清水通道蛋白4抗体( AQP4-Ab )在视神经炎患者中的阳性表达率及其意义。
　　方法：选取2012-01/2015-12本院眼科中心确诊的98例128眼视神经炎患者进行研究,检测患者的血清AQP4-Ab、抗核抗体( ANAs )的阳性率,根据AQP4-Ab表达进行分组,对比两组间最佳矫正视力、盘周视网膜神经纤维层厚度( pRNFL )、黄斑容积、黄斑部RNFL ( mRNFL )、黄斑部内核层容积( mINL)测定值。
　　结果：确诊的视神经炎患者98例128眼,经过检查发现AQP4-Ab 阳性患者22例(22%),阴性患者76例(78%);ANAs 阳性患者21例(21%),阴性患者77例(79%);视神经炎患者的血清AQP4-Ab阳性率与ANAs阳性率具有显著的相关关系(r=0.707,P<0.01);经过检查发现AQP4-Ab阳性患者和阴性患者的最佳矫正视力差异无统计学意义( P>0.05);经过检查发现AQP4-Ab阳性患者的pRNFL、黄斑容积测定值均显著地小于阴性患者,差异均具有统计学意义( P<0.05);AQP4-Ab阳性患者和阴性患者的mRNFL、mINL测定值差异均无统计学意义(P>0.05)。
　　结论：AQP4-Ab在视神经炎患者中的阳性表达与ANAs存在显著的相关性, AQP4-Ab 阳性视神经炎患者的pRNFL变薄、黄斑容积下降明显。     

视神经脊髓炎谱系疾病患者临床特点分析

目的　分析并总结视神经脊髓炎谱系疾病患者的临床特点,为临床诊治提供参考依据。方法　回顾性分析中日友好医院神经眼科联合门诊2016年3月至2018年12月收治的75例视神经脊髓炎谱系疾病患者,对患者的一般情况、临床症状特点、实验室检查以及影像学检查结果进行分析,以及对比水通道蛋白4（aquaporin-4,AQP4）-IgG阳性和AQP4-IgG阴性患者的临床症状异同点。结果　75例患者中,男12例、女63例,男女比例接近为1∶5,发病年龄7~77岁,病程1个月~30 a,发病次数1~20次,16例（21.3%）患者起病前有明显诱因,其余59例（78.7%）患者无明显诱因发病。首次发病症状视神经炎发病28例（37.3%）,脊髓炎发病18例（24.0%）,视神经和脊髓同时受累19例（25.3%）,颅内发病10例（13.3%）,有19例患者伴随其他疾病,包括干燥综合征、系统性红斑狼疮等;45例（60.0%）患者检测了视神经脊髓炎特异性抗体（AQP4-IgG）,其中31例（68.9%）患者呈现AQP4-IgG阳性表现,13例（28.9%）患者呈现AQP4-IgG阴性表现,尚有1例（2.2%）患者呈现髓鞘少突胶质细胞糖蛋白抗体阳性表现。结论　视神经脊髓炎谱系疾病多发于女性,大部分患者无明显诱因起病,复发率较高,首发病症累及视神经为主,其特异性抗体AQP4-IgG阳性患者多见,应予以早期诊断和治疗。     

水通道蛋白-4抗体阳性视神经脊髓炎谱系疾病患者光相干断层扫描血管成像特征及相关因素分析

目的观察水通道蛋白-4(AQP-4)抗体阳性视神经脊髓炎谱系疾病(NMOSD)患者光相干断层扫描血管成像(OCTA)特征,分析其相关因素。方法病例对照研究。2015年9月至2017年8月在复旦大学附属眼耳鼻喉科医院眼科检查确诊的AQP-4抗体阳性NMOSD患者48例96只眼及同期年龄性别与之匹配的健康志愿者20名40只眼(正常对照组)纳入研究。均为双眼入组。根据视神经炎(ON)发作次数将NMOSD患者分为0 ON组、1 ON组、2 ON组、3+ON组,分别为30、22、31、13只眼。0 ON组为无ON发作史;1 ON组、2 ON组、3+ON组分别为ON发作1、2、≥3次。受检眼均行最佳矫正视力(BCVA)、视野、OCTA检查。BCVA检查采用国际标准Snellen视力表进行,并将结果转换成最小分辨角对数视力记录。采用Humphery视野计测量受检眼视野平均缺损值(MD)。采用OCTA仪对受检眼视盘区4.5 mm×4.5 mm和黄斑区6.0 mm×6.0 mm范围进行扫描,测量放射状视盘周围毛细血管(RPC)血流密度、黄斑旁中心凹视网膜浅层毛细血管丛血流密度(SVD)以及视盘旁神经纤维层(pRNFL)、黄斑旁中心凹神经节细胞-内丛状层(GCIPL)厚度。采用广义估计方程分析4组受检眼之间BCVA、视野MD、pRNFL与GCIPL厚度、RPC血流密度、SVD的差异,以及视网膜灌注与视网膜结构、视功能的关系。结果与正常对照组比较,0 ON组患眼RPC血管密度、SVD明显降低,差异有统计学意义(Waldχ²=7.190、10.134,P<0.01);两组受检眼之间BCVA和pRNFL、GCIPL厚度比较,差异均无统计学意义(Waldχ²=2.308、1.020、2.558,P>0.05)。与0 ON组比较,1 ON组、2 ON组、3+ON组患眼BCVA、视野MD、RPC血流密度、SVD和pRNFL、GCIPL厚度均明显降低,差异有统计学意义(Waldχ²=12.390、11.346、38.860、18.040、45.418、26.608,P<0.01);1 ON组、2 ON组、3+ON组患眼之间上述各项指标比较,差异无统计学意义(P>0.05)。相关性分析结果显示,RPC血流密度与pRNFL厚度(β=0.372,标准误=0.018,P<0.001)、SVD与GCIPL厚度(β=0.115,标准误=0.204,P<0.001)密切相关。BCVA、视野MD与RPC血流密度密切相关(BCVA:β=0.025,标准误=0.005,P=0.000;视野MD:β=0.737,标准误=0.185,P=0.000)。结论NMOSD患眼ON发作前RPC血流密度、SVD下降;ON发作后,视功能、视网膜结构和灌注进一步受损,但损伤程度与ON发作次数无明显相关性。NMOSD患眼RPC血流密度与视功能密切相关。     

青光眼与非炎症性缺血型视神经病变的傅立叶OCT扫描视神经形态学对比

目的 比较青光眼与非炎症性缺血型视神经病变(non-arteritic ischemic optic neuropathy,NAION)患者患眼的视盘及盘周视网膜神经纤维层(peripapillary retinal nerve fiber layer,pRNFL)参数变化情况及诊断能力.方法 选择我院眼科年龄≥40岁就诊患者71例(71眼).受试者分为青光眼组26例、NAION组15例、对照组30例,排除视野缺损范围大于两个象限或等效球镜度数大于±6D的受试眼以及发病时间小于6个月的NAION患眼.所有患者均接受眼部常规检查,使用傅立叶OCT测量视盘及pRNFL各参数.结果 3组之间除视盘面积外(P =0.059),其余视盘及pRNFL各参数差异均有统计学意义(均为P＜0.05).经LSD两两比较发现:青光眼组的视杯面积(1.438±0.714)mm2最大(均为P ＜0.05),盘沿面积(0.965 ±0.652)mm2最小(均为P＜0.05),盘沿容积和视神经盘容积[(0.103 ±0.089)mm3、(0.195±0.168)mm3]最小(均为P＜0.05),视杯容积(0.482 ±0.420)mm3最大(均为P ＜0.05),杯盘比最大(均为P＜0.05);NAION组的视杯面积(0.493±0.344) mm2最小(均为P＜0.05),盘沿面积(1.255±0.294) mm2与对照组(1.243±0.509) mm2差异无统计学意义(P＞0.05),盘沿容积(0.196±0.094)mm3、视神经盘容积(0.339±0.109) mm3与对照组差异均无统计学意义(均为P＞0.05),视杯容积(0.083 ±0.073)mm3最小(均为P＜0.05),杯盘比最小(均为P＜0.05).在pRNFL方面,经LSD两两比较发现:3组在平均值及TU、ST、SN、NU、IT区之间两两比较差异均有统计学意义(均为P＜0.05);在NL、IN、TL区,青光眼组与对照组两两比较差异均有统计学意义(均为P ＜0.05).将青光眼组与对照组进行AROC分析发现:视盘参数中除视盘面积外(P＞0.05),其他参数及所有pRNFL各参数差异均有统计学意义(均为P＜0.05).将NAION组与对照组进行AROC分析发现:pRNFL中的上方(即ST、SN区)、鼻上(即NU区)和下方偏颞侧(即IT区)AROC差异均有统计学意义(均为P＜0.05),视盘所有参数及pRNFL其余部位差异均无统计学意义(均为P ＞0.05).将NAION组与青光眼组进行AROC分析发现:视盘参数中视杯面积、视杯容积、杯盘比(包括面积、水平和垂直)的AROC差异均有统计学意义(均为P＜0.05),而pRNFL中的颞上方(即TU区)、鼻上方(即NU区)和平均值AROC差异均有统计学意义(均为P＜0.05),其余部位差异均无统计学意义(均为P＞0.05).结论 通过傅立叶OCT检测可发现青光眼与NAION在视盘及pRNFL方面的差异,为理解此两种视神经疾病的发病特点及鉴别诊断提供临床依据.     

麦考酚酸酯治疗AQP4抗体阳性视神经炎疗效观察

目的：评价麦考酚酸酯(MMF)对AQP4抗体阳性视神经脊髓炎谱系障碍(NMOSD)患者预防复发和视力预后的作用。

方法：回顾性病例研究。收集2017-01/2019-12收治的AQP4抗体阳性NMOSD患者11例,其中男3例,女8例。NMOSD特有的重要临床表现为视神经炎。发病平均年龄36.3±6.0(27～47)岁,平均病程3.4±1.4(2.2～6.8)a。在NMOSD缓解期加用MMF 1a或1a以上。记录应用MMF患者的年复发率(ARR)、最佳矫正视力(BCVA)和不良反应。

结果：MMF治疗的中位时间为18(12,36)mo。ARR在基线时为0.66/a,治疗后为0.16/a。91%的患者ARR下降,82%的患者无临床复发。MMF治疗后ARR明显改善(P<0.05)。治疗后平均BCVA与治疗前比较无显著差异(P>0.05)。11例患者中,3例(27%)出现不良反应,其中1例(9%)出现转氨酶升高,2例(18%)出现轻度胃肠道反应。没有因不良反应而停用MMF的情况。

结论：MMF治疗AQP4抗体阳性NMOSD患者能在一定程度上降低其视神经炎的ARR,保护患者的视功能。     

儿童抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关性视神经炎的临床特征及预后分析

目的 观察髓鞘少突胶质细胞糖蛋白(MOG)抗体相关视神经炎患儿的临床特征及预后。方法 回顾性队列研究。选取2016年10月至2020年10月在北京儿童医院神经内科确诊为MOG-ON患儿资料,分析临床、神经影像学及预后特点。结果 共入组25例患儿,男13例,女12例,起病年龄(8.3±2.2)岁。(1)临床特点:首次发病时单眼发病10例,双眼发病15例,31/40 (78%)眼最佳矫正视力BCVA≤0.1,9/25例(36%)伴眼痛。10例为复发性ON,最终累及47眼,双眼受累22例(88%)。(2)辅助检查:首次发作时,29/40眼(72%)伴视盘水肿;23/25例(92%)患儿视觉诱发电位异常,为P100潜伏期延长伴或不伴振幅减低;16/19 (84%)视神经MRI异常,10例累及视神经1/2段以上,10例伴视神经周围组织强化;16/25(64%)头颅MRI可见颅内病变,9/23 (39%)脊髓MRI异常。(3)治疗及随访:所有患儿接受糖皮质激素联合或不联合丙种球蛋白治疗有效。9例复发患儿接受吗替麦考酚酯或利妥昔单抗治疗,中位随访2.2年(范围1.7～3.6),5例(55.6%)复发...     

中国人不同类型视神经炎临床特征分析--3年随访研究

目的观察并随访以血清抗体分型的视神经炎(ON)患者临床特征及转归。方法单中心队列研究。纳入2015年1月~2017年3月在复旦大学附属眼耳鼻喉科医院首次确诊的ON患者,按照血清抗体类型分组:抗髓鞘寡突胶质细胞糖蛋白抗体阳性组(MOG-ON)、抗水通道蛋白4抗体阳性组(AQP4-ON)及2种抗体均阴性组(seronegative-ON),评估视觉功能及光学相干层析成像(OCT),并随访3年。结果共纳入评估患者280例(405眼),其中MOG-ON组57例(20.4%)、AQP4-ON组98例(35.0%)、seronegative-ON组125例(44.6%)。3年后随访各组患者,MOG-ON组和seronegative-ON组最佳矫正视力高于0.8的眼数相仿,且均较AQP4-ON组明显增多(P=0.001)。MOG-ON组和AQP4-ON组的复发率相当,均高于seronegative-ON组(P<0.001)。3年后,MOG-ON组和AQP4-ON组视盘周围视网膜神经纤维层(RNFL)厚度相似,分别为(63.41±13.39)μm和(59.40±11.46)μm(P=0.476),均较seronegative-ON组[(74.06±11.14)μm,P=0.001]明显变薄。黄斑区神经节细胞-内丛状层(GCIPL)厚度呈现同样的趋势。结论MOG-ON组患者与seronegative-ON组患者视力预后较好,但MOG-ON组患者不论RNFL或GCIPL均明显变薄。AQP4-ON组患者视力预后差,且OCT随访RNFL与GCIPL显著变薄。     

息肉状脉络膜血管病变的临床特征分析

目的观察息肉状脉络膜血管病变(PCV)的眼底表现及荧光素眼底血管造影(FFA)、吲哚青绿血管造影(ICGA)与相干光断层扫描(OCT)特征。方法分析经眼底检查、FFA、ICGA及OCT确诊的PCV患者16例(16只眼)的眼底图像资料。其中OCT检查资料为8例(8只眼)。结果 16例(16只眼)全部为单眼发病,其中男性10例,占62.5%,女性6例,占37.5%。年龄36～75岁,平均年龄63.4岁。眼底检查14只眼病变部位位于黄斑区,占87.5%,2只眼病变位于视盘颞侧,占12.5%。在黄斑区4只眼见橘红色结节样隆起病灶,13只眼视网膜下有出血,11只眼见脂质沉着。FFA检查16只眼早期均呈强荧光,后期进行性渗漏。其中6只眼表现血液性视网膜色素上皮脱离(PED),1只眼为浆液性PED,2只眼为血液性合并浆液性PED,1只眼呈脉络膜血管网及息肉样结构。ICGA检查16只眼均可见强度不等、簇状或孤立的息肉状扩张灶,其中12只眼可见伞样或树枝样异常扩张的脉络膜血管网。OCT检查8只眼显示7只眼视网膜色素上皮(RPE)和脉络膜毛细血管高反射层呈穹隆状隆起,其下可见结节状改变。1只眼无脉络膜毛细血管高反射层改变。6只眼表现血液性PED,2只眼为浆液性PED合并神经上皮脱离。结论 PCV单眼发病为主,好发部位为黄斑区,最常见表现为视网膜下出血及脂性渗出,部分患眼存在PED。大部分患眼ICGA可见特征性的脉络膜血管网及其末梢的息肉状扩张灶。     

OCT参数对原发性开角型青光眼性视神经损伤的诊断价值

目的:探究频域光学相干断层成像技术(OCT)对原发性开角型青光眼性视神经损伤的诊断价值。方法:选择2018-01/2020-03我院收治的80例80眼原发性开角型青光眼患者及100例100眼健康受试者作为研究对象,将原发性开角型青光眼患者分为早期组、中期组及晚期组,采用OCT测定各组患者上方、下方、鼻侧、颞侧视乳头旁视网膜神经纤维层(pRNFL)厚度值及上方、下方黄斑区视网膜神经节细胞复合体(mGCC)厚度值,比较各组受试者OCT参数之间差异,采用Spearman相关性分析OCT参数与视野缺损程度的相关性,绘制受试者特征工作曲线(ROC)计算OCT参数诊断原发性开角型青光眼的价值。结果:入组患者早期组24例、中期组39例、晚期组17例,各组患者pRNFL、mGCC参数均有差异(P<0.05),晚期组青光眼患者上方、下方、鼻侧pRNFL、平均pRNFL及上方、下方、平均mGCC显著低于早期组、中期组,中期组患者各指标显著低于早期组(P<0.05)。Spearman相关性分析示,pRNFL、mGCC参数与原发性开角型青光眼严重程度呈负相关关系(P<0.05)。ROC曲线显示,上方pRNFL、下方pRNFL、鼻侧pRNFL、颞侧pRNFL、平均pRNFL诊断原发性开角型青光眼视神经损伤的曲线下面积为0.693、0.846、0.676、0.579、0.844,上方mGCC、下方mGCC及平均mGCC诊断原发性开角型青光眼视神经损伤的曲线下面积分别为0.542、0.677、0.676;平均pRNFL联合平均mGCC诊断原发性开角型青光眼视神经损伤的曲线下面积为0.883。结论:OCT测定pRNFL、mGCC参数与开角型青光眼视神经损伤程度密切相关,两者有较高的诊断价值,临床可将其用于诊断及病情评估。     

抗AQP4抗体阳性视神经炎

介绍抗AQP4抗体阳性视神经炎,实验证实视神经脊髓炎(NMO)患者的血液、脊髓液中存在的抗AQP4抗体对NMO具高度敏感性和特异性,所以对来诊的视神经炎全部患者行抗AQP4抗体检查,其中的阳性病例即抗AQP4抗体阳性视神经炎,其临床表现多复发,视力严重受损乃至失明(临床表现见文中表1),以后还能发生脊髓炎,留下脊髓障碍后遗症,抗体阳性确定之后,须使用正确的治疗方法.结合本院的NMO材料得出结论:脑核磁共振无多发硬化症改变、视力降低迅速且严重、复发、女性,激素低效或无效的特发性视神炎病例可能是抗AQP4抗体阳性视神经炎,要留意以后可能出现脊髓炎,针对性治疗不可缺.     

儿童视神经炎临床特点及预后分析

目的 探讨儿童视神经炎的发病特点,为视神经炎患儿的诊疗及护理提供依据。设计 回顾性病例系列。研究对象2010年1月1日至2012年12月31日就诊于解放军总医院的75例（124眼）儿童视神经炎患者。方法 回顾性分析75例（124眼）儿童视神经炎患者的病例资料,记录其发病诱因、发病特点、临床表现,分析其临床特点。跟踪随访6~42个月,进一步分析其预后及转归。主要指标 发病诱因、临床表现、复发率及预后。结果 入组患者平均年龄（10.67±3.57）岁,其中女性56例（74.67%）,29例（38.67%）患者发病前有明确诱因。所有患者发病以来均有不同程度的视力下降,49例（65.33%）患者双眼同时或先后发病。32例（42.67%）患者发病时伴有眼痛或眼球转动痛。所有患者发病眼视盘均有不同程度颜色变淡并与发病时间有关,约51.52%的患者病情复发,但近70%的患者视力恢复>0.5。分别有4例（6.78%）及9例（15.25%）患者转变为多发性硬化及视神经脊髓炎。结论 儿童视神经炎以严重的视力下降、双眼发病为主及良好的预后为特点;年龄偏大的女性患者较易转变为视神经脊髓炎。     

Anti‐aquaporin‐4 antibody‐positive optic neuritis

Purpose: It has recently been reported that the anti‐aquaporin‐4 antibody (AQP4‐Ab) can be a specific marker of neuromyelitis optica. We present three cases of optic neuritis (ON) where the patients tested positive for AQP4‐Ab, but showed no neurological signs. Methods: Sera were obtained from 32 Japanese patients with ON and no other neurological abnormalities (mean age 46 ± 20 years). AQP4‐Ab was detected by indirect immunofluorescence staining using human‐AQP4‐transfected HEK 293 cells. Results: AQP4‐Ab was positive in three female patients (aged 9, 64 and 82 years). Their illness was characterized by bilateral severe optic nerve involvement, insufficient visual recovery, and autoimmune abnormalities (such as positive antinuclear antibody). Two of these patients experienced recurrent episodes of ON. In at least two episodes, the intracranial portion of the optic nerve showed significant inflammation on magnetic resonance imaging. Conclusions: These cases indicate that some ON patients have an immunological pathogenesis similar to that seen in neuromyelitis optica. In addition, examination for AQP4‐Ab positivity in the initial phase of ON is important in predicting the prognosis, including the possibility of the development of transverse myelitis.     

Clinical Profile of Anti-Myelin Oligodendrocyte Glycoprotein Antibody Seropositive Cases of Optic Neuritis

We have studied the clinical picture of anti-aquaporin antibody (AQP4-Ab)– and anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab)–positive optic neuritis. However, optic neuritis associated with MOG-Abs has not been elucidated using new methods such as cell-based assay. Hence, we conducted a comprehensive investigation on its clinical profile. Serum samples from 70 patients (17 males and 53 females, mean age 43.1 years) with optic neuritis were tested for MOG-Abs by cell-based assay. In MOG-Ab seropositive patients, the disease type, recurrence status, and visual function outcome were analysed. Among 70 patients, 18 were MOG-Ab seropositive. The 18 patients comprised 2 with chronic relapsing inflammatory optic neuropathy, 2 with AQP4-Ab seropositive optic neuritis (neuromyelitis optica), 12 with idiopathic optic neuritis, and 2 with optic neuritis associated with multiple sclerosis. Excluding two cases that were also AQP4-Ab seropositive, MOG-Ab seropositive cases had relatively favourable visual acuity outcome (although not significantly different from seronegative cases) but had significant residual visual field deficit (p = 0.0015). Furthermore, the number of relapses of optic neuritis per year was significantly greater in MOG-Ab seropositive cases than in seronegative cases (0.82 vs. 0.40; p = 0.0005). MOG-Abs may contribute to the heterogeneous clinical picture of optic neuritis, and although visual acuity outcome is favourable, there is a tendency of residual visual field deficit and a possibility of repeated relapses.     

Clinical Profile of Anti-Myelin Oligodendrocyte Glycoprotein Antibody Seropositive Cases of Optic Neuritis

Abstract

We have studied the clinical picture of anti-aquaporin antibody (AQP4-Ab)– and anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab)–positive optic neuritis. However, optic neuritis associated with MOG-Abs has not been elucidated using new methods such as cell-based assay. Hence, we conducted a comprehensive investigation on its clinical profile. Serum samples from 70 patients (17 males and 53 females, mean age 43.1 years) with optic neuritis were tested for MOG-Abs by cell-based assay. In MOG-Ab seropositive patients, the disease type, recurrence status, and visual function outcome were analysed. Among 70 patients, 18 were MOG-Ab seropositive. The 18 patients comprised 2 with chronic relapsing inflammatory optic neuropathy, 2 with AQP4-Ab seropositive optic neuritis (neuromyelitis optica), 12 with idiopathic optic neuritis, and 2 with optic neuritis associated with multiple sclerosis. Excluding two cases that were also AQP4-Ab seropositive, MOG-Ab seropositive cases had relatively favourable visual acuity outcome (although not significantly different from seronegative cases) but had significant residual visual field deficit (p?=?0.0015). Furthermore, the number of relapses of optic neuritis per year was significantly greater in MOG-Ab seropositive cases than in seronegative cases (0.82 vs. 0.40; p?=?0.0005). MOG-Abs may contribute to the heterogeneous clinical picture of optic neuritis, and although visual acuity outcome is favourable, there is a tendency of residual visual field deficit and a possibility of repeated relapses.     

Clinical Features of Optic Neuritis in China

Objective: To document the clinical features of optic neuritis in a population of China and compare with reports of Western countries. Background: Optic neuritis is a common optic neuropathy well studied in Western countries. In English literature very few studies addressed optic neuritis in China. Method: Retrospective medical chart review of all patients admitted in a teaching hospital from 2002 to 2005 with a final diagnosis of idiopathic demyelinating optic neuritis. Results: Ninety-eight patients including 45 men and 53 women were collected, with a mean age of 25.7 years. Sixty-six cases (67.3%) were unilateral optic neuritis and 32 (32.7%) were bilateral. Eye pain was reported in 42 cases (42.9%). Visual acuity in 130 affected eyes varied from 0.8 to no light perception, with 0.1 or worse in 101(77.7%), between 0.1 and 0.4 in 15 (11.5%) and 0.5 or better in 14(10.8%). Fifty-two eyes (40%) showed disc swelling. Central scotoma was the most common (61%) localized visual field defect. Optic nerve enhancement was found in 85 of 121 eyes (70.2%) while 15 cases (15.3%) showed periventricular plaques in brain MRI. Positive oligoclonal band or elevated myelin basic protein was found in 17 (17.3%) cases. Eight (8.2%) cases met the criteria of clinical definite multiple sclerosis and 4 cases had neuromyelitis optica. Visual acuity of 35 eyes (26.9%) improved to 1.0 or better while 37 eyes (28.5%) remained 0.1 or worse at the 3-month follow-up. Conclusion: Clinical features of optic neuritis in a population of China were documented. Less ocular pain, less brain MRI abnormalities, more severe visual loss and poor visual outcome were seen compared to reports of Western countries.     

Diagnostic criteria in acute neuromyelitis

Neuromyelitis optica, also known as Devic disease, was identified in the 19th century, is one of the inflammatory idiopathic demyelinating diseases of the central nervous system, often mistaken for severe multiple sclerosis. In 1999 it had been proposed diagnostic criteria for neuromyelitis optica, but in 2006 these criteria were revised by Dean Wingerchuck. These criteria are 99% sensitive and 90% specific for differentiating neuromyelitis optica from multiple sclerosis that present with optic neuritis or a myelitis syndrome. In the following article we present clinical, spinal and cerebral MR imaging, serological and aspects of cerebrospinal fluid examination features of neuromyelitis optica and the revised criteria of neuromyelitis optica established in 2006. The recently identified serum antibody biomarker: neuromyelitis optica immunoglobulin G (NMO Ig G), which target aquaporin 4 water channel, distinguish neuromyelitis optica from multiple sclerosis, is one of the revised criteria of neuromyelitis optica.     

Uso del análisis de la capa de células ganglionares para el diagnóstico en la neuromielitis óptica anti-glucoproteína de la mielina del oligodendrocito

The case concerns a 26-year-old patient with bilateral recurrent optic neuritis episodes in the context of suspected neuromyelitis optica. In the first outbreak, she had greatly impaired visual acuity of the left eye, as well as seeing ganglion cell layer damage in both eyes in the optic coherence tomography, with evidence of a possible extensive lesion in the optic chiasma. Likewise, MRI with contrast showed a great involvement of the left optic nerve that compromises the chiasma increasing the suspicion of a neuromyelitis origin. Althogh the anti-myelin oligodendrocyte glycoprotein (MOG) and anti-AQP4 (aquaporin-4) antibodies were negative at first, bilateral involvement of the ganglion cells suggested an extensive lesion that is more characteristic of seropositive anti-MOG neuromyelitis.     

Neuromyelitis Optica Spectrum Disorder–Related Optic Neuritis Mimicking Idiopathic Orbital Inflammation

The authors report a case of neuromyelitis optica spectrum disorder–related optic neuritis. The patient showed rapid vision loss in both eyes, and brain magnetic resonance imaging indicated perineuritis due to idiopathic orbital inflammation. Although minor improvement was noted following corticosteroid pulse therapy, the patient’s vision worsened after 7 days. Further evaluation revealed anti-aquaporin-4 antibody positivity, therefore the authors diagnosed the case as neuromyelitis optica spectrum disorder. This case suggests that patients with severe bilateral visual loss and poor corticosteroid response should be tested for anti-aquaporin-4 antibody and may require further aggressive management.     

伴发脑部异常对髓鞘少突胶质细胞糖蛋白抗体阳性的视神经炎与水通道蛋白4抗体阳性的视神经炎视网膜视神经结构和功能的影响

目的 分别比较伴和不伴脑部异常的水通道蛋白4抗体阳性的视神经炎(AQP4-ON)与髓鞘少突胶质细胞糖蛋白抗体阳性的视神经炎(MOG-ON)患者视野和光学相干层析血管成像(OCTA)特征,分析其临床特点并讨论可能的影响因素.方法 病例对照研究.入组2015年9月～2017年8月就诊于复旦大学附属眼耳鼻喉科医院的AQP4-...