视神经脊髓炎与多发性硬化的临床表现和视神经脊髓炎IgG抗体阳性率的对比

目的 比较视神经脊髓炎(NMO)和多发性硬化(MS)在临床表现、辅助检查等方面的不同;比较NMO和MS等脱髓鞘疾病患者血清NMO-IgG抗体的阳性率,判断该抗体能否作为鉴别诊断的一项实验室依据.方法 对34例NMO、22例MS、24例高危综合征、5例临床孤立综合征以及35例其他神经科疾病患者进行NMO-IgG检测,并对其中NMO、MS患者的人口学、临床表现、免疫学指标、脑脊液、头颅MRI等资料进行对比.结果 NMO的起病年龄较MS大且年龄跨度更广;从年复发率和进展指数来看,NMO更为严重,预后更差;NMO长节段脊髓损害者比MS多.NMO-IgG在NMO组和高危综合征组的阳性率分别为58.8%(20/34)和45.8%(11/24),高于MS组(1/22)、临床孤立综合征组(1/5)和其他疾病组(1/35;X2=37.2,P<0.01).NMO-IgG阳性率与脊髓病变长度相关.结论 NMO和MS在临床表现、辅助检查等方面都有所不同,提示NMO与MS可能是2种不同的疾病.NMO-IgG在NMO患者中的阳性率高于MS患者,可以作为鉴别诊断的一项实验室依据.     

视神经脊髓炎与多发性硬化的临床表现和视神经脊髓炎IgG抗体阳性率的对比

目的 比较视神经脊髓炎(NMO)和多发性硬化(MS)在临床表现、辅助检查等方面的不同;比较NMO和MS等脱髓鞘疾病患者血清NMO-IgG抗体的阳性率,判断该抗体能否作为鉴别诊断的一项实验室依据.方法 对34例NMO、22例MS、24例高危综合征、5例临床孤立综合征以及35例其他神经科疾病患者进行NMO-IgG检测,并对其中NMO、MS患者的人口学、临床表现、免疫学指标、脑脊液、头颅MRI等资料进行对比.结果 NMO的起病年龄较MS大且年龄跨度更广;从年复发率和进展指数来看,NMO更为严重,预后更差;NMO长节段脊髓损害者比MS多.NMO-IgG在NMO组和高危综合征组的阳性率分别为58.8%(20/34)和45.8%(11/24),高于MS组(1/22)、临床孤立综合征组(1/5)和其他疾病组(1/35;X2=37.2,P<0.01).NMO-IgG阳性率与脊髓病变长度相关.结论 NMO和MS在临床表现、辅助检查等方面都有所不同,提示NMO与MS可能是2种不同的疾病.NMO-IgG在NMO患者中的阳性率高于MS患者,可以作为鉴别诊断的一项实验室依据.     

视神经脊髓炎与多发性硬化的临床表现和视神经脊髓炎IgG抗体阳性率的对比

目的 比较视神经脊髓炎(NMO)和多发性硬化(MS)在临床表现、辅助检查等方面的不同;比较NMO和MS等脱髓鞘疾病患者血清NMO-IgG抗体的阳性率,判断该抗体能否作为鉴别诊断的一项实验室依据.方法 对34例NMO、22例MS、24例高危综合征、5例临床孤立综合征以及35例其他神经科疾病患者进行NMO-IgG检测,并对其中NMO、MS患者的人口学、临床表现、免疫学指标、脑脊液、头颅MRI等资料进行对比.结果 NMO的起病年龄较MS大且年龄跨度更广;从年复发率和进展指数来看,NMO更为严重,预后更差;NMO长节段脊髓损害者比MS多.NMO-IgG在NMO组和高危综合征组的阳性率分别为58.8%(20/34)和45.8%(11/24),高于MS组(1/22)、临床孤立综合征组(1/5)和其他疾病组(1/35;X2=37.2,P<0.01).NMO-IgG阳性率与脊髓病变长度相关.结论 NMO和MS在临床表现、辅助检查等方面都有所不同,提示NMO与MS可能是2种不同的疾病.NMO-IgG在NMO患者中的阳性率高于MS患者,可以作为鉴别诊断的一项实验室依据.     

临床孤立综合征转归为视神经脊髓炎的相关因素分析

目的探讨临床孤立综合征(CIS)转归为视神经脊髓炎(NMO)的影响因素。方法收集2004-09-2011-09就诊于作者医院神经内科CIS患者109例。回顾性分析所有患者首次发病时头颅和脊髓MRI特点及临床表现。采用酶联免疫吸附法(ELISA)检测血清水通道蛋白4抗体(AQP4-Ab)水平,另备30份健康者血清作为健康对照组,以高于健康对照组血清AQP4-Ab浓度的均值+3倍标准差者为阳性。结果 (1)随访0.5～7年,中位数为3.0年,四分位数间距为4.6年,转归为NMO 46例,转归为多发性硬化(MS)29例,其余仍是CIS,包括24例脊髓炎,10例视神经炎(ON)。(2)转归为NMO组血清AQP4-Ab水平明显高于MS组、脊髓炎组、ON组和健康对照组(P<0.05)。(3)转归为NMO组AQP4-Ab阳性率为63.03%(29/46),高于转归为MS组的13.79%(4/29)、脊髓炎组的29.17%(7/24)、ON组的20.00%(2/10),差异均有统计学意义(P<0.05)。(4)多因素分析结果提示:AQP4-Ab阳性、NMO颅内典型病灶、脊髓损伤>3个节段、扩展残疾状态量表(EDSS)与CIS转归为NMO有关。结论 AQP4-Ab阳性、NMO颅内典型病灶或者脊髓损伤>3个节段、EDSS评分对预测CIS转归为NMO有临床价值。     

水通道蛋白4启动子基因多态性与多发性硬化、视神经脊髓炎遗传易患性的关系

目的 探讨水通道蛋白4(AQP4)启动子区基因多态性与我国南方多发性硬化(MS)、视神经脊髓炎(NMO)患者血清抗AQP4抗体水平及遗传易患性的关系.方法 收集18例NMO、38例MS、13例复发性脊髓炎(RM)、6例复发性视神经炎(RON)患者及39名对照,PCR扩增AQP4外显子0及外显子1启动子基因(即AQP4-promoter0和AQP4-promoter 1),并行DNA测序.结果 共发现14个AQP4-promoter0及6个AQP4-promoter 1基因多态性位点.血清抗AQP4抗体阳性患者AQP4-promoter 0中-1003 bp多态性位点(A突变为G)发生率比血清抗AQP4抗体阴性患者(13/18与20/45,P=0.046)及对照组(13/18与10/39,P=0.001)高,差异有统计学意义.血清抗AQP4抗体阳性患者及血清抗AQP4抗体阴性患者AQP4-promoter 1中- 401 bp与-400 bp之间多态性位点(插入1个C)发生率均比对照组高(5/16与0/28,P=0.008; 8/38与0/28,P=0.027),差异有统计学意义.NMO及MS患者-1003bp多态性位点及-401 bp与-400 bp之间多态性位点发生率均比对照组高,差异有统计学意义(NMO:11/18与10/39,P=0.010;4/15与0/28,P=0.020;MS:19/38与10/39,P=0.027;8/34与0/28,P=0.018).结论 AQP4启动子区基因存在多态性位点,且与NMO、MS易患性有一定的关系;AQP4外显子0启动子中- 1003 bp多态性位点可能与血清抗AQP4抗体的出现有关.     

视神经脊髓炎患者血清及脑脊液中B淋巴细胞活化因子的表达水平与临床意义

目的 探讨视神经脊髓炎患者血清及脑脊液中B淋巴细胞活化因子的表达水平及其临床意义。方法 选取2011年1月-2015年1月本院收治的视神经脊髓炎(NMO)患者50例及多发性硬化(MS)患者50例,将其分别作为NMO组与MS组,另选取同期于本院进行体检的非炎性神经系统疾病患者50例作为对照组,对3组血清及脑脊液中的B淋巴细胞活化因子(BAFF)水平进行检测。结果 与对照组比较,NMO组与MS组血清中BAFF水平均无明显变化(P>0.05),而NMO组与MS组脑脊液中BAFF水平均明显升高(P<0.05); 与MS组比较,NMO组脑脊液中BAFF水平明显升高(P<0.05)。NMO组与MS组脑脊液中BAFF水平与EDSS评分呈正相关,即脑脊液中BAFF水平随EDSS评分升高而升高(r=0.887,0.885,P<0.01)。结论 视神经脊髓炎患者脑脊液中的B淋巴细胞活化因子水平较高,可能是诊断视神经脊髓炎的重要标志物,对疾病严重程度的判定具有重要的临床意义。     

早期多发性硬化和视神经脊髓炎高危综合征临床特点的比较

目的 比较早期多发性硬化(MS)和视神经脊髓炎(NMO)高危综合征患者的临床特点差异. 方法 回顾性收集广州医科大学附属第二医院神经内科自2004年1月至2013年8月收治的早期MS患者49例和NMO高危综合征患者30例(包括长节段的横贯性脊髓炎22例、复发性视神经炎8例)的临床资料、影像学检查结果、血清中NMO-IgG抗体情况等进行分析和比较. 结果 早期MS患者和NMO高危综合征患者的EDSS评分、病程以及感觉症状、脑干症状比例比较差异均有统计学意义(P＜0.05).早期MS组患者中病灶数＞9个的比例(77.6％)高于NMO高危综合征组患者,差异有统计学意义(P＜0.05).NMO高危综合征组患者在脑脊液蛋白异常率(19例,63.3％)、蛋白水平[(0.57±0.45) g/L]以及脑脊液白细胞计数异常率(19例,63.3％)、白细胞计数[中位数为24.317个/mm3 (0～274个)]方面与早期MS患者组比较差异有统计学意义(P＜0.05).10例NMO高危综合征患者中6例长节段横贯性脊髓炎患者NMO-IgG阳性,13例早期MS患者中2例出现阳性反应,阳性率比较差异有统计学意义(P＜0.05). 结论 早期MS患者和NMO高危综合症的临床特点明显不同,这些差异对早期鉴别MS与NMO具有一定意义.     

水通道蛋白4抗体检测对视神经脊髓炎的诊断价值

目的:探讨水通道蛋白4(AQP4)抗体检测对视神经脊髓炎(NMO)的诊断价值.方法:采用表达人AQP4基因的人胚肾(HEK)293细胞株分别对35例NMO患者(NMO组)、14例NMO高危综合征患者(HRS组)、37例多发性硬化患者(MS组)、23例其他脱髓鞘疾病患者(ODD组)及75例其他神经系统疾病患者(MD组)的血标本进行AQP4抗体检测,比较各组抗体的阳性率.结果:NMO组中31例患者(88.6%)及HRS组中6例患者(42.9%n)AQP4抗体检测为阳性;MS组、ODD组和MD组患者AQP4抗体检测均为阴性.NMO组AQP4抗体检测阳性的敏感性为88.6%,95%CI,73.11%-96.79%;HRS组AQP4抗体检测阳性的敏感性为42.9%,95%CI:16.97%-68.83%;NMO患者AQP4抗体检测阳性的特异性为100%.结论:AQP4抗体检测对NMO及其疾病谱早期确诊有重要意义,且具有较高的敏感性和特异性.     

构建M23-AQP4稳定表达的HEK293细胞应用于血清抗AQP4抗体检测的研究

目的构建M23-AQP4稳定表达HEK293细胞(HEK293-M23-AQP4)并用于抗AQP4抗体检测,以探索临床可行的抗AQP4抗体检测方法。方法用磷酸钙转染试剂将pEGFP-N1-M23-AQP4质粒转入HEK293细胞,通过G418筛选HEK293-M23-AQP4,以细胞间接免疫荧光法(CBA)检测M23-AQP4表达及分布。以HEK293-M23-AQP4为底物的CBA法检测视神经脊髓炎(NMO)6例、多发性硬化(MS)16例、其他脱髓鞘疾病(视神经炎、脊髓炎、吉兰-巴雷综合征、急性播散性脑脊髓炎)30例、非脱髓鞘性疾病患者10例血清抗AQP4抗体及其滴度,并比较4组抗体阳性率,计算抗AQP4抗体诊断NMO的敏感性,分别以非NMO的脱髓鞘疾病和非脱髓鞘疾病作对照计算抗AQP4抗体诊断NMO的特异性。将HEK293-M23-AQP4细胞于室温、4℃、-20℃保存4周,分别作为底物检测经首次检测所得抗AQP4抗体阳性标本并随机选取5例抗体阴性标本的抗AQP4抗体及滴度,比较其阳性率及滴度变化;将上述首次检测所得抗AQP4抗体阳性标本及5例抗体阴性标本反复冻融3次后分别于室温、4℃、-20℃保存1周后,检测其抗AQP4抗体及滴度,比较阳性率和滴度变化。结果 HEK293-M23-AQP4构建成功,M23-AQP4主要表达在细胞膜上。NMO患者抗AQP4抗体阳性率达83.3%(5/6),显著高于MS患者〔6.3%(1/16)〕、其他脱髓鞘疾病〔3.3%(1/30)〕和非脱髓鞘性疾病〔0.0%(0/10)〕(均P0.01);抗AQP4抗体诊断NMO的敏感性为83.3%(5/6),以非NMO的脱髓鞘疾病作对照时,其诊断NMO特异性为95.6%(44/46),以非脱髓鞘疾病作对照时,其诊断NMO特异性为100%(10/10)。HEK293-M23-AQP4于不同温度保存后所检测抗AQP4抗体阳性率和滴度与首次检测比较均无统计学意义(均P=1.0)。血清标本4℃及-20℃保存1周后所检测抗AQP4抗体阳性率和滴度与首次检测比较差异均无统计学意义(均P=1.0);室温保存1周后抗体滴度(1∶400、1∶400、1∶3200、1∶6400、1∶6400、1∶12800、1∶51200)与首次检测(1∶800、1∶1600、1∶12800、1∶25600、1∶25600、1∶51200、1∶120400)比较均显著下降(均P0.01),但阳性率差异无统计学意义(P=1.0)。结论成功构建HEK293-M23-AQP4细胞。以此细胞为底物的CBA法检测血清抗AQP4抗体对诊断NMO的敏感性和特异性较高,且对标本存储条件要求较低。     

视神经脊髓炎患者血清神经丝重链SMI-35临床意义初探

目的探讨视神经脊髓炎(NMO)患者血液神经丝重链(NfH)的水平及其临床意义。方法选取27例NMO患者、20例多发性硬化(MS)患者以及健康志愿者26名,采用ELISA法检测各组血清NfHSMI-35水平及NMO患者血清水通道蛋白抗体(AQP4-IgG)水平,观察NfH在NMO、MS和健康人群间以及在NMO患者AQP4-IgG阳性和阴性组间的差异,同时分析NMO患者NfH水平同NMO受累脊髓节段和临床神经功能缺损程度的相关性。结果NMO组、MS组和健康对照组血清NfHSMI-35水平分别为(0.100±0.024)、(0.082±0.012)和(0.064±0.021)ng/mL,NMO组和MS组血清NfHSMI-35水平均高于健康对照组(P0.05),且NMO组NfHSMI-35水平高于MS组(P0.05);NMO患者AQP4-IgG阳性和阴性者间NfHSMI-35水平比较无统计学差异〔(0.106±0.029)ng/mνs.(0.091±0.013)ng/mL,P0.05〕;NMO组NfHSMI-35水平与脊髓受累节段长度呈正相关(r=0.556,P0.01),与临床扩展致残量表评分(EDSS)无相关性(r=0.214,P0.05)。结论 NMO患者外周血NfHSMI-35明显升高,有可能成为NMO的生物标志物,并有可能成为与MS相鉴别的临床监测指标,但仍需要进一步观察和研究。     

中枢神经系统免疫性疾病单细胞组学研究进展

中枢神经系统免疫性疾病包括多发性硬化、视神经脊髓炎谱系疾病、抗髓鞘少突胶质细胞糖蛋白抗体相关疾病和抗N-甲基·D-天冬IK酸受体脑炎等,发病机制复杂,多种中枢神经系统和外周来源细胞参与其中.单细胞组学通过一系列测序技术实现高分辨检测单个细胞水平表达谱,以解析细胞异质性及其功能表型.本文对单细胞测序技术在常见中枢神经系统...     

Comparative clinical characteristics of neuromyelitis optica spectrum disorders with and without medulla oblongata lesions

Brainstem involvement, especially the medulla oblongata (MO), has been reported in neuromyelitis optica spectrum disorders (NMOSDs). The purpose of this study was to investigate retrospectively and compare clinical, laboratory, and imaging features of NMOSDs with and without MO lesions. A total of 170 patients with NMOSDs were enrolled, including 44 patients with MO lesions and 126 patients without MO lesions. Clinical features, laboratory tests, and magnetic resonance imaging findings among these patients were assessed. MO lesions were found in 25.9 % of the NMOSDs patients. The mean duration was 13 months. Patients with MO lesions had a higher Annualized relapse rate and Expanded Disability Status Score Scale. Headache, dizziness, nystagmus, dysarthria, intractable hiccup and nausea, choking cough or dysphagia, movement disorders, and neuropathic pain were more common in MO lesion patients. Patients with MO lesions were more frequently complicated with thyroid diseases. Multiple brain involvement, More importantly, Longitudinally extensive transverse myelitis were more frequently found in patients with MO lesions. MO lesions might be a symbol of more severe neurologic deficits and worse prognosis of NMOSDs.     

A comparative study on clinical characterizations between acute myelitis onset of neuromyelitis optica spectrum disease and idiopathic transverse myelitis

ABSTRACT

Background/Aims

Both of neuromyelitis optica spectrum disease (NMOSDs) and idiopathic transverse myelitis (ITM) could present as acute transverse myelitis. However, long-term immunological treatment and prognosis are different for high recurrence of NMOSDs. In this study, we summarized clinical differences between acute attack myelitis of NMOSDs and ITM, we further screened serum auto-antibodies to help understand the two distinct clinical entities.     

视神经脊髓炎谱系疾病患者脑脊液蛋白水平与致残状况的相关性研究

目的探讨视神经脊髓炎谱系疾病(Neuromyelitis optica disorders,NMOSDs)患者脑脊液蛋白与临床致残状况的关系及其临床意义。方法回顾性分析2010年1月~2016年2月我院神经内科确诊的108例NMOSD患者临床与生化资料,根据临床扩展致残量表评分(Expanded disability status scale,EDSS)将患者分为独立行走受限与不受限组,并比较两组患者间的临床、生化特征;根据脑脊液蛋白值将患者分为脑脊液蛋白正常组与异常组,并比较两组间EDSS评分的变化;分析脑脊液蛋白与临床致残状况的相关性。结果独立行走受限组与不受限组之间患者年龄、二便障碍、外周血白细胞(WBC)、中性粒细胞比(N%)、脑脊液蛋白、脑脊液Ig G较有统计学差异(P0.05);性别、病程、视力损害、AQP4抗体、24 h鞘内合成率、寡克隆带比较无统计学差异(P0.05),两组不同脑脊液蛋白水平与患者EDSS值比较有统计学差异(T=3.13,P=0.002);脑脊液蛋白水平与独立行走受限呈正相关(r=0.286,P0.01)。结论 NMOSD患者脑脊液蛋白值越高,患者致残发生的可能性越大,脑脊液蛋白有助于视神经脊髓炎谱系疾病患者致残状况的病情评估。     

Classification and diagnostic criteria for demyelinating diseases of the central nervous system: Where do we stand today?

The diagnosis of multiple sclerosis (MS) and other demyelinating diseases of the central nervous system is challenging, and although the currently available biological and imaging tools offer considerable support to physicians, these tools often fail to provide a simple and final answer at the time of a first event. Thus, sets of diagnostic criteria have been published and tested on patient cohorts, and are now used in clinical trials and in daily clinical practice. These criteria have evolved over time to take into account physicians’ and patients’ needs, along with emerging paraclinical tests. The different presentations of MS have given rise to the use of a common classification system to identify patient profiles and adapt care protocols accordingly. This article reviews the various classifications of the forms and diagnostic criteria of MS and related syndromes, including neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSDs), acute disseminated (demyelinating) encephalomyelitis (ADEM) and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Also discussed is their validity in the light of the currently available literature.     

肠道微生物在神经系统自身免疫性疾病中的研究进展

肠道微生物是人体最直接的外环境,对维持健康具有重要作用.肠道微生物可维持人体免疫系统稳态,与宿主共生可影响人体营养、代谢和免疫功能.肠道微生物通过免疫系统和内分泌系统与神经系统产生双向联系,即微生物-肠-脑轴.肠道微生物失调引起神经系统及外周异常免疫反应,参与自身免疫性疾病的发病机制.本文综述肠道微生物在多发性硬化、视...     

Neuromyelitis optica and non organ-specific autoimmunity

BACKGROUND: Neuromyelitis optica (NMO) is often associated with other clinical or serological markers of non-organ-specific autoimmunity. OBJECTIVE: To evaluate the relationship between NMO spectrum disorders (NMOSDs), including NMO, longitudinally extensive transverse myelitis, and recurrent optic neuritis, and autoimmune disease. We concentrated on the association with systemic lupus erythematosus (SLE), Sj?gren syndrome (SS), or serological evidence of these disorders, which commonly is a source of diagnostic confusion. DESIGN: Retrospective blinded serological survey. SETTING: Mayo Clinic College of Medicine, Rochester, and Centre Hospitalier Régional Universitaire de Lille. METHODS: Group 1 included 153 US patients with NMOSDs (78 with NMO and 75 with longitudinally extensive transverse myelitis) and 33 control subjects with SS/SLE. Group 2 included 30 French patients with SS/SLE, 14 with NMOSDs (6 with NMO, 6 with longitudinally extensive transverse myelitis, and 2 with recurrent optic neuritis), 16 without NMOSDs, and 4 with NMO without SS/SLE. RESULTS: For group 1, NMO-IgG was detected in 66.7%, antinuclear antibodies in 43.8%, and Sj?gren syndrome A (SSA) antibodies in 15.7% of patients with NMO and longitudinally extensive transverse myelitis. Five NMO-IgG-seropositive patients with NMOSDs had coexisting SLE, SS, or both. Antinuclear antibodies and SSA antibodies were more frequent in NMO-IgG-seropositive patients than in NMO-IgG-seronegative patients (P= .001). For group 2, NMO-IgG was detected in 5 of 14 patients (35.7%) with NMOSDs and SS/SLE and in 2 of 4 patients (50.0%) with NMO without SS/SLE (P= .59). We detected NMO-IgG only in patients with NMOSDs and not in 49 controls with SS/SLE but without optic neuritis or myelitis from the 2 cohorts (P= .01). CONCLUSION: Neuromyelitis optica spectrum disorders with seropositive findings for NMO-IgG occurring with SS/SLE or non-organ-specific autoantibodies is an indication of coexisting NMO rather than a vasculopathic or other complication of SS/SLE.     

The clinical course of neuromyelitis optica (Devic's syndrome).

OBJECTIVES: To evaluate the spectrum of neuromyelitis optica (NMO), including characteristics of the index events (optic neuritis [ON]) and myelitis), neuroimaging, CSF, and serologic studies, and to evaluate the long-term course. METHODS: Review of 71 patients with NMO evaluated at the Mayo Clinic between 1950 and 1997. RESULTS: NMO was either monophasic or relapsing. Patients with a monophasic course (n = 23) usually presented with rapidly sequential index events (median 5 days) with moderate recovery. Most with a relapsing course (n = 48) had an extended interval between index events (median 166 days) followed within 3 years by clusters of severe relapses isolated to the optic nerves and spinal cord. Most relapsing patients developed severe disability in a stepwise manner, and one-third died because of respiratory failure. Features of NMO distinct from "typical" MS included >50 cells/mm3 in CSF (often polymorphonuclear), normal initial brain MRI, and lesions extending over three or more vertebral segments on spinal cord MRI. CONCLUSIONS: Clinical, laboratory, and imaging features generally distinguish neuromyelitis optica from MS. Patients with relapsing optic neuritis and myelitis may have neuromyelitis optica rather than MS. Patients with a relapsing course of neuromyelitis optica have a poor prognosis and frequently develop respiratory failure during attacks of cervical myelitis.     

Profile of multiple sclerosis in the bombay region. On the basis of critical clinical appraisal.

Using internationally accepted strict clinical criteria, 30 patients with probable multiple sclerosis (MS) fron the Bombay region have been described. They were personally studied and followed up over a period of 15 years. In addition, 9 patients with neuromyelitis optica and 6 with a mixed neurological picture of MS and neuromyelitis optica have been described. As in Japan, the neuromyelitis optica type of presentation was relatively more frequent in our material than in the West. In this small series MS was more frequent in patients with a better socio-economic status, in the Parsee community and in patients with a fair complexion, whereas neuromyelitis optica was more frequent in patients with poor socio-economic status. A relatively high proportion of patients (nearly half of the MS patients with optic nerve involvement and two-thirds of the MS--neuromyelitis optica group) had bilateral optic nerve involvement. Except in 2 patients, the tempo and course of the disease in our patients with MS was comparable to that seen in the West. In 3 patients with MS the initiation of symptoms or appearance of fresh symptoms was related to vaccination. In addition to the above 45 patients, 29 other cases of possible demyelination have been reviewed. Nine of these had one or more remission and relapse but in the same site as the first episode. Seventeen had a solitary episode of spinal cord or brainstem disturbance with some remission and 3 had multiple lesions but without remission or clear dissemination in time. In the absence of a specific diagnostic test or autopsy, such atypical cases will remain unclassified. To conclude, MS does occur in the Bombya region and elsewhere in India but the incidence is much less than in the West. Further studies and especially careful follow-up of atypical cases may show that the prevalence is higher than it seems today.     

视神经脊髓炎谱系疾病临床特点分析及发病机制探讨

目的 分析视神经脊髓炎谱系疾病(Neuromyelitis optica spectrum disorders,NMOSDs)的临床特点,并探讨相关发病机制及诊治方案。方法 回顾性收集2018年6月-2019年12月入住本院神经内科并诊断为NMOSDs的患者56例,分析患者的一般人口学特征、临床表现、实验室及影像学检查表现、诊疗及预后等。结果 共纳入56例患者,男女比例为1:10.2,首次发病年龄20～77(48.36±14.52)岁,73.2%患者表现为急性-亚急性起病,62.5%患者以急性脊髓炎起病,21.4%患者以急性视神经炎起病,16.1%患者表现为最后区综合征。30例患者中22例脑脊液水通道蛋白-4(Aquaporin-4,AQP4)-IgG抗体阳性,2例髓鞘少突胶质细胞糖蛋白(Myelinoligodendrocyte glycoprotein,MOG)-IgG抗体阳性,颈髓多与延髓、胸髓合并受累,也可出现颅内病灶。结论 NMOSDs临床表现复杂多样,好发于中青年女性,复发率及致残率较高,首发症状以急性脊髓炎多见,以最后区综合征起病多易误诊。多数患者在发作急性期经过大剂量激素冲击治疗后预后良好,硫唑嘌呤、他克莫司、利妥昔单抗等免疫抑制剂仍是预防复发的一线药物。