临床孤立综合征的研究进展

临床孤立综合征(clinically isotated syndrome,CIS)是一种常见且复杂的中枢神经系统综合征,为多种脱髓鞘疾病的首发表现,早期预测CIS的转归对选择最优化治疗方案极为重要,本文就其临床及MRI研究进展进行综述,以增进对临床孤立综合征的了解。     

Interobserver agreement for diagnostic MRI criteria in suspected multiple sclerosis

MRI is the paraclinical test most widely used to support the diagnosis of multiple sclerosis (MS). We evaluated interobserver agreement in applying diagnostic criteria to MRI obtained at first presentation. Five experienced observers scored 25 sets of images consisting of unenhanced T2- and gadolinium-enhanced T1-weighted images (approximately half the sets were normal). We scored frontal, parietal, temporal, occipital, infratentorial and basal ganglia lesions and the total number of lesions on T2-weighted images; periventricular, callosal, juxtacortical and ovoid lesions and those > 5 mm in maximum diameter; contrast-enhancing and hypointense lesions. Based on a combination of imaging findings patients were classified as compatible or not compatible with MS according to composite criteria. Observer concordance was characterised by weighted kappa values (ϰ) and mean average difference to the median (MADM) scores. Using the raw scores, there was poor agreement for the total number of lesions on T2-weighted images, and for occipital, oval, juxtacortical and hypointense lesions. Moderate agreement was found for frontal, callosal, basal ganglia and large lesions on T2 weighting. Good agreement was attained for parietal, temporal, infratentorial and periventricular lesions. After dichotomisation according to accepted cut-off values, most criteria performed better, especially the number of lesions on T2-weighted images (P < 0.05). Good agreement was found for the criteria of Paty and Fazekas and moderate agreement for those of Barkhof. While experienced observers may not agree on the total number of lesions, they show quite good agreement for commonly used cut-off points and elements in the composite criteria. This validates the use of MRI in the diagnosis of MS, and the use of dichotomised and composite criteria. Reveived: 19 October 1998 Accepted: 17 November 1998     

多发性硬化MRI诊断（附22例报告）

目的：提高对多发性硬化（MS）MRI特征的认识。材料与方法：对22例MS患者行脑和／或脊髓MRI检查，对病变的分布、形态及病变的强化进行评价。结果：脑内病变位于侧脑室周围、半卵圆中心及脑干，室旁病变其长轴多于侧脑室垂直；脊髓病变多位于颈髓及上段胸髓，呈长条状。病变急性期及活动期强化，稳定期或斑痕期不强化。结论：MRI可显示MS的特征性表现，增强扫描可提高MRI对MS早期诊断的特异性，并能对MS的病理、生理特点进行时间顺序方面的观察。     

多发性硬化临床表现与MRI分析(附85例分析)

目的:为提高多发性硬化(multiplesclerosis,MS)的临床诊断水平。方法:回顾性分析了85例临床明确MS患者的临床与MRI资料,总结该病的临床与MRI特点。结果:MS好发于中年(平均发病年龄33.0±10.81岁)女性(61.2%);受凉和/或上呼吸道感染为最常见诱因(53.3%);常见临床症状依次为肢体无力(68.2%)、视力障碍(42.4%)和感觉障碍(42.4%);寡克隆区带和诱发电位检查是最重要的辅助检查手段。MS好发部位依次为侧脑室体旁(67.1%)、半卵圆中心(44.7%)、颈髓(41.2%)、胸髓(34.1%)和胼胝体(31.8%);病灶多呈斑片状(91.8%);在T1WI为低、稍低或等信号,在T2WI上多为高信号;病灶多呈斑片状、斑点状或环形强化。结论:结合临床与MRI表现,可提高对MS诊断的准确性。     

多发性硬化MRI表现及其诊断价值

目的：探讨多发性硬化(MS)的MRI表现特征及其诊断价值。方法：对21例MS患者进行了MRI检查，所有病例均行Gd-DTPA增强扫描。扫描序列包括：T1WI、T2WI、FLAIR，6例进行了随访观察。结果：21例均有MRI阳性表现，3例仅有脊髓病灶，12例仅有脑部病灶，6例脊髓与脑均有病灶。脑内病灶共358个，以侧脑室旁白质区和半卵圆中心多见。形态大小不等，幕上多于幕下。胼胝体受累6例，典型者病灶长轴与侧脑室体部垂直。脊髓表现为髓内长条状长T2信号影，颈髓多见。19例有不同程度的增强表现，病灶强化程度与病灶活动性明显相关。随访病例发现：2例病情稳定者无强化表现并无新病灶出现，4例病情反复者出现新病灶并有强化表现。结论：MRI能清楚显示脑和脊髓的MS病灶，并具有相对特征性的表现，能客观地反映病灶的活动性。     

临床孤立综合征和复发缓解型多发性硬化患者表现正常脑白质及脑灰质的MR扩散张量直方图比较

目的 评价扩散张量成像(DTI)对临床孤立综合征(CIS)的研究价值,了解CIS的病理变化机制及与复发缓解型多发性硬化(RRMS)的关系.方法 选择19例CIS患者(CIS组)、19例RRMS患者(RRMS组)和19例性别、年龄与之匹配的健康志愿者(正常对照组)为研究对象.用1.5 T超导型MR机采集数据,经图像后处理得到表现正常脑白质(NAWM),表现正常脑灰质(NAGM)的平均扩散率(MD)、各向异性分数(FA)直方图,其中提取出下列指标:平均值、直方图峰高和峰位置,进行单因素方差分析和秩和检验,并对3组NAWM、NAGM的MD、FA值与扩展残疾状态量表(EDSS)评分进行Spearman相关分析.结果 RRMS组患者表现正常脑白质MD为(0.83±0.04)×10-3mm2/s,较正常对照组(0.78±0.02)×10-3mm2/s、CIS组(0.79±0.02)×10-3mm2/s均显著增高(F=15.304,P＜0.01),但CIS组与正常对照组间差异无统计学意义(P＞0.05);MD图峰高CIS组明显低于正常对照组(P＜0.01);RRMS组平均FA值(0.36±0.03)较正常对照组(0.41±0.01)及CIS组(0.40±0.02)均降低(F=17.965,P＜0.01),但CIS组与正常对照组间差异无统计学意义(P＞0.05),平均FA图峰位置CIS组较正常对照组明显左移.NAGM MD在正常对照组、CIS组、RRMS组分别为(1.03±0.05)、(1.08±0.06)、(1.18±0.12)×10-3mm2/s,依次增高,且差异均有统计学意义(F=15.261,P＜0.01).CIS患者的各项DTI指标与EDSS评分均无显著性相关.RRMS患者NAGM的MD与EDSS评分呈正相关(r=0.568,P＜0.05).结论 DTI直方图可以敏感的显示及量化CIS及多发性硬化(MS)NAWM、NAGM的异常,作为MS最早期表现的CIS患者NAWM、NAGM均已发生了病理改变,但较MS病变轻.     

Visual analysis of serial T2-weighted MRI in multiple sclerosis: intra- and interobserver reproducibility

We evaluated the effect of consensus formation and training on the agreement between observers in scoring the number of new and enlarging multiple sclerosis (MS) lesions on serial T2-weighted MRI studies. The baseline and month 9 MRI studies of 16 patients with a range of MRI activity were used (dual-echo conventional spin-echo sequence, TR 2000, TE 34 and 90 ms, 5 mm contiguous slices, in-plane resolution 1 mm). First, the serial studies were visually analysed for the presence of new and enlarging lesions, on two occasions, by five experienced observers, without adopting any consensus strategy and in isolation. Next, the observers met to identify the common sources of inconsistencies in reporting between observers and formulate consensus rules. Finally, a further independent reading session was performed on the same MRI dataset, this time applying the consensus rules. Agreement between observers was assessed using kappa scores. Without the consensus rules, interobserver kappa scores for the first and second reading sessions for new lesions were only 0.51 and 0.39 respectively; agreement for enlarging lesions was even worse. The mean intraobserver kappa score for new lesions was higher at 0.72, reflecting the fact that the observers were consistently applying their individual assessment strategies. Application of the consensus rules did not lead to a significant improvement in inter observer kappas; the kappa scores adopting the guidelines were 0.46 and 0.21 for new and enlarging lesions respectively. Consensus guidelines thus did not improve the reproducibility of visual analysis of serial T2-weighted MRI, and the level of agreement between observers remained only moderate. Suboptimal repositioning is likely to be a major source of residual variability and this suggests a future role for image registration strategies; until then, a single observer, or pair of observers working in consensus, should be used in MS studies. Received: 6 April 1999 Accepted: 21 April 1999     

儿童多发性硬化的临床特点及MRI特征

目的 探讨中国儿童多发性硬化(MS)的临床孤立综合征( CIS)和复发时的临床及MRI特征.方法 回顾性分析16例MS患儿的首次发作及复发时临床及影像学资料.随访时间4个月至7年,期间患儿复发次数为1～5次.由1名儿科神经医师对CIS及复发的临床表现进行了归类总结.由1名资深神经影像学医师对患儿CIS及复发的头颅MRI表现进行分析,内容包括病灶的位置、大小、分布.病灶位置的分析包括皮层、皮层下白质、中央白质、脑室旁白质、深部灰质核团以及脑干和小脑.结果 (1)临床表现:儿童MS发病急,CIS以皮层症状及视觉障碍表现多见,14例1年以内复发,复发时皮层症状减少,而视觉症状仍较多,随访时康复良好.(2)颅脑MRI表现:CIS时,13例出现皮层下白质病灶,且大片融合,与中央白质病灶相连,好发部位依次为额、顶叶.皮层9例受累.10例可见中央白质病灶.6例可见脑室旁白质小病灶.4例可见对称性深部灰质核团病灶.5例可见脑干病灶.3例可见小脑病灶.3例可见视束或视神经肿胀、增粗.2例可见锥体束异常信号.1例可见胼胝体病灶.复发时,12例可见皮层下病灶,较CIS时数量增多,以小病灶为主.9例可见中央白质病灶,病灶大小较前减小.8例可见脑室旁病灶,病灶数量较CIS时增多.仅有2例出现皮层病灶.5例出现小脑病灶.4例可见脑干病灶.6例可见锥体束病灶,发生率较CIS时明显增多,且出现“轨道征”.结论 儿童MS的MRI表现具有一定特征,CIS时额、顶叶皮层下白质病灶融合成大片并常累及中央区白质,复发时有时可见“轨道征”,结合临床可以提高对儿童MS诊断的正确性.     

脊髓多发性硬化的MRI诊断

目的：提高对脊我发性硬化ＭＲＩ特征的认识。材料与方法：地１４例脊髓多发性硬化患者进行颈部ＭＲＩ检查。对病变的长度，横断面上病变大小、位置及病变的强化进行评价结果：１４例患者共发现病变３１个。脊髓多发性硬化ＭＲＩ特征性表现为；大多数为矢状位长度小于２个椎体（８７．１％），病变长度大于宽度，病变局部脊髓政党或轻度肿胀。结论：ＭＲＩ不仅可以发现脊髓多发化病变，并且能显示其特征性表现，有助于与其他脊人病变     

MRI parameters in multiple sclerosis patients

Summary Magnetic resonance imaging (MRI) findings of 20 patients with clinically definite multiple sclerosis (MS) are presented. The studies were performed on a 0.5 Tesla magnet using spin-echo technique. Analysis of the MRI findings included detailed linear measurements of the ventricular and the subarachnoid spaces and reading of the intensity of the grey and white matter and intensity of the MS plaques. The plaques were sorted according to their number and size. The younger patients (20–40 years) had overall more plaques than the older ones (over 40 years). The small plaques were the most numerous and the large ones were the least common. Statistically significant association was found between the number of plaques and the cella media width. The intensity ratios between the nonplaque white matter/grey matter showed a significant correlation with the ventricular score. A significant negative correlation was found between the antero-posterior diameter of the spinal cord and the number of MS plaques in the brain. The plaque/white matter ratio had a significantly negative correlation with the cervical cord's width.     

适合临床型MRI研究的多发性硬化模型的建立

斑块的病理特点.结论 2次注射MOG35-55能诱导出与多发性硬化临床表现和病理特点极为近似的大鼠模型.所建模型发病率高、抗原价廉易得,应用3.0 T临床型MR设备及正交腕关节线圈能活体监测急性期大鼠脑和脊髓病灶.MOG35-55诱发的多发性硬化大鼠模型是适合临床型MRI研究的理想模型.     

Klippel-Trenaunay综合征的MRI诊断

目的分析Klippel—Trenaunay综合征（KTS）的MRI影像表现，评价其在KTS诊断中的作用。方法对经临床和影像检查符合KTS诊断标准的31例KTS患者患肢进行MR常规扫描、MR血管成像（MRA）、静脉血管成像（MRV）和X线顺行静脉造影（XRV）检查，并观察肢体及静脉病变情况。结果12例MRI发现肢体软组织内血管瘤。软组织肥大27例。浅静脉曲张21例。静脉畸形27例。20例同时行MRV与下肢XRV的患者显示浅静脉曲张和特征性坐骨神经均分别为17和11例。下肢XRV检查显示静脉属支增多10例；MRV显示静脉属支增多15例和静脉行径异常4例。MRV显示深静脉异常8例，下肢XRV显示深静脉异常7例。结论MRI是诊断KTS有效和可靠的影像检查方法。     

Hallervorden-Spatz综合征的MRI诊断

目的：探讨Hallervorden-Spatz综合征的颅脑M RI诊断价值，提高对该病的认识。方法回顾性分析2007年2月～2012年4月经临床证实为 Hallervorden-Spatz综合征的4例患者颅脑MRI资料。结果 Hallervorden-Spatz综合征颅脑M RI表现为：双侧苍白球、黑质T2 WI像对称性低信号，苍白球低信号的前内侧出现斑点状高信号，即典型的“虎眼征”。SWI像上病灶呈低信号，MRS检查可见NAA、NAA/Cr及Cho/Cr下降。结论 Hallervorden-Spatz综合征具有典型的MRI表现，MRI检查在Hallervorden-Spatz综合征中有重要的诊断价值。     

多发性硬化性脑萎缩MRI评估及其临床意义

目的;研究多发性硬化的脑萎缩和神经机能缺损以及与病程的相关性,分析脑结构萎缩与病残程度的关系。方法：多发性硬化患者42例,其中复发缓解型（RR）25例,平均年龄31岁（17-41岁）;进展型（SP）17例,平均年龄38例（32-53岁）,健康对照组15例。据MRI多发性硬化病灶计算病损的体积。结果：MS患者的大脑白质、幕下结构、胼胝体容积较健康对照组明显减少（P＜0.01),上颈髓减小44.4%、小脑减小20.3%、脑干减小23.1%、胼胝体减小21.8%。SP组较RR组上的颈髓和大脑白质萎缩更为明显（P＜0.05至P＜0.01)。脑室扩大（r= 0.50,P＜0.01)与胼胝体体积减小（r=-0.55,P＜0.01)之间有明显相关性,病人组上颈髓萎缩与临床神经机能障碍明显相关,临床技能评分（SNRS）减少与上颈髓萎缩具相关性（r= 0.48,P＜0.01）,在脑白质上结构变化与对照组比较与病程明显相关（r=-0.47,P＜0.005),大脑白质萎缩与SNRS亦相关（r=0.41,P＜0.05)。中枢神经结构萎缩在多发性硬化的RR组、SP组之间的差异,尤其幕下结构在复发缓解型多发性硬化有更明显的变化。提示急性炎症所致的中枢性传导束变性,可能在多发性硬化中是较早发生的病理过程。MRI对多发性硬化的随访与预后评估有一定意义。     

Medullary lesion revealed by MRI in a case of MS with respiratory arrest

Summary A 66-year-old MS patient who suffered respiratory arrest followed by complete remission is reported. Magnetic resonance imaging (MRI) revealed a medullary lesion. Clinicoradiological relationships are discussed.     

MRI对脊髓型多发性硬化临床分期的价值

目的:探讨脊髓型多发性硬化(MS)的MRI表现及其病理基础,评价MRI对MS临床分期价值。方法:脊髓型MS患者43例,按临床特点分为急性期、静止期和缓解复发期。对所有患者行头部和脊柱磁共振检查,分析各期的MRI表现。结果:脊髓型MS的主要MRI表现为髓内长或等T1、长T2信号改变。脊髓型MS各期的MRI特点:①急性期11例,均表现为脊髓轻度~中度肿胀,增强扫描时10例(90.9%)髓内病灶有明显强化,其中9例呈斑片状或边缘环状强化,1例呈结节状强化,范围明显小于T2WI上所见;1例无强化。4例伴脊髓中央管扩张。有8例经治疗后病灶缩小,脊髓肿胀消失。②静止期17例,14例(82.4%)脊髓形态、大小未见异常,髓内病灶均无强化。③缓解复发期15例中,11例(73.3%)伴有脊髓萎缩,增强扫描时5例出现轻度点状或条状强化,病变范围与T2WI所见相似。各期脊髓型MS的病变范围及分布差异无显著性意义(P>0.05)。结论:MRI能反映脊髓型MS各期的病理变化,对脊髓型MS的分期具有重要价值。     

MRI in the investigation of patients with myelopathy thought to be due to multiple sclerosis

The role of cerebral and spinal cord MRI was investigated in 65 patients with myelopathy suspected of having demyelinating disease. Cerebral MRI demonstrated lesions compatible with demyelination in 80% and spinal cord MRI in 68.6%. In 28.5% of our patients brain lesions were present with normal spinal cord images, but in 17% spinal cord lesions were depicted with a normal brain MRI. The combination of the two examinations demonstrated lesions in 97% of the patients. The frequency of coexistent cerebral lesions in patients with spinal cord lesions was over 85% in patients with chronic disease but only 28.5% in patients with acute myelitis.     

Ventricular enlargement in multiple sclerosis: a comparison of three-dimensional and linear MRI estimates

Atrophy of central white matter is related to irreversible clinical disability in multiple sclerosis (MS) and ventricular enlargement may be a sensitive marker of this tissue loss. Therapeutic trials in MS have provided MRI data for investigation of cerebral atrophy in MS. These studies use almost exclusively two-dimensional (2-D) images, which may be limited in the assessment of three-dimensional (3-D) structures. We used 3-D MRI data to estimate ventricular volumes in 40 patients with MS and 10 healthy controls, to look at associations with clinical disability and the stage of the disease. We then compared simple linear measures of ventricular size from conventional 2-D images, with 3-D volume estimates to establish the best available linear indices of ventricular volume. Mean ventricular volumes were increased in the patients and significantly larger in the more disabled patients. The estimated volume of the third ventricle obtained from 3-D MRI showed the strongest association with the clinical stage of the disease, duration of symptoms and levels of disability. Finally, we confirmed that in patients with MS accurate data on ventricular size can be obtained from 2-D images by two simple and convenient linear measures, the width of the third ventricle and of the anterior horn of the lateral ventricle. Received: 17 April 2000 Accepted: 25 August 2000     

Limited duration of the effect of methylprednisolone on changes on MRI in multiple sclerosis

Treatment with methylprednisolone reduces the duration and severity of clinical relapses in multiple sclerosis (MS), while reducing the number of gadolinium-enhancing lesions on T1-weighted MRI. We performed serial MRI imaging after methylprednisolone treatment to see whether suppression of enhancement persists and whether related abnormalities on T2-weighted images disappear at follow-up. Thirteen patients with definite MS received a total of 31 courses of methylprednisolone over an average period of 50 weeks. Gadolinium-enhanced MRI was obtained before and after treatment, then at monthly intervals, using a standardised repositioning and imaging protocol. Two experienced readers in conference defined the number of active (gadolinium-enhancing and new or enlarging nonenhancing) lesions. We detected 609 active lesions on 195 examinations. Directly after treatment the reduction in the number of enhancing lesions was 78%, indicating restoration of the BBB and suppression of inflammation. It was uncommon for a lesion which stopped enhancing to show enhancement on a subsequent examination. No beneficial effect was observed on the rate of disappearance of related abnormalities on T2-weighted images, indicating persistent change such as oedema, cellular infiltration or demyelination. Moreover, in 89% of cases, an increase in the number of active lesions was observed before new clinical activity, if any, was observed (on average 52% earlier). MRI enabled us to demonstrate that the duration of the effect of methylprednisolone treatment is temporary (on average 9.7 weeks).Presented at the 11th Annual Meeting of the Society of Magnetic Resonance in Medicine, Berlin, August 1992, and the 8th Congress of the European Committee for Treatment and Research in Multiple Sclerosis, Barcelona, October 1992     

超小超顺磁性氧化铁粒子标记免疫细胞在多发性硬化症动物模型MRI中的应用

超小超顺磁性氧化铁粒子(USPIO)是一种外周包裹右旋糖酐的氧化铁纳米颗粒,可以被多种细胞吞噬,主要为单核巨噬细胞。由于其含铁,在T2和T2*加权磁共振(MR)图像上产生低信号。多发性硬化症(MS)是一种发生在中枢神经系统的自身免疫性脱髓鞘性疾病,实验性变态反应性脑脊髓炎(EAE)为其常用的动物模型,T细胞、单核巨噬细胞在疾病的发生、发展中起着重要作用,参与病程的各个阶段。USPIO标记免疫细胞MRI可以在体监测MS、EAE病灶的炎性细胞浸润情况,对疾病的复发、发展、早期诊断以及疗效监测均具有重要意义。