视神经脊髓炎的MRI研究进展

视神经脊髓炎 (neuromyelitis optica,NMO),又称Devic病或Devic′s综合征,是多发性硬化(multiple sclerosis,MS)的亚型还是一种独立的疾病一直存在争议.现在越来越多的证据表明,NMO是一种不同于MS的离子通道疾病.1999年Wingerchuk等[1]建立了NMO的诊断标准,几经修订,至2006年发表了最新推荐诊断标准[2],都明确了MRI在NMO诊断中的价值.特别是近年各种高级MRI检测技术的发展和完善,为研究NMO提供了新的方法.     

视神经脊髓炎、多发性硬化患者脊髓改变的磁共振表现及鉴别诊断

目的:探讨视神经脊髓炎(Neuromyelitis optica,NMO)及多发性硬化(Multiple sclerosis,MS)患者脊髓改变以及相关临床资料的差异,为鉴别诊断两种疾病提供一定的依据.方法:利用GE Signa 1.5T磁共振成像系统,对2004年7月到2008年9月我院临床确诊或疑似NMO及MS的患者做免费MRI检查.根据影像资料、脑脊液(Cerebrospinal fluid,CSF)及神经诱发电位(Visual evoked potential,VEP)检查结果,将满足Wingerchuk等推荐的NMO诊断标准及MS的最新McDonald诊断标准的患者纳入研究对象.结果:确诊NMO患者41例,MS患者121例.脊髓受累:NMO患者以受累节段≥5个(78.1%)最为常见,其中以颈髓、颈胸髓联合受累最多,占患者总数的80.5%.病灶均为连续性改变,急性期脊髓肿胀明显,多为灰质及白质同是受损;MS 患者以胸髓受累最为常见(46.4%),41例(59.4%)受累脊髓节段≥3个,但为非连续损害,表现为渐进性过程,以白质受累为主.经统计学检验,两组之间受累脊髓节段数有统计学差异(P<0.001).结论:NMO与MS患者脊髓表现具有一定的差异,可以成为两者的鉴别点.     

视神经脊髓炎的MRI与临床观察

视神经脊髓炎 (ＤＮＯ)作为多发性硬化 (ＭＳ)的一种分型 ,亚洲地区被多数学者所公认[1,2 ] 。通过磁共振 (ＭＲＩ)检查 ,发现ＤＮＯ并非只有视神经和脊髓病损 ,而有相当ＤＮＯ病人存在中枢神经系统其它部位的病损 ,符合了ＭＳ空间播散的特点。现将我院收治 17例ＤＮＯ病人报告如下。1　资料与方法17例中 ,男 8例 ,女 9例 ,年龄 16～ 46岁 ,平均 30 2岁。临床表现视神经和脊髓病损的 14例 ,只表现脊髓病损而视觉诱发电位 (ＶＥＰ)异常的 3例 ,拟诊为ＤＮＯ。病程 1ｍｏ～ 19ａ。有缓解复发者 10例 ,视神经和脊髓病损间隔时间为 1ｍｏ～ 7ａ…     

35例视神经脊髓炎的临床分析

视神经脊髓炎(NMO)为视神经和脊髓同时或相继受累的急性或亚急性脱髓鞘性疾病.对于视神经脊髓炎是一个独立的疾病还是多发性硬化的一种亚型引起了广泛的争议[1,2],在临床诊断上常有误诊及漏诊[3].我院2000～2006年共收治视神经脊髓炎病人35例,现对临床、脑脊液、视觉诱发电位及影像学改变进行分析,探讨NMO的临床特征.     

视神经脊髓炎特异性IgG抗体对视神经脊髓炎的诊断价值

目的 研究NMO-IgG对视神经脊髓炎的诊断价值.方法 收集解放军总医院2008年11月至2009年6月住院和门诊临床诊断的患者共80例,其中视神经脊髓炎(NMO)及高危NMO(HR-NMO)患者23例,经典多发性硬化(MS)患者36例,临床孤立综合征(CIS)11例,其他神经系统疾病患者10例,采用细胞间接免疫荧光法检测所有患者血清视神经脊髓炎特异性IgG抗体(NMO-ISG)滴度水平,比较各组患者临床特点及NMO-IgG水平.结果 NMO、HR.NMO组与MS、CIS组相比,发生严重视神经炎、横贯性脊髓炎、有颅脑MRI正常表现、脊髓病灶超过3个椎体节段及血清NMO-IgG阳性均有统计学意义(P<0.05).NMO与HR-NMO组血清NMO-IgG显著高于MS组,而MS组血清NMO-IgG又略高于CIS组与其他疾病组.血清NMO-IgG诊断NMO的灵敏度达82.6%,特异性达96.2%.结论 血清NMO-IgG的检测对视神经脊髓炎的诊断及鉴别诊断有一定的价值.     

多发性硬化和视神经脊髓炎患者血尿酸水平分析

目的研究急性期的多发性硬化和视神经脊髓炎患者同血尿酸之间的关系。方法选择住院的均为急性期的36例MS和32例NMO患者,并以同期年龄相仿、饮食结构大致相同的40例健康体检者作为对照,分别检测其血尿酸水平。结果MS组血尿酸水平[(250.069±55.33)μmol/L]和NMO组患者血尿酸水平[(231.121±65.05)μmol/L]均较对照组[(287.35±61.79)μmol/L]明显减低,且差异具有统计学意义;但MS组和NMO组之间差异无统计学意义。结论 MS组和NMO组血尿酸水平均降低,低血尿酸可能是中枢神经系统脱髓鞘疾病的一个生物学标志的危险因素。     

视神经脊髓炎的MRI特征

目的探讨视神经脊髓炎的磁共振成像特征.方法回顾性分析11例经临床随访证实的视神经脊髓炎的平扫及增强磁共振影像资料,寻找其特征性表现.结果病灶仅累及颈髓3例,颈胸髓6例,仅胸髓2例.矢状位呈线样损害1例,线样损害合并弥漫性损害6例,弥漫性损害4例.结论 MR对诊断视神经脊髓炎有特征性,可作为检查首选.     

以急性脊髓炎为首发表现的视神经脊髓炎谱系疾病与多发性硬化的脊髓影像特点比较

目的 比较以急性脊髓炎为首发表现的视神经脊髓炎谱系疾病(NMOSD)与多发性硬化(MS)患者的临床特征,侧重比较脊髓影像特点.方法 收集2017年1月至2020年6月于郑州大学第一附属医院和郑州大学第五附属医院确诊并治疗的83例NMOSD患者(NMOSD组)和34例MS患者(MS组)的临床资料.患者均以急性脊髓炎为首发...     

视神经脊髓炎与多发性硬化的关系

目的 探讨视神经脊髓炎的临床特点、病程、影像学特征及其与多发性硬化的区别。方法 对38例视神经脊髓炎的临床特点、影像学特征及诱发电位进行回顾性分析。结果 13例出现上呼吸道感染为主的前驱症状。31例急性和亚急性起病，女性多于男性，21～41岁发病29例(76．3％)。双侧视神经受损28例(73．6％)；脊髓横贯性损害31例(81．6％)，以颈、胸髓受损多见，脊髓病灶节段为3个以上者26例(68．4％)。其中18例为复发型，复发症状及病灶仅限于视神经和脊髓。结论 视神经脊髓炎有单相型及复发型，与多发性硬化不同，为一独立疾病。     

视神经脊髓炎11例误诊分析

视神经脊髓炎是多发性硬化症的一种亚型 ,早期临床表现不典型 ,极易误诊。本院 1992年 6月至 2 0 0 0年 4月共收治视神经脊髓炎 15例 ,其中误诊 11例 ,现将原因分析如下。1　临床资料1.1　一般资料　男 5例 ,女 6例 ,年龄 18～ 70岁 ,平均 41岁。1.2　临床表现　 11例均呈亚急性起病 ,病程 1个月至 4年 ,10例有脊髓不全损害的表现 ,2例以肢体阵发性麻木、抽搐为首发症状 ,先后出现视力减退 3例 ,复视 2例。1.3　误诊情况　误诊时间 3～ 4年 3例 ,1～ 4个月 8例。误诊为脊髓压迫症 4例 ,急性脊髓炎 4例 ,癫痫 1例 ,腰椎间盘突出症 1例 ,球后…     

Differentiation of neuromyelitis optica from multiple sclerosis in a cohort from the mainland of China

Background Although there were criteria for diagnosis of neuromyelitis optica （NMO） and multiple sclerosis （MS）,it is still difficult to differentiate NMO from MS,due to the overlapping clinical manifestations.Therefore it is necessary to characterize clinical features of NMO and MS patients in the mainland of China,to simplify the process of disease diagnosis,and to identify criteria for the differential diagnosis of NMO and MS.Methods A total of 138 Chinese Han patients from the mainland of China including 73 NMO,60 MS and 5 MS-like patients with positive NMO-IgG were included in the study.Clinical records were reviewed retrospectively and the results of clinical examination,laboratory experiments,magnetic resonance imaging （MRI） and evoked potentials （EPs） were compared between NMO and MS patients.In addition,the relationship between the NMO-IgG serologic status and clinical characteristics were analyzed.Results Compared with MS patients （1.3∶ 1.0）,more female prevalence was observed in NMO patients （4.2∶ 1.0; P=0.003）.There were also statistically significant differences in visual EPs,oligoclonal bands,brainstem lesions in MRI and longitudinally extensive spinal cord lesions （LESCLs） between NMO and MS patients.Brainstem lesions observed in brain MRI were found in 17.9% of MS patients,over 3.7 times higher than in NMO patients （4.8%,P=0.024）.When stratified NMO patients by NMO-IgG,LESCLs were found in 42.1% of NMO-IgG-negative NMO patients,over 3.5 times higher than in NMO-IgG-positive patients （11.9%,P=0.008）.Statistical difference was also observed in CD4＋/CD8＋ ratios between NMO-IgG-positive and-negative NMO patients.Conclusions Comprehensive analysis of MRI,laboratory and EPs data can facilitate differential diagnosis of MS and NMO.In addition,the combination of LESCLs and brain MRI findings failing to satisfy MRI criteria for MS is highly sensitive and specific for NMO.     

B淋巴细胞活化因子在视神经脊髓炎患者血清及脑脊液中的表达及意义

目的探讨B淋巴细胞活化因子（BAFF）在视神经脊髓炎（NMO）患者血清及脑脊液中的表达情况及临床意义。方法收
集44例NMO患者及38例多发性硬化（MS）患者的临床资料,患者均为急性复发期。30例健康人和15例非炎性神经系统疾病
患者分别作为对照组,采用酶联免疫吸附测定（ELISA）方法检测血清及脑脊液中BAFF水平。结果NMO组、MS组和对照组
血清中BAFF水平分别为250.2±126.9、249.6±130.7和222.9±126.1 pg/ml,3组间两两比较,差异无统计学意义（P>0.05）。NMO
组、MS组和对照组脑脊液中BAFF水平分别为525.8±230.0、298.4±141.9和141.4±76.2 pg/ml,3组间两两比较,NMO组和MS
组脑脊液中BAFF水平较对照组明显增高,NMO组脑脊液中BAFF水平较MS组明显增高（P<0.05）。且NMO组、MS组脑脊液
中BAFF水平与EDSS评分存在相关性,EDSS评分越高,其脑脊液中BAFF水平越高。但是NMO组脑脊液中BAFF水平与
AQP4抗体滴度之间不具有相关性。结论脑脊液中BAFF水平可能是NMO和MS鉴别诊断的生物标记物,并在判定疾病严重
程度方面具有重要价值。
     

Gray matter involvement in patients with multiple sclerosis as shown by magnetic resonance imaging

Objective  To summarize the main findings seen on conventional and advanced magnetic resonance imaging (MRI) used to assess gray matter (GM) involvement in patients with multiple sclerosis (MS).

Data sources  The data used in this review were obtained mainly from studies reported in the PubMed database using the terms of multiple sclerosis, gray matter, magnetic resonance imaging. 

Study selection  Relevant literatures on studies of GM involvement in MS patients were identified, retrieved and reviewed.

Results  MS is the most common chronic, disabling central nervous system disease in young adults. Although traditional thinking has considered MS to be a chronic inflammatory demyelinating condition affecting solely the white matter (WM) of the central nervous system, over the last few years it has been shown that GM pathology is also common and extensive. GM demyelinating lesions can not only be found in the cerebral cortex but also in the deep gray nuclei. Apart from focal demyelinated lesions, diffuse neuronal loss and atrophy is also present in the GM of MS patients.

Conclusions  The widespread use of conventional and quantitative MRI based techniques in MS has led to an improved understanding of the mechanisms underlying the inflammatory and neurodegenerative processes of the disease. However, more researches are needed to unravel GM pathology in MS patients, which at present remains enigmatic.     

Serum antibodies to 25 myelin oligodendrocyte glycoprotein epitopes in multiple sclerosis and neuromyelitis optica: clinical value for diagnosis and disease activity

Background  Whether antibody to myelin oligodendrocyte glycoprotein (MOG) can be a diagnostic marker for multiple sclerosis (MS) is still controversial. Recent studies suggested that serum specific anti-MOG epitope antibody might be an MS specific marker. However, these studies did not include neuromyelitis optica (NMO) which might be proven to also have anti-MOG antibody. Hence, the present study was undertaken to investigate the clinical value of serum antibodies to 25 MOG epitopes in conventional MS (CMS) and NMO.

Methods  Serum anti-MOG epitope IgG was detected in 61 CMS patients, 54 NMO patients, and 77 healthy controls, using enzyme-linked immunosorbent assay (ELISA).

Results  Anti-MOG_27-38 IgG levels in both CMS and NMO patients were significantly higher than that in healthy controls (optical density (OD): 0.64±0.38, 0.48±0.23 vs. 0.19±0.09; P=0.000). CMS and NMO patients in relapse stage had significantly higher anti-MOG_27-38 IgG level than patients in remission stage (OD: 0.55±0.14 vs. 0.24±0.09, P=0.027).

Conclusion  Although serum anti-MOG epitope IgG could not differentiate MS from NMO, it may be a useful marker for monitoring disease activity.

     

Functional magnetic resonace imaging in patients with optic neuritis

Optic neuritis （ON）,an acute inflammatory optic neuropathy,causes conduction blocks in the optic nerve and a series of visual functional changes.It is characterized not only by the inflammation of the optic nerve but also the visual dysfunction caused by the neuropathy involving optic chiasm,lateral geniculate body,optic radiation,and visual cortex.Clinical diagnosis mainly relies on the visual function evaluation using electrophysiology,vision examination,and magnetic resonance imaging （MRI）.However,pattern-visual evoked potential （P-VEP） and visual field examination have limitation.Blood-oxygenation level dependent functional magnetic resonance imaging （BOLD-fMRI）,an integrative modality of the function,images and anatomy,can be used to monitor the status of brain activity in the functional areas in vivo.The present study aimed to examine the status of functional activities of the visual cortex in the patients with optic neuritis using of BOLD-fMRI technique and to evaluate the application of fMRI in the optic neuritis.Keywords：optic neuritis; functional magnetic resonance imaging,; visual field; pattern-visual evoked potential     

视神经炎患者的功能磁共振成像研究

目的 应用功能磁共振成像（functional magnetic resonance imaging,fMRI）探讨视神经炎患者的皮层功能变化;评价fMRI检查在视神经炎中的应用价值。方法 收集确诊为视神经炎的患者11例,行中心静态视野检查,图形视觉诱发电位（pattern-visual evoked potential,P-VEP）检查,同时行fMRI检查,观察视觉刺激产生视觉皮层血氧水平依赖 (blood-oxygenation level dependent, BOLD)信号变化的体素数量和变化率,评价其与中心15度静态视野、P-VEP与皮层功能变化的关系。结果 视神经炎患者fMRI可见枕叶视皮层、颞下回、后顶叶都有不同程度的兴奋,主要兴奋区位于枕叶距状裂周围。患眼与未发病眼相比,患眼刺激的皮层激活体素减少,激活信号变化的百分比降低。中心15度静态视野平均缺损度(mean defect,MD)值与皮层激活体素数量成负相关,P-VEP P100波振幅与皮层激活体素数量成正相关。结论 功能磁共振成像可以反应视神经炎的皮层功能改变,在视神经炎患者中具有一定的应用价值。     

视神经脊髓炎NMO-IgG和脊髓病灶特点关系研究

背景：水通道蛋白-4抗体（antibodies against aquaporin-4, AQP4-Ab, NMO-IgG）是视神经脊髓炎（neuromyelitis optica,NMO）的诊断标志物,已纳入2006年修订的NMO诊断标准。最近研究表明,脑脊液（cerebrospinal fluid,CSF）NMO-IgG检测提高NMO诊断的敏感度。因此,NMO-IgG阳性患者应包括CSF NMO-IgG阳性的患者,而只有血清和CSF NMO-IgG均呈阴性的患者才能认为是NMO-IgG阴性。过去的研究提示,血清NMO-IgG 阳性与长节段脊髓病灶（longitudinally extensive spinal cord,LESC）和线样病灶密切相关。然而,NMO-IgG阳性和阴性患者脊髓MRI的特点,包括两者LESC和线样病灶的差别还不清楚。 目的：分析视神经脊髓炎患者NMO-IgG状态和脊髓MRI( magnetic resonance imaging)特点的关系。 方法：回顾性分析52名视神经脊髓炎患者的临床资料和脊髓MRI图像。8名患者血清及CSF NMO-IgG均呈阴性,血清或CSF NMO-IgG阳性患者44名。对比NMO-IgG阳性和阴性患者的临床和脊髓MRI特点。 结果：NMO-IgG阴性患者性别比率（男/女）较高（NMO-IgG阴性组4/4,阳性组4/40,p=0.014）。轴位MRI上,NMO-IgG阴性组病灶于脊髓横断面周围(50.0%)多见,这与NMO-IgG阳性组主要分布在脊髓中央(54.5%)不同(p=0.051)。LESC在两组均多见。在NMO-IgG阴性患者中未发现线样病灶,而NMO-IgG阳性组线样病灶常见(47.7%) (p=0.016)。阳性患者中,延髓（linear medullary,LML）、延髓脊髓（linear medullospinal lesion,LMSL）和脊髓（linear spinal lesion,LSL）线样病灶比例分别是27.3%, 11.4%和9.1%。 结论：NMO-IgG阴性患者与NMO-IgG阳性患者有不同的临床特点。NMO的确诊应综合考虑,NMO-IgG阴性和非典型脊髓病灶出现不能排除NMO的诊断。进一步阐明NMO-IgG的作用,并寻找其他潜在抗原对NMO发病机制的研究有重要意义。     

视神经脊髓炎谱系疾病与多发性硬化患者部分脑脊液免疫标志物的比较分析

目的 观察血清、脑脊液(cerebrospinal fluid,CSF)白蛋白(albumin,ALB)、免疫球蛋白、补体水平及相关合成指标在多发性硬化(multiple sclerosis,MS)和视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)中的变化,初步分析上述指标在两种疾病诊断及鉴别诊断中的意义。方法 选取91例已确诊神经系统脱髓鞘患者,包括MS患者33例,NMOSD患者58例,选取头痛患者50例作为非炎性对照组。测定血清及CSF中ALB、IgA、IgM、IgG以及血清C3、C4等浓度,并计算血清免疫球蛋白与CSF免疫球蛋白比值IgA Q、IgG Q、IgM Q、ALB Q及与年龄相关的Reiber双曲线上部判别值(Reiber's upper hyperbolic discrimination line, Qlim)(ALB),计算ALB Q>Qlim (ALB)比例及IgG 24 h鞘内合成率,比较分析MS、NMOSD与头痛对照组间各指标差异。结果 MS和NMOSD组患者CSF IgG、IgG Q、IgG 24 h鞘内合成率和ALB Q>Qlim (ALB)比例均显著高于对照组(P<0.05),且MS组患者IgG 24 h鞘内合成率和IgG Q明显高于NMOSD组(P<0.05)。结论 MS和NMOSD均有血-脑脊液屏障功能损伤,而鞘内IgG的合成是MS和NMOSD的一个关键区分点,在两者的鉴别诊断中有重要意义。     

Cardiovascular magnetic resonance imaging findings in children with myocarditis

Background Myocarditis is a common,potentially life-threatening disease that presents a wide rang of symptoms in children,as an important underlying etiology of other myocardial diseases such as dilated and arrhythmogenic right ventricular cardiomyopathy.The incidence of nonfatal myocarditis is probably greater than that of the one actually diagnosed,which is the result of the challenges of establishing the diagnosis in standard clinical settings.Currently,no single clinical or imaging finding confirms the diagnosis of myocarditis with absolute certainty.Historically,clinical exam,electrocardiogram （ECG）,serology and echocardiography had an unsatisfactory diagnostic accuracy in myocarditis.Endomyocardial biopsy remains as a widely accepted standard,but may not be suitable for every patient,especially for those with less severe disease.Our aim was to find the changes in cardiovascular magnetic resonance （CMR） imaging of children with myocarditis diagnosed by clinical criteria.Methods We studied 25 children （18 male,7 female; aged from 5-17 years） with diagnosed myocarditis by clinical criteria.CMR included function analyses,T2-weighted imaging,T1-weighted imaging before and after i.v.gadolinium injection （early gadolinium enhancement （EGE） and late gadolinium enhancement （LGE））.Results The T2 ratio was elevated in 21 children （84％,11 in anterolateral （44％）,5 in inferolateral （20％）,and 5 in septum （20％））,EGE was present in 9 children （36％,3 in anterolateral （12％）,4 in inferolateral （20％）,and 2 in septum （8％））,and LGE was present in 5 children （20％,2 in anterolateral （8％）,1 in inferolateral （4％）,1 in septum （4％）,and 1 in midwall of left ventricular （LV） wall）.In 9 children （36％）,two （or more） out of three sequences （T2,EGE,LGE） were abnormal.Conclusions The CMR findings in children with clinically diagnosed myocarditis vary within the groups,including regional or global myocardial signal increase in T2-w