视神经脊髓炎谱系疾病合并干燥综合征10例临床分析

目的探讨视神经脊髓炎谱系疾病(NOMSD)合并干燥综合征(SS)的临床特点、治疗及预后。方法分析2012年1月至2016年9月在上海市第一康复医院及仁济医院住院的NMOSD合并SS的患者临床资料。结果患者10例,其中男性1例,女性9例;年龄34~63岁,平均年龄46.7±9.4岁;病程1~60个月,平均34.9±25个月。AQP4抗体均为阳性;4例患者以双眼视力下降起病,4例患者以脊髓病变起病,脊髓病变的平均节段长度为9.8±5.9厘米;2例患者以极后区病变起病;EDSS治疗前评分为5.0±2.160,治疗后EDSS的评分为4.4±2.319。结论 NMOSD合并SS的患者AQP4抗体的阳性检出率高,EDSS评分高,预后差,NMOSD可能和SS存在某些共同的发病机制。     

甲泼尼龙冲击治疗对视神经脊髓炎谱系病患者外周血滤泡辅助性T细胞百分率的影响

目的探究视神经脊髓炎谱系病(NMOSD)患者外周血滤泡辅助性T细胞(Tfh)百分率在甲泼尼龙冲击治疗前、后的变化。方法收集AQP-4抗体阳性的急性期NMOSD患者(NMOSD组)23例,采用流式细胞术检测甲泼尼龙冲击前、后外周血CXCR5~+ICOS~+的CD4~+T细胞(Tfh细胞),记录NMOSD组的EDSS评分,分析NMOSD组患者Tfh细胞百分率与患者AQP-4抗体滴度及EDSS评分的相关性。结果 NMOSD组患者甲泼尼龙冲击治疗前外周血Tfh细胞百分率[(1.77±0.74)%]显著高于甲泼尼龙冲击治疗后[(1.26±0.45)%](t=2.284,P=0.032)。首次发病NMOSD患者甲泼尼龙冲击治疗前Tfh细胞百分率与EDSS评分呈正相关(r=0.625,P=0.040),甲泼尼龙冲击治疗前、后Tfh细胞百分率与AQP-4抗体滴度无相关性(r=--0.02,P=0.928)。结论经甲泼尼龙冲击治疗后,NMOSD患者急性期外周血Tfh细胞百分率降低,首次发病NMOSD患者Tfh细胞百分率水平与EDSS评分有关。Tfh细胞百分率水平可能与NMOSD的复发有关。     

视神经脊髓炎谱系病患者痛性强直痉挛发作的临床特点

目的探讨视神经脊髓炎谱系病(NMOSD)患者痛性强直痉挛发作(PTS)的临床特点。方法回顾性分析143例NMOSD患者的临床表现、实验室检查以及影像学资料等,探讨NMOSD与PTS发生的关系。结果 39例(27.3%)NMOSD患者有PTS表现,其中37/39例(94.9%)与脊髓病变相关,25/37例(67.6%)于首次脊髓炎发作后出现;伴发PTS的NMOSD患者发病年龄、年复发率及脊髓炎为首发症状的比例较无伴发PTS的NMOSD患者高(P0.05);两者脊髓病变分布、EDSS评分等则差异无显著性(P0.05)。结论 PTS多伴发于年龄较大及以脊髓炎发作为主的NMOSD患者,伴发PTS患者的复发风险可能高于无伴发PTS患者。     

视神经脊髓炎谱系疾病复发相关因素的临床研究

目的研究视神经脊髓炎谱系疾病(NMOSD)的复发率及其临床特征,分析多因素与NMOSD复发的相关性。方法分析2009年7月29日~2015年12月31日就诊于本院神经内科确诊73例NMOSD患者的临床、实验室检查及扩展残疾状态量表(EDSS)评分等资料。运用卡方检验、Logistic回归分析等统计学方法分析影响NMOSD复发的危险因素。结果 73例NMSOD患者中,男性12例,女性61例,男女比例约1∶5,首次发病中位年龄38岁。血清水通道蛋白-4抗体(AQP4-Ab)阳性检出率84.9%(62/73),血清其他自身免疫抗体阳性72.6%(53/73),脑脊液(CSF)AQP4-Ab阳性检出率45.1%(23/51),CSF蛋白升高率43.9%(25/57)。有序Logistic回归分析结果显示血清AQP4-Ab(OR=14.5561,95%CI 0.120~2.809)、自身免疫抗体(OR=4.3281,95%CI 0.684~4.673)阳性可能是NMOSD发病3 y内复发的独立危险因素。结论血清AQP4-Ab及其他自身免疫抗体阳性可能与NMOSD发病3 y内复发频次呈正相关。     

视神经脊髓炎谱系病患者疼痛的初步研究

目的初步探讨视神经脊髓炎谱系病(NMOSD)患者相关的疼痛问题。方法收集57例NMOSD患者和51例多发性硬化(MS)患者的临床资料,采用数字疼痛强度量表(NRS)对患者疼痛程度及部位进行评估,对比分析两组疼痛发生情况、严重程度、部位及治疗情况。结果 NMOSD组患者疼痛发生率明显高于MS组患者(63.16%vs.35.29%,χ2=8.359,P=0.004)。NMOSD组患者痛性痉挛发生率与MS组差异无统计学意义(24.56%vs.11.76%,χ2=2.921,P=0.087)。NMOSD组患者的疼痛评分在0~8分,以中度疼痛为主[20例(55.56%)],MS组患者的疼痛评分在0~7分,以轻度疼痛为主[11例(61.11%)],但两组间轻度疼痛与中重度疼痛患者比例差异无统计学意义(36.11%vs.61.11%,63.89%vs.38.89%,χ2=3.038,P=0.081)。NMOSD组[16例/36例(44.44%)]及MS组[6例/18例(33.33%)]患者的疼痛部位均以躯干部位最常见。Logistic逐步回归分析显示NMOSD患者的疼痛程度与患者的性别、年龄、病程、发作次数、NMO-IgG及EDSS评分无相关性。结论疼痛在NMOSD患者中十分常见,其疼痛应受到重视,并应积极对症治疗。     

以脑干症状起病的视神经脊髓炎谱系疾病32例临床特征及预后分析

目的研究以脑干症状起病的视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)的临床特点、影像学表现及预后情况。方法收集2010年1月1日-2017年6月1日郑州大学第一附属医院收治的符合NMOSD诊断标准的345例视神经脊髓炎患者的临床病例资料,对其中32例以脑干症状起病的NMOSD患者的临床特点、影像学表现及预后情况进行分析。结果 32例患者中女性30例,男性2例,发病年龄15~65岁,平均发病年龄36.5岁。22例表现为顽固性恶心、呕吐及呃逆,10例表现为头晕、复视及共济失调,此外尚有个别病例伴有饮水呛咳、吞咽困难、四肢无力、头面痛等临床表现。头部MRI示:病变多位于延髓背侧、脑干背侧、四脑室及中脑导水管周围。32例患者的EDSS(Expanded disability status scale)评分及复发指标明显高于其他患者(P0.05)。结论脑干症状可以是NMOSD的首发症状,常见的表现包括恶心呕吐、顽固性呃逆、头晕、复视、共济失调等,病变多位于脑干室管膜周围,临床漏诊及误诊率高,且预后情况相对较差,复发率较高。     

托珠单抗治疗难治性视神经脊髓炎谱系疾病的疗效与安全性初步观察

目的观察托珠单抗治疗难治性视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)的疗效与安全性。方法纳入6例血清水通道蛋白抗体(anti-aquaporin-4 antibody,AQP4-Ig G)阳性且经糖皮质激素联合硫唑嘌呤治疗疗效不佳的NMOSD患者,予托珠单抗治疗(8 mg/kg,每个月1次静脉滴注,连续治疗1年以上),前瞻性评估患者的年复发率(annualized relapse rate,ARR)、扩展残疾状态量表评分(expanded disability status scale score,EDSS)及疼痛数字评价量表(numerical rating scales,NAS),评价托珠单抗的疗效及副作用。结果 5例女性和1例男性,年龄34~63岁,病程16~96个月,托珠单抗治疗6~16次,经治疗后平均ARR由1.28±0.58降为0(P0.05),平均EDSS评分由5.50±2.81降为3.83±3.48(P0.05),平均NAS评分由5.83±3.06降为2.5±2.07(P0.05),无严重不良反应发生。结论托珠单抗可改善难治性NMOSD患者的年复发率及神经病理性疼痛,可能是一种治疗难治性NMOSD的有效药物。     

CD19+CD24~(high) CD38~(high)调节性B细胞及相关细胞因子在视神经脊髓炎谱系疾病患者外周血中的变化及意义

目的检测NMOSD患者外周血中CD19~+CD24~(high)CD38~(high)Breg及其细胞因子IL-10、TGF-β水平,探讨CD19~+CD24~(high)CD38~(high)Breg、IL-10、TGF-β在NMOSD急性期的变化及可能作用;检测NMOSD患者外周血GFAP水平,结合EDSS评分,探讨GFAP与疾病严重程度的相关性。方法选取2016年12月-2018年1月就诊于河北医科大学第二医院神经内科NMOSD急性期患者27例为实验组,性别、年龄相匹配的20例健康人为对照组。入组的NMOSD患者进行EDSS评分、流式细胞术检测外周血CD19~+CD24~(high)CD38~(high)Breg数量,PCR法检测外周血IL-10、TGF-β、GFAP的mRNA水平,并与健康人比较。结果 NMOSD组CD19~+CD24~(high)CD38~(high)Breg水平较对照组下降,差异有统计学意义,CD19~+CD24~(high)CD38~(high)Breg与EDSS之间无线性关系。NMOSD组IL-10、TGF-β的mRNA水平较对照组下降,差异有统计学意义。NMOSD组GFAP水平较对照组升高,差异有统计学意义。GFAP与EDSS之间无线性关系。结论 CD19~+CD24~(high)CD38~(high)Breg在NMOSD急性期发病中数量和功能上均受损,血清GFAP水平增高,但不能反应复发患者疾病残疾程度。     

视神经脊髓炎患者的甲状腺功能、抗体改变及并发自身免疫性甲状腺病的分析

目的 探讨37例视神经脊髓炎(NMO)患者甲状腺功能及自身抗体的改变,分析并发自身免疫性甲状腺病(AITD)的NMO患者的临床特点.方法 回顾性分析2006～2011年首都医科大学宣武医院收治的37例视神经脊髓炎患者的甲状腺功能( TSH、T3、T4、FT3、FT4)和自身甲状腺抗体(TPOAb、TGAb),38名健康体检者作为对照组,比较2组研究对象的甲状腺功能和自身甲状腺抗体有无差异.同时比较并发AITD的NMO患者(5例)与无AIDT的NMO患者(32例)的临床特征、脊髓磁共振检查结果.结果 (1)NMO组病人TSH、T3、FT3、T4和FT4与健康对照组之间差异无统计学意义;NMO组病人甲状腺自身抗体TPOAb、TGAb均高于健康对照组,两组之间差异有统计学意义(P值均＜0.05).(2)5例(13.5％)并发AITD的NMO患者发病与第一次复发间隔的时间和第一次发作的EDSS评分,与无AITD的NMO患者之间的差异有统计学意义(P值均＜0.05),而两组病人病程持续时间、发作次数、第一年复发阳性率、脊髓MRI病变节段数之间的差异均无统计学意义.结论 NMO患者的甲状腺功能正常,自身甲状腺抗体升高.并发AITD的NMO患者首次发作较轻,发病与首次复发间隔长,但不影响患者临床的总体病程.     

利妥昔单抗治疗5例难治性视神经脊髓炎谱系疾病长期疗效观察

目的观察利妥昔单抗治疗难治性视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)的长期临床疗效及安全性。方法回顾性分析作者医院神经内科应用利妥昔单抗治疗的5例难治性NMOSD患者(经糖皮质激素或丙种球蛋白治疗无效)的用药经过和临床情况,比较治疗前后年复发率(ARR)和扩展残疾状态量表评分(EDSS)的差异,评价利妥昔单抗的疗效和副作用。结果应用利妥昔单抗治疗前,5例患者的EDSS和ARR中位数(四分位数)分别为8.5(1.25)分和1(2.12)次;经利妥昔单抗治疗后,随访期间(随访时间中位数为52个月)5例患者的EDSS和ARR中位数(四分位间距)分别为2.5(5.75)分和0(0.35)次,治疗前后患者的EDSS和ARR差异有统计学意义。治疗期间仅1例患者出现轻微不良反应,无严重不良反应发生。结论利妥昔单抗可改善难治性NMOSD患者的长期临床症状,可能是一种治疗难治性NMOSD的有效药物。     

A clinical and radiological profile of neuromyelitis optica and spectrum disorders in an Indian cohort

Background:

There is insufficient data on the clinical and radiological features of neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) from India.

Objective:

The objective of the following study is to examine the clinico-radiological features of NMO and NMOSD in an Indian cohort.

Materials and Methods:

This retrospective study included 44 consecutive patients who (1) satisfied the 2006 Wingerchuk criteria for NMO (16 seropositive and 7 seronegative); or (2) had isolated or recurrent optic neuritis (ON) with seropositivity (n = 4); or (3) had isolated or recurrent myelitis with seropositivity (n = 17).

Results:

The female:male ratio was 7.8:1 with median age of onset 26.5 (range 8-72). Annualized relapse rate (ARR) was comparable across all groups (F [3, 40] = 0.938 and P = 0.431). Various presentations other than ON and myelitis were noted. All 40 patients with myelitis had spinal cord lesions involving ≥3 vertebral segments during the course of the disease. Cervicomedullary involvement was seen in 32.5% (13/40) patients. Brain magnetic resonance imaging was available for 40 patients; eight of these (20%) had brain lesions in locations described in multiple sclerosis (MS), 27.5% (11/40) had lesions at sites unusual for MS and 52.5% (21/40) had normal brain imaging.

Conclusion:

NMO and NMOSD patients in this cohort have comparable ARR regardless of clinical presentation, supporting the emerging trend of treating all patients with immunotherapeutic agents at an early stage. Varied presentations seen in NMO and NMOSD highlight the need for a high index of suspicion for NMO in demyelinating episodes not classical for MS.     

HIV感染合并视神经脊髓炎谱系疾病一例报道并文献复习

目的总结HIV感染合并视神经脊髓炎谱系疾病(NMOSD)患者的临床特点,以提高临床对其认识。方法报道作者医院收治的1例以视神经脊髓炎症状起病的AIDS患者的临床资料,并结合文献进行复习。结果文献检索HIV感染合并NMOSD患者7例,结合本文报道的1例共8例,年龄8~55岁,平均(39.1±14.4)岁,其中男5例、女3例。8例患者均发生脊髓炎,7例发生视神经炎。血CD4+T细胞降低3例(3/8)。脑脊液(CSF)细胞学检测异常4例(4/8),表现为白细胞轻度升高,以淋巴细胞炎性反应为主,6例蛋白水平升高(6/8)。血清水通道蛋白4抗体阳性3例(3/7)。CSF寡克隆区带阳性3例(3/5)。MRI检查显示颅内多发病灶2例(2/8),颈段或胸段脊髓病灶8例(8/8),其中4例为颈髓至胸髓连续长节段病灶。8例患者接受免疫治疗,其中7例接受糖皮质激素治疗,3例(3/8)肌力完全恢复或能独立行走,1例(1/8)肌力部分恢复,4例(4/8)肌力无明显恢复,3例(3/7)视力恢复至粗测正常,1例(1/7)视力部分恢复,3例(3/7)失明。复发5例,其中4例1年内复发。1例死亡。结论HIV感染合并NMOSD患者致残率高,易复发,部分患者免疫治疗有效。     

脑脊液MOG抗体阳性的视神经脊髓炎谱系疾病患者的临床特征

目的探讨MOG抗体阳性的NMOSD患者的临床特点。方法选择29例NMOSD患者,根据血清AQP-4抗体以及脑脊液MOG抗体检测结果,分为MOG抗体阳性、AQP4抗体阳性的NMOSD(剔除双阳性者),同时选择13例MS患者作为对照。回顾性分析上述三组患者临床信息,统计归纳其临床特点。结果 29例NMOSD患者中血清AQP4抗体阳性者11例,脑脊液MOG抗体阳性者8例。36.4%(4例/11例)AQP4抗体阳性、62.5%(5例/8例)MOG抗体阳性NMOSD患者,以及7.7%(1例/13例)MS患者合并脊髓炎与视神经炎,三组间差异有统计学意义(χ~2=7.128,P=0.028),其中MOG抗体阳性NMOSD患者较MS患者更易合并视神经炎(χ~2=7.289,P=0.014)。MOG抗体阳性NMOSD患者缓解期EDSS分数低于AQP4抗体阳性NMOSD患者[3.50(2.50,4.00),4.00(3.50,6.00),Z=-2.379,P=0.020]。MOG抗体阳性NMOSD脊髓病灶多表现为多发的长节段脊髓病灶,50%(4例/8例)MOG抗体阳性脊髓病灶个数大于1个,与MS组无明显差异,而AQP4抗体阳性组均为单个病灶。MOG抗体阳性NMOSD脊髓病灶长度较AQP4抗体阳性组短[分别(3(2,3)个椎体、4(3,5)个椎体,Z=-2.499,P=0.012],较MS组[(1.25(1,1.5)个椎体]长(Z=-3.447,P0.001)。8例MOG抗体阳性患者中5例存在颅内病灶,3例表现为NMOSD样颅内病灶,余2例表现为MS样颅内病灶,其病灶形态及部位与AQP4抗体阳性组无明显差异,而与MS组存在差异。结论 MOG抗体阳性NMOSD合并视神经炎的患者较多,临床残障程度较轻,预后较好,脊髓病灶为多发的长节段脊髓病灶;颅内病灶的形态及部位与MS无明显差异。     

光相干连续断层成像术在视神经脊髓炎谱系病的临床应用

目的探讨光相干连续断层成像术(OCT)在视神经脊髓炎谱系病(NMOSD)的临床应用。方法利用OCT对49例NMOSD患者(NMOSD组)和1 5例健康对照志愿者(对照组)视乳头周围视网膜神经纤维层(RNFL)厚度进行比较;NMOSD组再根据是否伴视神经炎、水通道蛋白抗体状态(AQP4-IgG)等对NMOSD组分为伴视神经炎(NMOSD-ON)亚组(34例);不伴视神经炎(NMOSD-NON)亚组(15例)。比较各亚组的RNFL厚度差异;采用扩展病残状态评分(EDSS)评价神经功能缺损程度,分析EDSS与RNFL是否存在相关性。结果 NMOSD患者受累眼的RNFL各象限厚度与未受累眼和对照组比较显著变薄(P0.01);NMOSD未受累眼与对照组比较,RNFL厚度差异无显著性(P0.05)。AQP4-IgG阳性或阴性NMOSD-ON亚组的RNFL厚度比较差异无显著性(P0.05);RNFL厚度变化与NMOSD残障的严重程度无相关性(P0.05)。结论 RNFL变薄仅在NMOSD受累眼表现,厚度变化与AQP4-IgG无关,OCT可能有助于临床对NMOSD诊疗提供参考指标。     

Perivascular Enhancement in a Patient with Neuromyelitis Optica Spectrum Disease during an Optic Neuritis Attack

We present a case with neuromyelitis optica spectrum disease (NMOSD) who had perivascular enhancement during an optic neuritis attack. Cloud‐like enhancement, pencil‐thin enhancement, and leptomeningeal enhancement have been defined as specific enhancement patterns to neuromyelitis optica (NMO). Perivascular enhancement has not been described before in NMO/NMOSD. This finding suggests that perivascular enhancement may also be seen in NMO/NMOSD patients.     

Neuromyelitis optica associated with myasthenia gravis: characteristic phenotype in Japanese population

We report two female patients with neuromyelitis optica (NMO, Devic's syndrome) following or coincidental with myasthenia gravis (MG). Their illnesses were characterized by subacute myelitis with optic neuritis, high serum levels of muscle acetylcholine receptor antibody, and autoimmune thyroid disease. Both patients fulfilled the clinical criteria of NMO, however, NMO-IgG, autoantibody against aquaporin-4 water channel, was absent from their sera. Both NMO and MG are relatively rare diseases. The considerable coincidence of these two disorders suggests that there is a subgroup of patients with NMO having a common immunological pathogenesis with MG.     

Espectro de neuromielitis óptica: ¿seropositivo para la anticuaporina es una entidad diferente de los pacientes que son seronegativos? Una perspectiva de Colombia

IntroductionNeuromyelitis optica (NMO) is an inflammatory disease of the central nervous system characterised by attacks of optic neuritis and longitudinally extensive transverse myelitis. The discovery of anti–aquaporin-4 (anti-AQP4) antibodies and specific brain MRI findings as diagnostic biomarkers have enabled the recognition of a broader and more detailed clinical phenotype, known as neuromyelitis optica spectrum disorder (NMOSD).ObjectiveThis study aimed to determine the demographic and clinical characteristics of patients with NMO/NMOSD with and without seropositivity for anti-AQP4 antibodies, in 2 quaternary-level hospitals in Bogotá.MethodsOur study included patients > 18 years of age and diagnosed with NMO/NMOSD and for whom imaging and serology results were available, assessed between 2013 and 2017 at the neurology departments of hospitals providing highly complex care. Demographic, clinical, and imaging data were gathered and compared in patients with and without seropositivity for anti-AQP4 antibodies.ResultsThe sample included 35 patients with NMO/NMOSD; the median age of onset was 46.5 years (P25-P75, 34.2-54.0); most patients had sensory (n = 25) and motor manifestations (n = 26), and a concomitant autoimmune disease was identified in 6. Twenty patients were seropositive for anti-AQP4 antibodies. Only age and presence of optic nerve involvement showed statistically significant differences between groups (p = .03).ConclusionsClinical, imaging, and laboratory variables showed no major differences between patients with and without anti-AQP4 antibodies, with the exception of age of onset and presence of optic nerve involvement (uni- or bilateral); these factors should be studied in greater detail in larger populations.     

2015年视神经脊髓炎谱系疾病诊断标准临床应用评估

目的 评估2015年视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)诊断标准的临床应用.方法 回顾性分析作者医院2008-3-2016-8收治的605例中枢神经系统脱髓鞘疾病患者临床资料,并分别采用2015年NMOSD诊断标准及2006年NMO诊断标准对其进行诊断分析.结果 共有176例患者符合2015年NMOSD诊断标准,其中108例(61.4％)符合2006年NMO诊断标准.AQP4-IgG阳性患者139例(79.0％).当假设该139例患者AQP4-IgG水平未知时,共有91例(65.5％)符合2015年NMOSD诊断标准,而仅有55例(39.6％)满足2006年NMO诊断标准(P＜0.05).176例患者中有39例同时满足2006年NMO诊断标准和201 5年NMOSD诊断标准且以临床孤立病灶起病,在疾病早期通过2006年NMO诊断标准尚不能与多发性硬化相鉴别,但已符合2015年NMOSD诊断标准.在核心症状方面,NMOSD患者中以急性脊髓炎(50.6％)和视神经炎(31.8％)起病者所占比例最高,其次为最后区综合征(5.7％).结论 2015年NMOSD诊断标准较2006诊断标准扩大了疾病的诊断范围,且在AQP4-IgG水平未知的情况下仍具有更高的诊断敏感性,对于疾病早期与多发性硬化的鉴别中具有重要作用,且更加强调了疾病的核心症状.     

New Insights into Neuromyelitis Optica

Neuromyelitis optica (NMO) is an idiopathic inflammatory disorder of the central nervous system (CNS) that preferentially affects the optic nerves and spinal cord. In Asia, NMO has long been considered a subtype of multiple sclerosis (MS). However, recent clinical, pathological, immunological, and imaging studies have suggested that NMO is distinct from MS. This reconsideration of NMO was initially prompted by the discovery of a specific antibody for NMO (NMO-IgG) in 2004. NMO-IgG is an autoantibody that targets aquaporin-4 (AQP4), the most abundant water channel in the CNS; hence, it was named anti-AQP4 antibody. Since it demonstrated reasonable sensitivity and high specificity, anti-AQP4 antibody was incorporated into new diagnostic criteria for NMO.The spectrum of NMO is now known to be wider than was previously recognized and includes a proportion of patients with recurrent, isolated, longitudinally extensive myelitis or optic neuritis, and longitudinally extensive myelitis or optic neuritis associated with systemic autoimmune disease or with brain lesions typical of NMO. In this context, a new concept of "NMO spectrum disorders" was recently introduced. Furthermore, seropositivity for NMO-IgG predicts future relapses and is recognized as a prognostic marker for NMO spectrum disorders. Humoral immune mechanisms, including the activation of B-cells and the complement pathway, are considered to play important roles in NMO pathogenesis. This notion is supported by recent studies showing the potential pathogenic role of NMO-IgG as an initiator of NMO lesions. However, a demonstration of the involvement of NMO-IgG by the development of active immunization and passive transfer in animal models is still needed. This review focuses on the new concepts of NMO based on its pathophysiology and clinical characteristics. Potential management strategies for NMO in light of its pathomechanism are also discussed.     

Th17 cells in neuromyelitis optica spectrum disorder: a review

Neuromyelitis optica spectrum disorder (NMOSD) has been identified as a central nervous system (CNS) autoimmune inflammatory disorder, which has been recognized as a B cell-mediated humoral immune disease. However, cases have been reported indicating that some of the neuromyelitis optica (NMO) patients have been resistant to B cell-related treatments. Recently, more and more evidence has shown that T cell-mediated immunity may take part in the pathogenesis of NMOSD, especially in the Th17 phenotype. In our PUBMED search, we used the following keywords: Th17 cell, Th17 cell-related cytokines, T cells, B cells, B cell-related productions, NMO, NMOSD, recurrent/bilateral optic neuritis, recurrent transverse myelitis and longitudinally extensive transverse myelitis. We systemically reviewed the role of Th17 cells and Th17 cell-related cytokines in NMOSD. We found that Th17 cells and Th17-related cytokines, such as IL-6, IL-1β, IL-17, IL-21, IL-22, IL-23 and TGF-β, are not only directly involved in the pathogenesis but also collaborated with B cells and B cell-related antibody production to induce CNS lesions. Th17 cell-related therapy has also been reviewed in this article, and the data suggested that Th17 may be a new therapeutic target of NMOSD.