磁共振中央静脉征对多发性硬化诊断的意义

正多发性硬化(multiple Sclerosis,MS)是免疫介导的中枢神经系统脱髓鞘疾病中的最常见一种类型,尽管目前在其诊断和治疗方面有了长足进步,仍有很多不完善的地方,特别是晚期治疗难度更大,因此MS早期诊断一直是临床研究的重点之一。由于MS临床表现特异性不强,亦缺乏疾病特异性的实验室检查指标,因此其诊断一直存在一定难度,目前仍主要依靠排他性诊断。     

复发-缓解型多发性硬化患者丘脑扩散张量成像研究

目的 通过扩散张量成像研究复发-缓解型多发性硬化患者常规MRI表现正常的丘脑扩散参数异常,以及与临床残疾程度和认知损害间的相关性.方法 24例复发-缓解型多发性硬化患者和与之性别、年龄相匹配的健康志愿者分别接受常规MRI和扩散张量成像检查,利用兴趣区法测量影像正常的丘脑扩散参数,比较两组受试者丘脑平均扩散率和部分各向异性间的差异性,并评价患者丘脑扩散参数与临床相关评分及病灶体积之间的相关关系.结果 复发-缓解型多发性硬化组患者丘脑平均扩散率[(85.34+14.68)x10-3mm2/s]低于正常对照组[(98.42±13.10)×10-3mm2/s],组间差异具有统计学意义(t=-3.257,P=0.002);丘脑部分各向异性(0.40±0.04)高于正常对照组(0.36±0.05),差异亦有统计学意义(t=3.163,P=0.003).复发-缓解型多发性硬化组患者丘脑平均扩散率与同步听觉连续加法测验评分呈显著正相关(r= 0.711,P=0.000).结论 对常规MRI表现正常的复发-缓解型多发性硬化患者,扩散张量成像可以发现丘脑异常.而且丘脑扩散异常与患者认知损害存在相关性,提示扩散张量成像作为评价临床功能的重要指标,具有很好的应用前景.     

复发缓解型多发性硬化患者脑灰质弥散张量成像研究

目的利用弥散张量成像(DTI)直方图分析,明确复发缓解型多发性硬化(RRMS)患者表现正常脑灰质(NAGM)是否存在隐匿性损伤,并研究脑灰质DTI直方图指标与扩展残疾功能状态量表(EDSS)评分的相关性。方法对24例RRMS患者和24名性别及年龄匹配的健康志愿者行常规磁共振成像(MRI)和DTI检查,分割提取NAGM后,绘制出NAGM的平均弥散率直方图并对其进行分析。结果RRMS患者NAGM的平均弥散率[(1·134±0·086)×10-3mm2/s]明显高于健康志愿者[(0·993±0·042)×10-3mm2/s,t=7·198,P<0·01],平均弥散率直方图峰位置[(0·880±0·089)×10-3mm2/s]也明显高于健康志愿者[(0·812±0·017)×10-3mm2/s,t=3·685,P=0·001],而平均弥散率直方图峰高(6·138‰±1·371‰)明显低于健康志愿者(8·889‰±1·339‰,t=7·032,P<0·01)。RRMS患者NAGM的平均弥散率直方图各指标间的相关性明显高于健康志愿者。在RRMS患者,所有NAGM的平均弥散率直方图指标与EDSS评分均无相关性。结论RRMS患者的NAGM内存在隐匿性损伤。     

模拟多发性硬化的脑小血管病:影像学特征和病理机制

如何准确诊断多发性硬化(multiple sclerosis,MS)是富有挑战性的临床问题。尽管已有诸多指南和共识定义了MS的典型影像学特征,然而在临床实践中,由于非典型临床表现和同病异像的干扰,许多其他的神经科疾病往往被误诊为MS。脑小血管病(cerebral small vessel disease,CSVD)的临床症状和影像学表现与MS相似,因此容易被误诊为MS。通过检索既往文献报道的误诊病例,发现遗传性CSVD和获得性CSVD的不典型影像学表现可以模拟MS,并且满足MS的影像学诊断标准而被误诊。然而,从病理机制角度进行深入探讨,发现MS和CSVD均有相应的特征性表现,同时引起误诊的白质高信号病灶在形态学和分布等特征上存在可辨识的差异。影像学特征的差异反映了2类疾病发病机制的差异。本文对既往文献报道的误诊为MS的CSVD病例进行复习,从影像学特征和病理机制角度探讨2者的异同,旨在帮助临床医师更好地鉴别MS与CSVD,避免误诊。     

复发缓解型多发性硬化患者疲劳的发生率及相关因素

目的 探讨复发-缓解型多发性硬化(relapsing remitting multiple sclerosis, RRMS)患者疲劳症状的发生率及相关危险因素。方法 收集2018年8月至2021年7月南京梅山医院神经内科收治的RRMS患者共80例,平均年龄(42.5±8.9)岁,其中男性38例,女性42例。根据患者是否发生疲劳将患者分为疲劳组(n=39)和非疲劳组(n=41)。采用自编问卷收集患者人口学资料与临床特征资料;采用疲劳影响量表修订版(MFIS)评估患者的疲劳症状,以MFIS分≥38定义为疲劳;采用贝克抑郁量表第2版中文版评估抑郁症状;采用临床扩展致残量表(EDSS)评估神经系统损伤程度;采用健康状况调查问卷(SF-36)评估健康状况。比较疲劳组和非疲劳组间临床资料的差异,并采用二元多因素Logistics回归分析RRMS患者疲劳的相关危险因素。结果 RRMS患者疲劳的发生率为48.8%(39/80),二元多因素Logistic回归分析显示,年龄(OR=0.91,95%CI:0.84～0.99)、躯体健康总评分(OR=0.99,95%CI:0.97～1.00)和精神健康总评分...     

基于功能连接的复发缓解型多发性硬化患者默认网络静息态功能磁共振成像

目的 采用静息态功能磁共振成像(rs-fMRI)基于种子点相关性分析技术对复发缓解型多发性硬化(RRMS)患者默认网络的功能连接改变进行研究.方法 使用3.0T磁共振采集RRMS组和健康对照组(各27例)rs-fMRI数据.数据经预处理后,选择后扣带回(-5,-49,40)为种子点,采用基于种子点相关性分析技术进行功能连接分析,分别在默认网络内和默认网络外脑区比较两组功能连接的差异.分析差异脑区与临床参数如临床扩展残疾量表、同步听觉连续加法测验评分(PASAT)、脑实质分数、T2可见病灶数和病程的相关性.结果 基于种子点相关性分析技术构建的RRMS患者默认网络包含脑区主要有前额叶皮质腹侧、双侧顶下叶、后扣带回及楔前叶等脑区.在默认网络内比较,RRMS患者较健康对照组右侧额上回功能连接下降;右侧小脑后叶、右侧小脑脚、右侧颞中回、右侧额中回、左侧楔前叶及扣带回、右侧角回、右侧扣带回功能连接增高.RRMS患者组默认网络内差异脑区中,右侧颞中回功能连接系数(0.387±0.216)与PASAT呈负相关(r=-0.590,P =0.001);患者右侧额上回功能连接系数(0.039±0.293)与病程之间呈负相关(r=-0.390,P=0.041).在默认网络外比较,RRMS组后扣带回功能连接下降脑区有右侧额上回、左侧枕中回、左侧中央前回;功能连接增高脑区有右侧小脑前叶(含齿状核)、右侧额叶白质区.RRMS组后扣带回与左侧中央前回、右侧小脑前叶功能连接系数(-0.924±0.253和0.217±0.208)分别与病程之间存在正相关(r =0.650,P=0.000;r =0.436,P=0.023).结论 RRMS患者默认网络内和默认网络外均出现后扣带回静息态功能连接的异常改变,表明患者存在功能下降和代偿的复杂过程.RRMS患者存在有限功能重构或重组,以维持默认网络的功能稳定.     

中枢神经系统脱髓鞘病变与复发-缓解型多发性硬化

参照Poser诊断标准．对1999年1月～2004年1月问经临床、头部和脊髓MRI、脑脊液IgG合成率和VEP检查后，诊断为中枢神经系统脱髓鞘病的53例患者．经5年临床和MRI随访．有36例符合缓解-复发型多发性硬化(MS)的临床确诊和实验室支持诊断标准，现将病情演变及诊断经过进行分析．以探讨复发-缓解型MS的早期诊断，为预防复发、控制病情进展提供治疗方面依据。     

应用体素的形态学研究复发缓解型多发性硬化患者的全脑灰质萎缩特点

目的 利用基于体素的形态学研究方法比较复发缓解型多发性硬化(relapsingremitting multiple selerosis,RRMS)患者和健康志愿者局部脑灰质的体积差异,推断灰质体积变化可能的病理生理机制.方法 对32例RRMS患者和32名性别、年龄匹配的健康志愿者进行常规MRI和三维T1WI扫描,采用参数统计软件包SPM5进行图像后处理,对RRMS组及对照组数据进行基于体素的统计学比较.利用相关分析检测患者灰质体积的变化与疾病病程、临床残疾程度及脑内可见病灶体积的相关性.结果 与对照组相比,RRMS患者的灰质萎缩区域分布广泛,在两侧丘脑(左侧2031,右侧1711)、尾状核(左侧815,右侧1031)、海马旁回(左侧313,右侧467)及额、颞、顶、枕叶多个皮质区域,灰质体积差异具有统计学意义(t=8.853～11.163,校正后均P＜0.01).RRMS患者两侧丘脑(左侧r=-0.596,右侧r=-0.694)和右侧尾状核(r=-0.409)的体积与脑内可见病灶的体积呈显著负相关(均P＜0.05).结论 RRMS患者灰质萎缩具有分布广泛的特征,尤其在深部灰质更显著.灰质萎缩的关键机制可能是继发于脑内可见病灶的神经元或轴索变性.

Abstract：

Objective To investigate the feature of regional grey matter volume changes in relapsing-remitting multiple sclerosis (RRMS) patients by voxel-based morphometry ( VBM) and presume the possible pathophysiological basis.Methods Conventional magnetic resonance imaging (MRI) and T1-weighted three-dimensional MRI were obtained from 32 RRMS and 32 sex- and age-matched normal controls.The comparison of grey matter volume between the two groups was analyzed by statistical analysis software SPM5 and VBM.A Pearson correlational analysis was used to assess correlation between gre matter loss and disease duration,expanded disability status scale (EDSS) and visible brain lesion volume.Results Compared with normal controls,RRMS patients had extensive bilateral grey matter atrophy in thalami (left 2031 and right 1711),caudate (left 815 and right 1031) and parahippocampal gyrus (left 313 and right 467),as well as several cortical regions in frontal,temporal,parietal,and occipital lobes (t value were between 8.853 and 11.163,all P ＜ 0.01).Regional grey matter loss in bilateral thalami ( r value were - 0.596 on left and were - 0.694 on right) and right caudate ( r = - 0.409 ) were strongly negatively correlated with visible brain lesion volume in RRMS (all P ＜ 0.05 ).Conclusions By means of VBM,extensive grey matter atrophy are found in RRMS patients,especially in deep grey matter.Axonal degeneration secondary to visible brain lesions may be a key pathogenesis of grey matter atrophy in RRMS.     

诱发电位和MRI检查在多发性硬化诊断中的价值

目的探讨诱发电位(EPs)和MRI检查在多发性硬化(MS)诊断中的价值。方法收集69例MS患者的临床资料、视觉诱发电位、体感诱发电位、脑干听觉诱发电位、磁刺激运动诱发电位以及MRI结果,比较不同检测方法对其临床诊断的价值。结果 MS患者的视觉诱发电位、体感诱发电位、听觉诱发电位、运动诱发电位以及MRI的异常检出率分别为69.57%(48/69)、50.72%(35/69)、55.07%(38/69)、42.03%(29/69)、78.26%(54/69)。4项诱发电位检查总异常检出率为86.96%(60/69),与MRI检查结果比较差异无统计学意义(P=0.178)。EPs和MRI检查均能发现临床下病灶:14例患者经MRI检查发现病灶但无相应临床症状;15例患者有临床症状而MRI检查未见相应病灶,但EPs检查可见异常。结论 MRI和EPs检查具有相互补充作用,结合临床合理选择使用此两种检查有助于提高MS诊断的敏感性。     

MRI在多发性硬化中的应用

近年来波谱成像、磁化传递成像、弥散张量成像、双反转回波成像等磁共振新技术在多发性硬化的早期诊断、病情评估、治疗监测、预后判断及研究中发挥着越来越大的作用。现就磁共振在多发性硬化中的应用做一综述。     

Central vein sign and paramagnetic rim sign: From radiologically isolated syndrome to multiple sclerosis

The widespread use of magnetic resonance imaging (MRI) has led to an increase in incidental findings in the central nervous system. Radiologically isolated syndrome (RIS) is a condition where imaging reveals lesions suggestive of demyelinating disease without any clinical episodes consistent with multiple sclerosis (MS). The prognosis for RIS patients is uncertain, with some remaining asymptomatic while others progress to MS. Several risk factors for disease progression have been identified, including male sex, younger age at diagnosis, and spinal cord lesions. This article reviews two promising biomarkers, the central vein sign (CVS) and the paramagnetic rim sign (PRS), and their potential role in the diagnosis and prognosis of MS and RIS. Both CVS and PRS have been shown to be accurate diagnostic markers in MS, with high sensitivity and specificity, and have been useful in distinguishing MS from other disorders. Further research is needed to validate these findings and determine the clinical utility of these biomarkers in routine practice.     

Disseminated encephalomyelitis and multiple sclerosis: two different diseases - a critical review

The practice of initiating immunomodulatory treatment immediately after a clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS) emphasizes the need to distinguish between disseminated encephalomyelitis (DEM) and MS. Their clinical, genetic, imaging, and histopathological characteristics establish that they are distinct disease entities. Acute and recurrent DEM are more common in children, but also occur in adults. DEM is polysymptomatic and includes signs and symptoms rarely encountered in MS, such as fever, alterations of the state of consciousness, cognitive and aphasic symptoms, and meningism. Cerebrospinal oligoclonal bands are rare. Magnetic resonance imaging (MRI) is the best means of distinguishing between DEM and MS. In the former, the lesion load is heavy, thalamus or basal ganglia are often affected, and early in the disease most of the lesions are usually larger than those of MS and enhance with gadolinium. The MRI spinal cord lesions are longer than three vertebral segments, and define neuromyelitis optica (NMO). Antibodies against aquaporin-4 are present in some NMO, but are also found in cases of MS and DEM. Most NMO are forms of DEM, not MS, and are identical with the 'Oriental' or 'optico-spinal' form of MS.     

Depressive symptoms and MRI changes in multiple sclerosis

To determine whether changes in specific regions of the brain can contribute to the development of depression in patients with multiple sclerosis (MS). We prospectively studied 90 patients with clinically definite MS. Disability, independence, cognitive performances, and depressive and anxiety symptoms have been assessed at baseline and 2 years later. At these two time-points, patients underwent a 1.5-T magnetic resonance examination of the brain including T1- and T2-weighted images. Calculation of regional and total lesion loads (LL) have been performed by a semiautomatic technique; total and regional brain volumes have been calculated by a fully automatic highly reproducible computerized interactive program. Measurements of LL did not show any significant difference between depressed and non-depressed patients. Brain atrophy was significantly more conspicuous in the left frontal lobe (P=0.039), in both frontal lobes (P=0.046) and showed a trend towards a difference in the right frontal lobe (P=0.056), in the right temporal lobe (P=0.057) and in both temporal lobes (P=0.072) of depressed patients. Disability, independence and cognitive performances were similar in depressed and non-depressed patients (P=NS). Spearman correlation analysis and multiple-regression analysis demonstrated that the severity of the depressive symptoms score was associated both with the disability score and the right temporal brain volume. Destructive lesions in the right temporal lobe can contribute to the severity of depression in patients with MS but the influence of the severity of neurological impairment should be taken into account.     

Magnetization transfer MRI in multiple sclerosis and other central nervous system disorders

The present review summarizes the major contributions given by magnetization transfer-magnetic resonance imaging to provide an accurate in vivo picture of the heterogeneity of central nervous system pathology and, ultimately, to improve our ability to monitor the evolution of various neurological conditions.     

Cerebral Cortex Involvement in Neuromyelitis Optica Spectrum Disorder

MethodsThe study enrolled 215 NMOSD patients who were seropositive for the anti-AQP4 antibody from 5 referral hospitals, and retrospectively analyzed their demographic, clinical, and MRI findings. Abnormal cerebral cortex lesions on brain MRI were identified by a neuroradiologist and two neurologists using consensus.ResultsMost of the 215 enrolled patients (87%) were female. The median age at onset was 22.5 years (range: 15–36 years) and the mean follow-up duration was 123 months. Brain lesions were found in 143 of 194 patients (74%) in whom MRI was performed during follow-up. Brain lesions involving the cerebral cortex were identified in 6 of these 194 patients (3.1%). Five of the patients were female, and the six patients together had a median age of 29 years (range: 15–36 years) at the time of lesion presentation. Three of them showed leptomeningeal enhancement in the lesions. At presentation of the cortex-involving lesions, five of these patients were not being treated at the time of presentation, while the sixth was being treated with interferon-beta.ConclusionsAlthough rare, cortical involvement occurs in NMOSD and is commonly combined with leptomeningeal enhancement. We speculate that this occurs only in patients who are not treated appropriately with immunosuppressant drugs.     

Brain MRI in late-onset multiple sclerosis

Multiple sclerosis (MS) with clinical onset after 50 years of age is unusual (between 1 and 6%) and is frequently misdiagnosed. Furthermore, brain magnetic resonance imaging (MRI) abnormalities are frequently observed in subjects over 50 years of age. The aim of this study was to describe brain MRI in late-onset MS to evaluate the sensitivity and specificity of radiological MS criteria in patients aged over 50 years. We evaluated the brain MRI of 20 patients with onset of MS after 50 years of age. We compared these MRI with 26 controls matched for age, sex and vascular risk factors. MRI were blindly analysed by two neuroradiologists according to Paty et al.'s [Neurology38 (1988) 180] criteria, Fazekas et al.'s [Neurology38 (1988) 1822] criteria and Barkhof et al.'s [Brain120 (1997) 2059] criteria. The mean age at MRI scanning was 58 years. Sensitivity was 90% for Paty et al.'s criteria, 80% for Fazekas et al.'s criteria and 85% for Barkhof et al.'s criteria. Specificity was 54% for Paty et al.'s criteria, 69% for Fazekas et al.'s criteria and 65% for Barkhof et al.'s criteria. Barkhof et al.'s criteria are less specific in older patients than in young patients. We suggest that spinal cord MRI and cerebrospinal fluid analysis should be systematically performed in suspected late-onset MS in order to increase the specificity of the diagnosis.     

磁共振质子波谱分析对多发性硬化的诊断价值

目的研究磁共振质子波谱分析(1HMRS)对多发性硬化(MS)的诊断价值。方法对29例MS患者(MS组)和26例正常志愿者(正常对照组)进行头颅MRI及1HMRS检查;计算其峰下面积,对脑部代谢产物氮-乙酰天门冬氨酸(NAA)、肌酸(Cr)、胆碱(Cho)的浓度进行定量,比较两组间NAA/Cr、Cho/Cr比值的差异。用扩展的功能障碍分级法(EDSS)对MS患者进行评分,分析MS组患者的NAA/Cr、Cho/Cr比值与EDSS评分之间的相关性。结果MS组的NAA/Cr、Cho/Cr比值分别为1.38±0.43、1.99±0.84,正常对照组为1.89±0.49、1.48±0.36。MS组的NAA/Cr比值显著低于正常对照组(P<0·05),Cho/Cr比值显著高于正常对照组(P<0·05)。MS组的NAA/Cr比值与EDSS评分之间呈负相关(r=-0.588,P<0·05),Cho/Cr比值与EDSS评分之间不相关(r=0·012,P>0·05)。结论MS患者的1HMRS检查有明显异常改变,NAA/Cr比值可反映MS患者临床神经功能障碍的程度。