中国109例视神经脊髓炎谱系疾病的临床特点分析

目的 了解我国视神经脊髓炎谱系疾病患者的临床特点.方法 检索维普数据库1989年至2016年收录的视神经脊髓炎谱系疾病病例报道文献,并分析所报道病例的临床特点.结果 共纳入87个相关研究,共计109例患者,男女比例为1∶4,发病年龄为5 ～76(38.44±17.08)岁,诱因多为受凉感冒(12.80％),66.05％以视神经炎症状为首发症状,23.85％以脊髓炎为首发症状.脊髓磁共振显示累及到胸髓者80.73％,颈髓占49.54％,腰椎占10.09％,12.84％累及最后区.结论 视神经脊髓炎谱系疾病好发于青壮年,女性多见,男女发病年龄无明显差异,首发症状以视神经炎多见,脊髓病灶以胸髓多见.     

NMOSD合并HIV感染/AIDS的诊疗：病例报告并文献复习

报告一例视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)合并人体免疫缺陷病毒(human immunodeficiency virus,HIV)感染/获得性免疫缺陷综合征(acquired immune deficiency syndrome, AIDS)，并通过文献复习，总结其发病机制、临床特征、治疗及预后。检索文献包括7篇英文文献(8个病例)，1篇中文文献，共报道9例NMOSD合并HIV感染/AIDS病例，结合本文报道的1例共10例，其中5例为女性，5例为男性，3例HIV感染/AIDS为新发，其他病例的HIV感染/AIDS发病均早于NMOSD。临床表现上，7例均为视神经炎和脊髓炎同时或相继发生，2例表现为单相病程或复发性脊髓炎，1例仅表现为双眼相继发生的视神经炎，10例患者头颅或脊髓MRI均有典型的视神经或脊髓异常信号，伴或不伴强化。2例患者未进行水通道蛋白4(aquaporin protein-4,AQP4)抗体Ig G检测，其余8例中5例AQP4抗体阳性、3例阴性。针对AIDS的治疗，10例患者中，8例接受了高效...     

水通道蛋白4抗体阳性视神经脊髓炎谱系疾病相关性视神经炎患者临床特征及预后因素分析

目的观察并分析水通道蛋白4(AQP4)抗体阳性视神经脊髓炎谱系疾病相关性视神经炎(NMOSD-ON)患者临床特征及预后相关因素。方法双向队列研究。2015年6月1日至2019年6月1日于解放军总医院第一医学中心神经眼科病区检查确诊的AQP4抗体阳性NMOSD-ON患者103例纳入研究。所有患者随访时间≥24个月。根据末次随访时最佳矫正视力(BCVA), 将患眼分为低视力组[最小分辨角对数(logMAR)BCVA≥1.0]、非低视力组(logMAR BCVA<1.0), 分别为66、37例。对比观察两组患者临床特征并采用logistic回归模型和COX比例风险模型分析影响患者视力预后及复发的相关因素。结果 103例中, 女性96例(93.2%, 96/103);单眼94例(91.3%, 94/103)。首次发病48例(46.6%, 48/103)。发病时伴眼痛或眼眶痛85例(82.5%, 85/103);视盘水肿21例(20.4%, 21/103)。血清自身免疫抗体阳性51例(49.5%, 51/103)。行眼眶核磁共振成像(MRI)检查101例, 视路未见明显异常信号5例(5.0...     

视神经脊髓炎谱系疾病动物模型的研究进展

视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)是一种中枢神经系统炎性脱髓鞘性疾病,以视神经、脊髓和大脑受累为主要特征,该疾病易复发且致盲、致残率高,严重威胁人类视力和健康。目前NMOSD病因尚不明确,现有治疗方案也无法彻底治愈NMOSD,而动物模型是探索其发病机制与病理生理特点的重要工具。NMOSD动物模型主要建立在抗水通道蛋白4抗体(anti-aquaporin 4 immunoglobulin G,AQP4-IgG)致病基础上,主要包括破坏或绕过血脑屏障(blood brain barrier,BBB)被动转移AQP4-Ig G或AQP4特异性T细胞等,目前还没有一种动物模型可以完整模拟人类NMOSD的临床和病理特征,因此在研究中选择合适的动物模型对相关研究至关重要。     

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。     

水通道蛋白4抗体阳性儿童视神经炎的临床特征和预后分析

目的观察水通道蛋白4(AQP4)抗体阳性儿童视神经炎(AQP4-PON)的临床、影像学特征和预后。方法回顾性病例系列研究。2015年1月至2018年12月于解放军总医院第一医学中心眼科确诊的AQP4-PON患儿14例23只眼纳入研究。所有患儿均行BCVA、眼底彩色照相、眼眶MRI检查;行OCT检查10例15只眼,测量患眼视盘周围神经纤维层(pRNFL)、黄斑区节细胞层+内丛状层(mGCIPL)厚度。采用基于细胞的间接荧光免疫法检测血清AQP4抗体和髓鞘少突胶质细胞糖蛋白抗体。随访时间28~59个月。分析患儿临床特征、神经影像学特征及预后情况。结果14例患儿中,男性2例(14.3%),女性12例(85.7%)。平均发病年龄(13.3±3.0)岁。首次发病时,单眼10例,双眼4例。表现为视神经炎11例(78.6%),极后区综合征2例(14.3%),脊髓炎1例(7.1%)。伴眼痛10例(71.4%),同时合并自身抗体阳性5例(35.7%)。首次发病时间<2周时,眼底检查可见视盘水肿7只眼(38.9%)。发病时间3个月时,行OCT检查的15只眼,平均pRNFL、mGCIPL厚度分别为(62.33±11.07)、(54.17±5.42)μm。眼眶MRI检查结果显示,视神经呈长T2信号14例(100.0%),伴有T1强化病灶11例(78.6%)。首次发病时间<2周时,BCVA≤0.1者16只眼(88.9%),经糖皮质激素治疗后BCVA≤0.1者7只眼(38.9%),≥0.5者9只眼(50.0%)。随访过程中出现复发11例,加用免疫抑制剂治疗。末次随访时,14例中,累及双眼9例(64.3%,9/14),进展为视神经脊髓炎5例(35.7%,5/14);23只眼中,BCVA≥0.5者8只眼(34.8%,8/23)。结论AQP4-PON以女性患儿多见;视功能受损严重,易复发;部分患儿可进展为视神经脊髓炎。     

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。     

致盲性视神经脊髓炎谱系疾病相关视神经炎的视功能预后分析

目的 分析致盲性视神经脊髓炎谱系疾病相关视神经炎的临床和免疫学特征,探讨影响视功能预后的预测因素。设计回顾性病例系列。研究对象2018-2022年北京同仁医院45例致盲性视神经脊髓炎谱系疾病相关视神经炎患者。方法根据6个月后Wingerchuk视力分级分为预后不良组(27例)和预后良好组(18例)。对两组患者人口特征、症状、实验室及影像学检查、治疗方法、治疗后6个月视力改善情况进行对比分析。主要指标临床表现及视力改善情况。结果预后不良组的视神经受累长度大于1/2比例更高(P=0.009),血清学免疫抗体阳性率较低(P=0.028),甲强龙冲击治疗距发病时间更长(P=0.032)。Logistic回归显示,视神经受累长度大于1/2(OR=16.247,95%CI:10.834～24.363;P<0.01)、血清学免疫抗体阴性(OR=13.965,95%CI:9.465～20.603;P<0.01)、甲强龙冲击治疗距发病时间(OR=1.107,95%CI:1.075～1.139;P<0.01)是预后不良的危险因素。ROC曲线分析显示甲强龙冲击治疗距发病时间大于7天预测不良...     

视神经脊髓炎谱系疾病相关性视神经炎的治疗研究进展

视神经脊髓炎谱系疾病相关性视神经炎是引起患者视力严重下降的常见神经眼科疾病。视神经脊髓炎谱系疾病具有复发率高、预后差的特点。控制急性炎性反应、预防复发是视神经脊髓炎谱系疾病治疗的两个主要目标。随着对视神经脊髓炎谱系疾病发病机制的深入研究, 不断出现针对病变发生过程中不同阶段新的治疗方法。本文就视神经脊髓炎谱系疾病相关性视神经炎的治疗现状和治疗前景综述如下。(中华眼科杂志, 2020, 56: 539-543)     

免疫吸附治疗视神经脊髓炎谱系疾病相关性视神经炎的效果

目的 初步评价免疫吸附对视神经脊髓炎谱系疾病相关性视神经炎(NMOSD-ON)的治疗效果。设计前瞻性同期非随机对照临床试验。研究对象纳入2017年11月～2020年7月就诊于上海德济医院的NMOSD-ON AQP4抗体阳性患者75例,均为急性发作时激素冲击治疗14天左右视力无明显恢复者。方法根据患者意愿将其分成研究组39例,对照组36例。研究组在激素冲击递减治疗基础上,予免疫吸附治疗法进行强化治疗,对照组仅继续使用激素冲击后递减治疗。治疗有效定义为视力上升不小于1级或抗体滴度下降至1:32及以下或转阴。主要指标治疗有效率、视力、AQP4-IgG滴度。结果研究组治疗总体有效率76.92%,高于对照组38.89%(χ²=9.65,P=0.002)。研究组视力提高率74.1%(43/58)高于对照组17.5%(10/57)(χ²=34.81,P=0.000)。研究组抗体滴度下降率94.87%(37/39)高于对照组30.56%(11/36)(χ²=30.88,P=0.000)。结论免疫吸附治疗能有效降低激素冲击治疗效果不敏感患...     

Neuromyelitis optica and aquaporin‐4 (AQP4) autoantibodies in consecutive optic neuritis patients in Southern Finland

Purpose: To analyse the frequency of neuromyelitis optica (NMO) among consecutive optic neuritis (ON) patients in Southern Finland and the feasibility of Aquaporin‐4 (AQP4) autoantibody assay in the diagnosis of NMO. Methods: Consecutive patients with symptoms suggestive of acute ON and managed in the Helsinki University Central Hospital were evaluated critically screened for AQP4 autoantibody during a 47.5‐month period. The antibodies were determined using radioimmunoprecipitation method. AQP4 index >15 was considered positive, 10–15 borderline and <10 normal. Brain magnetic resonance imaging (MRI) was performed for all patients. Results: Of the 300 patients with suspected ON, 191 were eventually diagnosed as ON, and 66 (35%) of them had a previous diagnosis or were diagnosed with multiple sclerosis (MS). Of the 125 patients without MS diagnosis, 62 (50%) had demyelinative lesions in MRI, which is a risk factor for developing MS. Two patients (1.1%; 95% CI 0.3–4.5) fulfilled the criteria of NMO. Positive AQP4 antibodies were found in three patients (1.6% 95% CI 0.3–4.5), one of them had NMO, one had MS and one became diagnosed with MS a month later. Borderline autoantibody levels were found in 10 patients, 7 of whom had MS. Conclusions: NMO is rare among ON patients in the population of Southern Finland. In this small cohort, the sensitivity and positive predictive values of the AQP4 autoantibody index for NMO were low, 1/2 and 1/3 respectively, and do not support initiating routine screening.     

Neuromyelitis optica (NMO) antibody positivity in patients with transverse myelitis and no visual manifestations

BACKGROUND AND PURPOSE: To describe a subgroup of patients with IgG antibody to Aquaporin 4 Protein (AQP4) specific to neuromyelitis optica (NMO), who did not have clinical manifestations of optic nerve involvement at the time of diagnosis. METHODS: Assessment of five patients (four African Americans and one Latino) with myelitis, who were NMO IgG antibody positive, who had no detected optic nerve involvement, over a span of one to eighteen years. RESULTS: Cerebrospinal fluid (CSF) studies showed lymphocytic pleocytosis, elevated interleukin (IL6), oligoclonal bands (OCB), myelin basic protein (MBP), and elevated albumin and IgG index. Serology showed an association with antinuclear antibody (ANA) positivity and antithyroid peroxidase (TPO) antibody. Our patients responded well to acute treatment with intravenous corticosteroids and long-term treatment with oral prednisone and azathioprine. CONCLUSIONS: The aquaporin protein autoimmune disease previously identified as neuromyelitis optica (NMO) may more correctly be identified as neuromyelitis (NM), and future diagnostic criteria should take into account the population of patients with antibody to aquaporin 4 protein, without clinically evident optic nerve pathology. Future research may indicate that the entity of NMO is a subcategory of the NM population.     

多发性硬化与视神经脊髓炎患者的眼部特征及其治疗效果

目的 观察伴有眼部异常的多发性硬化(MS)及视神经脊髓炎(NMO)患者的眼部特征及治疗效果.方法 回顾分析我院确诊为MS及NMO且伴有眼部异常的107例患者的临床资料.其中,MS患者81例,NMO患者26例.所有患者均接受MRI、腰椎穿刺脑脊液(CSF)检测红、白细胞计数、蛋白含量及寡克隆带,确定MS及NMO临床诊断.对所有患者行视力、裂隙灯显微镜、眼底等常规眼科检查,并行计算机视野和视觉诱发电位(VEP)检查,对比分析MS、MMO患者眼部临床特征的异同.对患者进行大剂量甲泼尼龙冲击治疗或中药活血化瘀、营养神经治疗后,分析不同治疗方法对眼部症状改善的影响.所有患者随访1个月～5年,平均随访时间26个月.结果 MS患者中球后视神经炎24例,占MS患者总数的29.6%;麻痹性斜视和复视36例,占44.4%.NMO患者中急性视盘炎12例,占NMO患者总数的46.2%;球后视神经炎14例,占53.8%.MS和NMO患者视野检查异常率分别为71.6%、96.2%.MRI检查结果显示MS患者脱髓鞘斑块位于脑室旁最常见;NMO患者病灶多累及脊髓.脑脊液检查结果显示MS患者寡克隆带阳性率为75.3%,NMO患者为19.2%.MS和NMO患者VEP检查主要表现为P100波潜伏期延长和(或)波幅降低.接受甲泼尼龙冲击治疗与未接受冲击治疗的患者视力提高率分别为84.7%、80.0%,差异无统计学意义(χ2=0.221,P>0.05).结论 MS及NMO患者均可能发生视神经炎.糖皮质激素冲击治疗可加速患者眼部症状的改善.     

Neuromyelitis optica spectrum disorders and anti-myelin oligodendrocyte glycoprotein positive optic neuropathies

AIM: To describe the clinical characteristics and treatments associated with antibody positive optic neuropathies including anti-myelin oligodendrocyte glycoprotein (MOG) and anti-aquaporin 4 (AQP4), alongside diagnostic modalities, investigations, and outcomes. METHODS: A cross-sectional single-centre retrospective case series consisting of 16 patients including 12 anti-MOG positive patients and 4 anti-AQP4 positive patients. Each of these patients had clinical signs and symptoms of optic neuritis and consisted of all patients who had a positive blood antibody result in our centre. Clinical findings including presence of a relative afferent pupillary defect, colour vision and disc assessment were recorded. Structured clinical exam and multimodal imaging was undertaken sequentially on each. Optical coherence tomography (OCT) scanning was preformed to examine the correlation between ganglion cell layer (GCL) thickness and visual acuity (VA) at presentation and as a determinant of final visual outcome in both groups. Initial and long-term treatment is also summarised. RESULTS: A total of 16 patients were included in the study consisting of 12 anti-MOG and 4 anti-AQP4 positive patients. Nine of the 16 patients were female and the average age of onset was 29.2y in the MOG group and 42y in the AQP4 group. There was no statistically significant correlation (Pearson correlation) between GCL thickness and presenting and final VA [r(10)=0.081, P=0.08 and r(10)=0.089, P=0.34 respectively]. The same statistical analysis was performed for the correlation between retinal nerve fibre layer (RNFL) and VA and similar outcomes were observed [r(10)=0.04, P=0.22 and r(10)=0.09, P=0.04]. No correlation was seen for initial RNFL thickness and final visual outcome in this group either [r(2)=0.19, P=0.38]. Visual field testing and radiological findings for each group are described. CONCLUSION: No correlation between initial VA or RNFL and final visual outcome is identified. A broad range of visual field and radiographic findings are identified, a consensus on treatment of neuromyelitis optica spectrum disorders and anti-MOG positive optic neuropathies has yet to be accepted but initial high dose immunosuppression followed by low dose maintenance therapy is favoured.     

Optical coherence tomography in central nervous system demyelinating diseases related optic neuritis

AIM: To compare the thickness of the peripapillary retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) among patients with various forms of optic neuritis (ON) and to identify whether any particular parameters or their thinning pattern can be used to distinguish the type of ON. METHODS: This prospective study was conducted at the Department of Ophthalmology, Faculty of Medicine, Siriraj Hospital, Thailand, between January, 2015 and December, 2016. We enlisted patients over 18 years of age with history of ON and categorized patients into 4 groups: 1) aquaporin 4 antibodies (AQP4-IgG) positive; 2) multiple sclerosis (MS); 3) myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) positive; 4) idiopathic-ON patients. Healthy controls were also included during the same study period. All patients underwent complete ophthalmological examination and spectral domain optical coherence tomography (OCT) imaging to analyze RNFL and GCIPL thickness after at least 3mo since the last episode of acute ON. The generalized estimating equation (GEE) models were used to compare the data amongst ON groups. RESULTS: Among 87 previous ON eyes from 57 patients (43 AQP4-IgG+ON, 17 MS-ON, 8 MOG-IgG+ON, and 19 idiopathic-ON), mean logMAR visual acuity of AQP4-IgG+ON, MS-ON, MOG-IgG+ON, and idiopathic-ON groups was 0.76±0.88, 0.12±0.25, 0.39±0.31, and 0.75±1.08, respectively. Average, superior, and inferior RNFL were significantly reduced in AQP4-IgG+ON, MOG-IgG+ON and idiopathic-ON eyes, relative to those of MS-ON. Differences were not statistically significant for RNFL or GCIPL between the AQP4-IgG+ON and MOG-IgG+ON groups, whereas visual acuity in MOG-IgG+ON was slightly, but not significantly, better (0.39 vs 0.76). Although RNFL thickness in MOG-IgG+ON was significantly reduced as compared to MS-ON, mean visual acuity and GCIPL were not different. CONCLUSION: Thinning of superior and inferior quadrants of RNFL are more commonly seen in MOG-IgG+ON and AQP4-IgG+ON. Long term visual acuity in MOG-IgG+ON is often better than AQP4-IgG+ON, whereas the structural change from OCT is comparable.     

神经内科住院患者神经眼科临床特征研究

目的:研究神经内科患者神经眼科临床方面的基本特征,为进一步发展我国神经眼科学和更好地指导临床工作服务。方法:调查我院神经内科2009-06-01/2010-06-01全年有神经眼科表现的患者的临床资料,研究其疾病构成,神经眼科主要临床表现进行统计分析得出结论。结果:共调查神经内科住院患者651例,其中有神经眼科表现的为24.6%。有神经眼科表现的患者年龄为非正态分布,M=54岁;神经内科患者神经眼科症状最多的是复视44例,持续性视力下降42例,体征最多的是眼球运动障碍43例,眼球震颤38例。引起神经眼科表现的神经内科主要疾病构成:脑梗死49例、视神经脊髓炎17例、重症肌无力15例、脑出血14例、椎基底动脉供血不足12例、颅内静脉窦血栓形成5例、多发性硬化5例、炎性脱髓鞘病5例和颅内恶性肿瘤8例等,以上疾病占81.3%。有神经眼科表现的神经内科病变性质为循环障碍92例、非感染性炎症48例、恶性肿瘤8例、颅内感染性炎症4例、外伤3例、中毒2例、原因不明3例。结论:神经内科住院患者中24.6%有神经眼科表现。神经内科患者的神经眼科临床表现以复视、眼球运动障碍、持续性视力下降、眼球震颤最多。有神经眼科表现的神经内科主要疾病有脑梗死、NMO,MG、脑出血、椎基底动脉供血不足、CVST,MS、炎性脱髓鞘病和颅内恶性肿瘤。有神经眼科表现的神经内科疾病的病变性质主要为循环障碍和非感染性炎症。     

Clinical profile,imaging features and short term visual outcomes of Indian optic neuritis patients with and without seromarkers for myelin oligodendrocyte glycoprotein and neuromyelitis optica

Purpose:To analyze clinical profile, imaging features, and short-term visual outcomes of optic neuritis patients in Indian population with and without seromarkers for myelin oligodendrocyte glycoprotein (MOG)/neuromyelitis optica (NMO).Methods:Electronic medical records of 203 optic neuritis patients who presented between June 2018 and December 2019 to the Neuro-ophthalmology services of a tertiary care center in India were retrospectively analyzed.Results:Of 203 patients, 57 patients (28.08%) were positive for MOG-antibody and 20 patients (9.85%) were positive for NMO antibody. 114 patients (56.16%) were double-negative (negative for both antibodies) and 12 patients (5.91%) were diagnosed as multiple sclerosis (MS). None of the patients had both antibodies. Mean age of presentation was 31.29 ± 1.035 years. There was female preponderance in NMO-optic neuritis (NMO-ON) and MS-optic neuritis (MS-ON) groups (1:5). Mean vision on presentation was worse (logMAR 1.570 ± 0.863) in NMO-ON group. The mean visual acuity showed statistically significant recovery (logMAR 0.338 ± 0.639) in the final follow-up in MOG-optic neuritis (MOG-ON) group. Multivariate logistic regression analysis revealed poor visual outcome in patients presenting with retrobulbar neuritis, optic disc pallor, bilateral sequential optic nerve involvement, and with positive NMO antibody. Optic neuritis patients presenting with disc edema associated with pain and positive for MOG antibody were found to have a better visual outcome.Conclusion:In this Indian optic neuritis cohort, the prevalence of MOG-ON was higher than NMO-ON. MOG-ON had a better visual outcome than NMO-ON. The incidence of MS-ON was less compared to the western literature. A significant number of patients (114 patients, 56.16%) were double negative for both seromarkers and yet had presented with optic neuritis with no clinical or imaging features suggestive of MS/MOG associated disease (MOG AD)/NMO spectrum disorder (NMO SD).