视神经脊髓炎谱系疾病临床和影像学特点及自身抗体分析

目的初步总结视神经脊髓炎谱系疾病患者临床和影像学特点,回顾分析其血清和脑脊液自身抗体表达变化,为临床选择合理诊断方法提供参考。方法与结果共10例视神经脊髓炎谱系疾病患者,头部或脊髓MRI检查显示病灶分别位于脑干(3例)、颈髓(3例)、胸髓(6例)或颈胸髓(1例)。其中血清NMO-IgG阳性3/8例、抗核抗体阳性1/3例、甲状腺球蛋白和甲状腺过氧化物酶抗体阳性2/3例、甲状腺功能减退2例和甲状腺功能亢进1例、同型半胱氨酸升高2例。结论视神经脊髓炎谱系疾病好发于青壮年,女性多见,对高度怀疑视神经脊髓炎谱系疾病的患者应行血清和脑脊液自身抗体,以及头部和脊髓MRI检查,为明确诊断和合理治疗提供临床依据。缓解期对自身抗体阳性病例进行实验室指标复查,能够明确自身抗体与视神经脊髓炎谱系疾病之间的关系。     

磁刺激运动诱发电位及磁共振对脊髓炎定位诊断的对比分析

目的比较磁刺激运动诱发电位(MEP)和脊髓磁共振(MRI)检查对急性横贯性脊髓炎(ATM)和视神经脊髓炎(NMO)病人进行定位诊断的价值。方法对临床确诊为ATM 40例和25例NMO的住院患者的MEP和MRI检查结果进行分析,比较两种检查定位符合率。结果MRI显示ATM患者以胸脊髓损害为主,NMO组患者以颈胸段联合损害为主。MEP显示ATM组患者主要表现为上肢锥体束传导正常、下肢锥体束传导异常,而NMO组患者在表现为上下肢锥体束传导均异常。当病变位于颈髓时,ATM组患者定位符合率为92.8%,NMO组患者定位符合率为95.2%;当病变位于胸髓时,ATM符合率100%,NMO符合率80%。结论在ATM和NMO患者中,MEP提示的锥体束异常部位与MRI所证实的病变部位相吻合。     

视神经脊髓炎患者33例脑部磁共振分析

目的 探讨视神经脊髓炎(neuromyelitis optica,NMO)患者脑部MRI影像学表现.方法 收集满足最新NMO诊断标准且脑部MRI表现不符合多发性硬化诊断标准的患者33例,均行脑部和脊髓MRI检查,分析其MRI影像学特点.结果 33例NMO中,脑部正常表现者5例(15.2%),异常表现28例(84.8%),其中脑内实质有明确病灶22例(66.7%),另6例(18.2%)脑内虽未见明确病灶,但深部脑白质显示了肉眼可视的对称性弥漫性脱髓鞘高信号影.22例明确病灶中,15例病灶数≥2个,7例为单个病灶.幕上近皮质、皮质下和深部脑白质区的点状非特异性病灶最多,少数为非典型的斑片状融合病灶.幕下脑干是易受累的部位(14/33,42.4%),特别是延髓(7/33,21.2%).结论 NMO患者出现脑内异常较为常见,有脑部的异常不能排除NMO的诊断.认识NMO脑内病灶对完善NMO诊断标准有帮助.     

视神经脊髓炎脑部病灶的影像学特征及临床表现

目的 探讨视神经脊髓炎(NMO)脑部病灶的影像学特征及主要临床表现.方法 回顾性分析南京医科大学附属脑科医院神经内科自2002年1月至2008年5月收治的19例NMO患者脑部MRI检查结果及相关病史资料.结果 本组19例患者中15例行头颅MRI检查,7例发现脑部病灶;病变累及大脑半球3例,丘脑2例,下丘脑1例,第三脑室和中脑导水管周边1例,中脑1例,桥脑1例,延髓2例;MRI上病灶为点状、斑片状或线样,呈T1低信号、T2和FLAIR高信号改变.临床表现恶心、呕吐、呃逆、复视、眼震、面部麻木、认知功能障碍及嗜睡等.结论 NMO可出现不同部位脑部病灶,以脑室管膜周边和下丘脑处病灶具有疾病特征性.部分NMO患者出现脑部受累表现,少数患者以脑部症状为首发临床事件.     

血清水通道蛋白4抗体阳性的中枢神经系统脱髓鞘病104例临床特点分析

目的探讨水通道蛋白4抗体(AQP4-Ab)阳性中枢神经系统脱髓鞘病患者的临床特点。方法回顾性分析104例患者的临床资料,结合临床特点和诊断进行综合分析。结果 57例符合视神经脊髓炎(NMO)诊断,42例符合NMO谱系病(NMOSD)诊断,5例仅有颅内典型病灶不伴视神经炎或脊髓炎。首次发病累及脊髓、视神经和脑干分别为55.8%、43.2%和29.8%。头颅MRI异常者占52.4%。4例脊髓病灶为短节段。结论少数AQP4-Ab阳性患者的孤立颅内典型部位病变,通过随访最终可能满足NMO或NMOSD诊断。提示目前的诊断标准需进一步完善,NMO可以出现短节段脊髓病灶。     

以顽固性呃逆和恶心呕吐为首发症状的视神经脊髓炎临床分析

目的 研究以顽固性呃逆和恶心呕吐(IHN)为首发症状的视神经脊髓炎(NMO)的临床特点及MRI表现.方法 收集50例NMO患者的临床资料,对其中5例以IHN为首发症状的NMO患者临床表现及MRI特点进行分析.结果 以IHN为首发症状的NMO患者女性多见,发病较晚,头MRI显示病灶主要分布在第三、四脑室,中脑导水管周围及延髓中央管等室管膜周边,脊髓MRI显示线样延髓脊髓损害,病灶常常为大于3个椎体节段,主要累及灰质,以脊髓中央管为中心呈H型分布.结论 IHN是NMO的独特首发症状,并完全可逆,主要累及孤束核和最后区引起IHN.其MRI病灶具有特异性,多分布在室管膜和脊髓中央管周边(即水通道蛋白4的高表达区),呈线样损害.     

视神经脊髓炎相关抗体

视神经脊髓炎是主要累及视神经和脊髓的自身免疫性中枢神经系统疾病,水通道蛋白4是主要靶抗原,其特异性抗体NMO-IgG阳性患者以女性多见,临床症状较重,同时出现双侧视神经炎或视神经炎和脊髓炎,受累脊髓节段较长。而在NMO-IgG阴性患者血清中可以检出抗水通道蛋白1(AQP1)抗体和抗髓鞘少突胶质细胞糖蛋白(MOG)抗体,抗AQP1抗体阳性患者女性少见,长节段脊髓病变多见,视神经炎少见;抗MOG抗体阳性患者男性多见,视神经炎多见,尤其是双侧视神经同时受累,胸腰髓受累多见。     

视神经脊髓炎脑部受累的MRI特征性影像表现及临床分析

目的分析成人视神经脊髓炎脑部影像表现及临床特点。方法回顾分析2010-10—2017-10开封市中心医院收治的25例视神经脊髓炎患者为研究对象,并行头颅及脊髓MRI检查,根据头颅MRI检查结果,将有脑部病灶的患者纳入研究病例。结果采用1.5T MR扫描仪对患者头部和脊髓进行检查获取MRI图像结果显示:25例患者中头部MRI检查中出现脑部病变者16例,未见出现脑部病变9例。病变主要位于延髓、脑桥、侧脑室周围、第三室周围、中脑导水管、胼胝体、大脑白质等处,根据病灶形态可分为斑片状、斑点状、线样状、融合型,不规则型,常见病灶为散在不规则斑片状、斑点状。15例患者MRI增强检查未见明显强化,1例患者病灶呈轻度强化。16例患者中均出现视神经和脊髓以外的中枢神经系统损害。结论大多数视神经脊髓炎患者都会伴有脑部病变,绝大多数病灶部位与患者的临床表现具有对应关系,病变部位易伴发相应临床的表现。     

视神经脊髓炎与脊髓型多发性硬化的临床研究

目的：视神经脊髓炎(NMO)又称Devic病,是视神经和脊髓同时或相继受累的急性或亚急性病变。它是一种独立的疾病实体还是MS的一种亚型,仍不清楚。本研究通过比较一些NMO和脊髓型MS的病例的临床特点,来探讨两者的关系。方法：回顾性分析20例NMO患者和40例脊髓型MS患者,对两组的人口统计学、临床表现、实验室检查(CSF和MRl、EP)及临床预后等进行比较。结果：NMO患者的神经系统表现局限于视神经和脊髓,多呈现横贯性脊髓损害(P＜0．001),而脊髓型MS多为不完全横贯性损害(P＜0．001)。NMO组出院时EDSS评分为5．3＋/－3．1,明显高于脊髓性MS组(2．2＋/－2．3,P＜0．001)。NMO组CSF细胞计数平均值明显大于脊髓型MS组(P＜0．001),蛋白质水平前者高于后者,但缺乏统计学意义。头MRI异常见于12．5％的NMO患者和83．3％的脊髓型MS患者(P＜0．001)。脊髓大型纵向融合病灶见于64．7％的NMO患者和21．6％的脊髓型MS患者(P＝0．005)。NMO组常累及髓内中心(P＝0．003),而MS组则多局限于侧后索(P＝0．003)。NMO组VEP异常率达100％,明显高于MS组(38．5％,P＜0．001)。BAEP异常率脊髓型MS组高于NMO组,并提示中枢受累(P＝0．372)。NMO组可分为单相型和复发型。女性、发病年龄晚、索引事件间隔时间较长可能与NMO复发病程有关。单相型临床表现比复发型重,但长期预后较好,复发型常在3年内经过一系列复发导致严重残疾。结论：根据临床数据,NMO和脊髓型MS应被视为两个独立的疾病实体。NMO发病时年龄大,女性居多,临床表现严重,预后差。头和脊髓MRI、CSF及诱发电位的特征可以帮助区别两种疾病。但是由于MS和NMO都没有特异性的生化指标,发现这样的生化指标将大大推动这一疾病的诊断和研究。此外,应进行大量多中心、大样本的长期随访研究,来对NMO和MS的各种治疗方法进行系统评价。     

原发性干燥综合征合并视神经脊髓炎谱系疾病临床分析及文献复习

目的探讨原发性干燥综合征合并视神经脊髓炎谱系疾病的临床表现、实验室检查和MRI特点,以及治疗和预后。方法回顾分析4例诊断明确的原发性干燥综合征合并视神经脊髓炎谱系疾病患者的临床资料,分析其发病特点。结果临床症状与体征主要表现为原发性干燥综合征合并复发性纵向延伸性脊髓炎或球后视神经炎,实验室检查血清抗干燥综合征抗原A抗体(4例)、抗干燥综合征抗原B抗体(3例)、视神经脊髓炎特异性抗水通道蛋白4(AQP4)抗体(3例)阳性。头部MRI病灶主要分布于双侧基底节和大脑脚皮质脊髓束走行区、双侧额叶、右侧皮质脊髓束区、双侧侧脑室旁和脑干;脊髓MRI病灶以累及颈胸髓为主,≥3个椎体节段(3例)或累及延髓(2例)。急性期以大剂量糖皮质激素冲击和序贯口服泼尼松,并辅助免疫抑制药治疗为主。结论原发性干燥综合征和视神经脊髓炎均为自身免疫性疾病,二者关系密切。对于视神经脊髓炎和(或)视神经脊髓炎谱系疾病患者建议行血清自身抗体和抗AQP4抗体检测,抗AQP4抗体阳性者复发率高,预后不良。建议辅助应用免疫抑制药,以减少复发。     

Neuromyelitis optica-IgG in idiopathic inflammatory demyelinating disorders amongst Hong Kong Chinese

Background:  Idiopathic inflammatory demyelinating disorders (IIDD) affect the central nervous system. In classical multiple sclerosis (CMS), brain, optic nerves [optic neuritis (ON)] and spinal cord [acute transverse myelitis (ATM)] are affected. In neuromyelitis optica (NMO), optic nerves and spinal cord are predominantly affected. NMO-IgG, an autoantibody targeting aquaporin-4, is a marker for NMO. We studied the frequency and clinical relevance of NMO-IgG seropositivity in IIDD patients.
Methods:  Neuromyelitis optica-IgG was detected by indirect immunofluorescence using primate cerebellum.
Results:  Neuromyelitis optica-IgG was detected in six of 10 NMO patients (60%), six of 10 idiopathic relapsing transverse myelitis (IRTM) patients (60%), two of nine idiopathic relapsing ON patients (22%), one of 11 patients (9%) having single ON attack, one of 30 CMS patients (3%), and none of patients having single ATM attack or controls. Comparing NMO-IgG seropositive ( n  = 12) with NMO-IgG seronegative ( n  = 8) patients having NMO or IRTM, NMO-IgG seropositivity was associated with a higher relapse rate in first 2 years, 1.5 and 0.6 attacks/year for seropositive and seronegative groups respectively ( P  = 0.006), and non-significant trend towards more severe ON and myelitis with poorer clinical outcome.
Conclusion:  Neuromyelitis optica -IgG facilitates diagnosis of NMO spectrum disorders. NMO-IgG seropositivity is associated with higher relapse rate in first 2 years.     

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。     

Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions

Multiple sclerosis (MS) in Asian populations is often characterized by the selective involvement of the optic nerve (ON) and spinal cord (SP) (OSMS) in contrast to classic MS (CMS), where frequent lesions are observed in the cerebrum, cerebellum or brainstem. In Western countries, inflammatory demyelinating disease preferentially involving the ON and SP is called neuromyelitis optica (NMO). Recently, Lennon et al. discovered that NMO-IgG, shown to bind to aquaporin 4 (AQP4), could be a specific marker of NMO and also of Japanese OSMS whose clinical features were identical to NMO having long spinal cord lesions extending over three vertebral segments (LCL). To examine this antibody in larger populations of Japanese OSMS patients in order to know its epidemiological and clinical spectra, we established an immunohistochemical detection system for the anti-AQP4 antibody (AQP4-Ab) using the AQP4-transfected human embryonic kidney cell line (HEK-293) and confirmed AQP4-Ab positivity together with the immunohistochemical staining pattern of NMO-IgG in approximately 60% of Japanese OSMS patients with LCL. Patients with OSMS without LCL and those with CMS were negative for this antibody. Our results accorded with those of Lennon et al. suggest that Japanese OSMS with LCL may have an underlying pathogenesis in common with NMO.     

引起顽固性呃逆、呕吐的视神经脊髓炎的临床表现和影像学研究

目的 探讨引起顽固性呃逆、呕吐(IHN)的视神经脊髓炎(NMO)患者的临床表现和脑干、脊髓MRI特点.方法 收集中山大学附属第三医院神经科17例NMO患者的临床资料,对其中8例合并IHN的NMO患者临床表现及MRI特点进行分析.结果 IHN在NMO患者中常见,本组17例NMO中有8例合并IHN,临床上表现为IHN、复视、眼球震颤,其中6例表现有线样延髓征(LML)或线样延髓脊髓征(LMSL).脊髓纵向MRI显示病灶常常大于3个椎体节段,且以脊髓中央管为中心;轴位脊髓MRI表现为部分性或横贯性,以脊髓的后角或侧角为主,前角受累较少. 结论 引起IHN的NMO临床上多伴有复视和眼球震颤,延髓脊髓MRI常常可见LML或LMSL征,而且病灶以脊髓中央管为中心,后角或侧角受累为主,这些可与多发性硬化相鉴别.     

Recurrent inflammatory optic neuritis and neuromyelitis optica

Inflammatory optic neuritis (ON) represents a frequent clinical situation in neurology and ophthalmology. When MRI and CSF analysis are normal, ON is considered idiopathic with a suspected viral etiology. However, in several cases either a recurrence or a myelitis may occur. In the first case, it is relapsing inflammatory optic neuritis (RION) and in the second case it is neuromyelitis optica (NMO). Nevertheless, predictive criteria of a recurrence or an extension of the disease to spinal cord remains unknown, excepted for anti-NMO IgG antibodies which are probably highly specific for a future evolution to NMO. In the present paper, the authors successively present the two clinical situations (RION and NMO) and attempt to summarize diagnostic and prognostic criteria.     

Anti-aquaporin 4 antibody in Japanese multiple sclerosis with long spinal cord lesions]

Multiple sclerosis (MS) in Asian populations is often characterized by the selective involvement of the optic nerve (ON) and spinal cord (SP) (OSMS) in contrast to classic MS (CMS), where frequent lesions are observed in the cerebrum, cerebellum, or brainstem, as well as in the ON and SP. In Western countries, inflammatory demyelinating disease preferentially involves the ON and SP is called neuromyelitis optica (NMO). Recently, Lennon et al. have reported that NMO-immunoglobulin G (NMO-IgG), which has been shown to bind to aquaporin 4 (AQP4), could be a specific marker of the NMO. To examine the epidemiological and clinical spectra of Japanese MS with this antibody, we established an immunohistochemical detection system for the AQP4-Ab using the AQP4-transfected human embryonic kidney cell line (HEK-293) and confirmed AQP4-Ab positivity together with the immunohistochemical staining pattern of NMO-IgG in approximately 60% of Japanese MS patients with long spinal cord lesions extending over three vertebral segments (LCL (+) MS). Patients with OSMS without LCL and those with classic MS were negative for this antibody. AQP4 is totally lost in the OSMS lesions. The AQP4-Ab titer correlates with the activity of the disease and the symptom improves after plasma exchange. These results suggested the close relation to the pathogenesis of AQP4-Ab to NMO/OSMS.     

Clinical characteristics, prognosis, and seropositivity to the anti-aquaporin-4 antibody in Korean patients with longitudinally extensive transverse myelitis

Longitudinally extensive transverse myelitis (LETM) is a syndrome with extensive spinal cord lesions spanning three or more vertebral segments on spinal cord MRI. Although many reports have indicated that LETM is a characteristic feature of neuoromyelitis optica (NMO) in Western countries, the clinical characteristics and risk for development of NMO in Korean patients with LETM is not clear. We retrospectively investigated the clinical, laboratory, radiological features, and prognosis of Korean patients with a first-ever episode of idiopathic LETM. Patients were classified into four subgroups, depending on their clinical course: monophasic LETM, recurrent LETM, NMO, and classic multiple sclerosis (MS). We compared various clinical, laboratory, and radiological features between groups. Of 20 patients with first-ever LETM, 15 (75%) were men, and 13 (65%) experienced clinical relapse over a mean follow-up period of 58 months. Three of 20 patients (two with NMO, one with recurrent myelitis) were seropositive for anti-AQP4 antibodies. The predominance of men in the monophasic and recurrent LETM groups compared to the NMO group was remarkable. In conclusion, Korean patients with LETM are predominantly male and have low seropositivity for anti-AQP4 antibody, which distinguishes them from LETM patients in Western countries. Patients with LETM and seropositivity for anti-AQP4 antibody have a high risk of relapse. The male predominance and the relatively low seropositive rate for anti-AQP4 suggests that rather than being a limited form of NMO, recurrent LETM is a new clinical entity in Koreans.     

视神经脊髓炎与多发性硬化的临床症状、脊髓MRI表现的对比分析

目的 结合视神经脊髓炎(NMO)与多发性硬化(MS)患者的临床症状和脊髓MRI特点探讨两者之间差异发生的机制.方法 回顾性分析中山大学附属第三医院自2004年1月至2007年1月收治的23例NMO患者及21例MS患者的临床资料,比较其临床症状及脊髓MRI上受损部位MRI上的差异.结果 NMO患者多为女性,且首次发病年龄、扩展病残状况评分(EDSS)评分均高于MS患者;双侧深感觉障碍、束带感、直肠或膀胱括约肌功能障碍3种临床症状在NMO、MS患者中的发生率不同,差异均有统计学意义(P<0.05);上述各临床症状基本能在脊髓MRI找到相应受损病灶.结论 NMO是不同于MS的脱髓鞘疾病,其特殊的发病机制导致其临床症状与脊髓MRI均有自己的特点.     

视神经脊髓炎脊髓磁共振成像特征

目的 比较视神经脊髓炎(NMO)与多发性硬化(MS)的脊髓MRI特点,从MBI的角度重新认识NMO.方法 对20例MS患者和23例NMO患者的脊髓MRI进行同顾性分析.结果 NMO患者脊髓MRI多表现为线样延髓征、线样延髓脊髓征、线样脊髓征、脊髓横贯性或次横贯性损伤,且常超过3个节段(23例),而MS患者脊髓MRI病变节段短(≥3个节段者8例,χ2=19.142,P<0.01),常呈偏心性分布(17例,与NMO组比较,χ2=25.256,P<0.01).结论 NMO不同于MS,在MRI方面,病灶的分布有其自身特征,而MS的脊髓病灶与髓鞘走向一致.因此,我们从影像学角度进一步证实NMO是有异于MS的一种独立的疾病.     

视神经脊髓炎临床与病理

目的：分析视神经脊髓炎（NMO）的特征。方法：对114例NMO患者的临床资料,3例尸检结果,28例随访情况进行研究。结果：该病患者男女之比为1：2．5,发病年龄以12－50岁居多（85％）,急性和亚急性起病占大多数（74．84％）,视神经症状为首发占58．77％,视神经与脊髓症状的间隔时间在1年内者60例,占52．33％。脊髓以横贯性损害为主,有95例（83．33％）,以胸段损害最多（64．33％）,尸体解剖例2、3为NMO;病程中有缓解－复发者65例,其中有14例发展为多发性硬化,包括尸体解剖例1。结论：NMO有两种类型;复发型中有一小部分可发展成为MS,MRI,脑干视觉诱发电位和长期随访有利于NMO和MS的鉴别。