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     

Growing Region Segmentation Software (GRES) for quantitative magnetic resonance imaging of multiple sclerosis: intra- and inter-observer agreement variability: a comparison with manual contouring method

Lesion area measurement in multiple sclerosis (MS) is one of the key points in evaluating the natural history and in monitoring the efficacy of treatments. This study was performed to check the intra- and inter-observer agreement variability of a locally developed Growing Region Segmentation Software (GRES), comparing them to those obtained using manual contouring (MC). From routine 1.5-T MRI study of clinically definite multiple sclerosis patients, 36 lesions seen on proton-density-weighted images (PDWI) and 36 enhancing lesion on Gd-DTPA-BMA-enhanced T1-weighted images (Gd-T1WI) were randomly chosen and were evaluated by three observers. The mean range of lesion size was 9.9-536.0 mm(2) on PDWI and 3.6-57.2 mm(2) on Gd-T1WI. The median intra- and inter-observer agreement were, respectively, 97.1 and 90.0% using GRES on PDWI, 81.0 and 70.0% using MC on PDWI, 88.8 and 80.0% using GRES on Gd-T1WI, and 85.8 and 70.0% using MC on Gd-T1WI. The intra- and inter-observer agreements were significantly greater for GRES compared with MC ( P<0.0001 and P=0.0023, respectively) for PDWI, while no difference was found between GRES an MC for Gd-T1WI. The intra-observer variability for GRES was significantly lower on both PDWI ( P=0.0001) and Gd-T1WI ( P=0.0067), whereas for MC the same result was found only for PDWI ( P=0.0147). These data indicate that GRES reduces both the intra- and the inter-observer variability in assessing the area of MS lesions on PDWI and may prove useful in multicentre studies.     

MR in the diagnosis and monitoring of multiple sclerosis: an overview

Multiple sclerosis is a chronic, persistent inflammatory-demyelinating disease of the central nervous system that typically presents as an acute clinically isolated syndrome attributable to a monofocal or multifocal demyelinating lesion, which usually affects the optic nerve, spinal cord, or brainstem and cerebellum. Although the diagnosis of multiple sclerosis is still based on clinical findings, magnetic resonance imaging is now integrated in the overall diagnostic scheme of the disease because of its unique sensitivity to demonstrate the spatial and temporal dissemination of demyelinating plaques in the brain and spinal cord. Conventional magnetic resonance imaging techniques, such as T2-weighted and gadolinium-enhanced T1-weighted sequences are highly sensitive in detecting multiple sclerosis plaques and provide a quantitative assessment of inflammatory activity and lesion load. However, there is a persisting mismatch between clinical and magnetic resonance imaging efficacy of approved treatments, which underlies the fact that this technique does not suffice to explain the entire spectrum of the disease process. In recent years, great effort has been dedicated to overcoming these limitations by using non-conventional magnetic resonance-derived metrics that can selectively measure the more destructive aspects of multiple sclerosis pathology and monitor the reparative mechanisms. These metrics, which include unenhanced T1-weighted imaging, measures of central nervous system atrophy, magnetization transfer imaging, proton magnetic resonance spectroscopy, diffusion-weighted imaging, and functional magnetic resonance imaging, provide a better approximation of the pathological substrate of the multiple sclerosis plaques, have increased our understanding of the pathogenesis of the disease, and have proven useful for studying the natural history of multiple sclerosis and monitoring the effects of new treatments. Therefore, magnetic resonance imaging not only plays an essential role in the diagnosis of multiple sclerosis, but can also serve as a true biological marker of the severity of this disease.     

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     

A single focus of probable multiple sclerosis in the cervical spinal cord mimicking a tumour

A case of probable multiple sclerosis (MS) in the cervical cord without brain involvement, mimicking a tumour is reported. The patient had a progressive left Brown-Séquard syndrome. The diagnosis was suggested by MRI. In spite of intensive corticotherapy, the neurological status worsened, in parallel with the radiological findings. Biopsy excluded an intramedullary tumour and supported the diagnosis of MS.     

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。     

Normalized regional brain atrophy measurements in multiple sclerosis

There is still a controversy regarding the best regional brain atrophy measurements in multiple sclerosis (MS) studies. The aim of this study was to establish whether, in a cross-sectional study, the normalized measurements of regional brain atrophy correlate better with the MRI-defined regional brain lesions than the absolute measurements of regional brain atrophy. We assessed 45 patients with clinically definite relapsing–remitting (RR) MS (median disease duration 12 years), and measured T1-lesion load (LL) and T2-LL of frontal lobes and pons, using a reproducible semi-automated technique. The regional brain parenchymal volume (RBPV) of frontal lobes and pons was obtained by use of a computerized interactive program, which incorporates semi-automated and automated segmentation processes. A normalized measurement, the regional brain parenchymal fraction (RBPF), was calculated as the ratio of RBPV to the total volume of the parenchyma and the cerebrospinal fluid (CSF) in the frontal lobes and in the region of the pons. The total regional brain volume fraction (TRBVF) was obtained after we had corrected for the total volume of the parenchyma and the CSF in the frontal lobes and in the region of the pons for the total intracranial volume. The mean coefficient of variation (CV) for RBPF of the pons was 1% for intra-observer reproducibility and 1.4% for inter-observer reproducibility. Generally, the normalized measurements of regional brain atrophy correlated with regional brain volumes and disability better than did the absolute measurements. RBPF and TRBVF correlated with T2-LL of the pons (r=–0.37, P=0.011, and r= –0.40, P=0.0005 respectively) and with T1-LL of the pons (r=–0.27, P=0.046, and r=–0.31, P=0.04, respectively), whereas RBPV did not (r=–0.18, P = NS). T1-LL of the frontal lobes was related to RBPF (r=–0.32, P=0.033) and TRBVF (r=–0.29, P=0.05), but not to RBPV (R=–0.27, P= NS). There was only a trend of correlation between T2-LL of the frontal lobes and RBPF (r=–0.27, P=0.06) and TRBVF (r=–0.28, P=0.057), and no correlation with RBPV (r=–0.23, P= NS). The magnitude of correlation between the expanded disability status scale (EDSS) and pontine and frontal lobe RBPF and TRBVF was more than twice as high as the correlation between EDSS and RBPV of the same regions. These data suggest that normalized regional brain atrophy measurements are preferable to absolute regional measurements in cross-sectional studies.     

多发性硬化的MR扩散加权成像研究

目的：总结多发性硬化（multiple sclerosis,MS)的扩散加权成像（diffusion-weighted imaging,DWI)表现，定量研究MS病灶区水分子表观扩散系数(apparent diffusion coefficient,ADC）值、扩散各向异性指数（anisotropy index,AI)的变化规律。方法：18例218个病灶分为5组：A组为MS急性期活动性病灶9例72个病灶，B组为A组中4例治疗后随访的病例，共31个病灶，C组为缓解－复发型的缓解期静止病灶9例（115个病灶），D组取病灶对侧或邻近的正常表现白质区域(normal appearance white matter,NAWM)，共218个，E组为正常对照组18例。总结病灶在DWI与常规MRI上的表现。测量病灶及临近正常表现白质区以及正常对照组相应区的ADC、AI。结果：在DWI上，进展型MS表现为高信号（T2WI表现为水肿样高信号）。缓解－复发型的急性发作期MS表现为环形或圆形高信号病灶。缓解－复发型的缓解期病灶与白质相比表现为稍高信号。各种分型与分期的MS病灶的ADC升高，AI下降，与NAWM及正常对照组间存在明显差异（F＝26．89，P＜0．01）。AI在病程后期表现为明显下降。MS病灶在T2WI上表现为高信号。强化MS病灶的ADC值 比非强化病灶的ADC值低（t＝4．19，P＜0．01），而2组的AI值之间无显著性差异（t＝0．99，P＞0．05）。结论：DWI与常规MR相比可以提供定量的诊断信息。能够反映MS不同临床分期的病理变化。扩散定量研究在MS的诊断、鉴别诊断以及疾病预后疗效中有重要的价值。     

A reproducible repositioning method for serial magnetic resonance imaging studies of the brain in treatment trials for multiple sclerosis

Serial MRI is an important measure of disease progression in evaluating the treatment of multiple sclerosis (MS). Accurate comparisons of scans for lesion activity and lesion volume require precise repositioning of patients. A simple, reproducible repositioning method is described. In a multicenter treatment trial of MS using β-interferon-1b, this method has been successful, with only 1.1% of scans being rejected because of poor repositioning.     

Database for serial magnetic resonance imaging in multiple sclerosis

The unique sensitivity of magnetic resonance imaging (MRI) in detecting disease activity in multiple sclerosis (MS) and the objective nature of the information obtained suggest that MRI will be a useful and reliable way of monitoring treatment trials. There is a need to develop an appropriate database which would provide a standardised means of assessment, not only of MRI, but also of essential clinical information. As part of the program of Concerted Action in Multiple Sclerosis, funded by the Commission of the European Community (CEC), we have developed a database for recording serial brain MRI results. The database consists of core, entry and follow-up sections. Both entry and follow-up parts are subdivided into clinical, MR system and MRI data. We expect that the use of this database will maximise efficiency of MRI monitoring in MS treatment trials, particularly in multicentre studies.     

Late-onset tumefactive multiple sclerosis

A patient with tumefactive multiple sclerosis (MS) initially presented at the age of 87 years; the revised diagnostic criteria from the International Panel on MS (2001) were fulfilled. Late-onset MS and tumefactive demyelinating lesions are discussed. This case suggests that MS can occur at any age. MS should be borne in mind for differential diagnosis if a brain tumor-like lesion with little mass effect and edema is found in an elderly patient.     

The contracting cord sign of multiple sclerosis

Summary A new sign of multiple sclerosis, the contracting cord sign, is described. The myelographic demonstration of a large cord that subsequently decreases in size may suggest multiple sclerosis. Multiple sclerosis must be considered in the differential diagnosis of an enlarged spinal cord. Distinguishing between the collapsing cord and the contracting cord is discussed.     

Magnetic resonance in multiple sclerosis

Summary Magnetic Resonance Imaging was performed in more than 200 patients with clinical suspicion or knowledge of Multiple Sclerosis. One hundred and forty-seven (60 males and 87 females) had MR evidence of multiple sclerosis lesions. The MR signal of demyelinating plaques characteristically has prolonged T1 and T2 relaxation times and the T2-weighted spin-echo sequences are generally superior to the T1-weighted images because the lesions are better visualized as areas of increased signal intensity. MR is also able to detect plaques in the brainstem, cerebellum and within the cervical spinal cord. MR appears to be an important, non-invasive method for the diagnosis of Multiple Sclerosis and has proven to be diagnostically superior to CT, evoked potentials (EP) and CSF examination. In a selected group of 30 patients, with the whole battery of the relevant MS studies, MR was positive in 100%, CT in 33,3%, EP in 56% and CSF examination in 60%. In patients clinically presenting only with signs of spinal cord involvement or optic neuritis or when the clinical presentation is uncertain MR has proven to be a very useful diagnostic tool for diagnosis of MS by demonstrating unsuspected lesions in the cerebral hemispheres.     

重型颅脑创伤合并多发伤漏诊分析

目的 探讨重型、特重型颅脑创伤伴有多发伤漏诊规律和防治策略. 方法 收集2000年1月-2007年8月收治的432例符合重型、特重型颅脑创伤合并多发伤(ISS≥20分)诊断标准患者的临床资料并对其进行回顾性分析,将其分为漏诊组和尤漏诊组,对ISS、GCS、漏诊的部位、时间、预后进行相关性分析. 结果 432例患者中有54例漏诊,ISS为(42.97±10.94)分,与无漏诊组比较,差异有统计学意义(P<0.05).漏诊组GCS≤8分的患者比例高于非漏诊组(P<0.05). 结论 迅速准确的程序化伤情判断、贯穿生命第一原则的全面体检、重点辅查及动态复查是防止漏诊、提高重型颅创伤合并多发伤生存率并减少致残率的有效措施.     

Intercenter agreement of brain atrophy measurement in multiple sclerosis patients using manually-edited SIENA and SIENAX

PURPOSE: To investigate intercenter agreement of brain volume (change) measurement in multiple sclerosis (MS) using structural image evaluation using normalization of atrophy (SIENA) and the cross-sectional version of SIENA (SIENAX) with additional manual editing to correct for inadequate brain extraction. MATERIALS AND METHODS: Baseline and follow-up T1-weighted MR images of 20 MS patients were dispatched to five centers. Each center performed fully-automated and manually-edited analyses for SIENAX, yielding normalized brain volume (NBV), and SIENA, yielding percentage brain volume change (PBVC). Intercenter agreement was assessed with the concordance correlation coefficient (CCC). RESULTS: Intercenter agreement was perfect for fully automated NBV and PBVC (both CCC = 1.0), and remained substantial upon manual editing (CCC = 0.94 for NBV, CCC = 0.95 for PBVC). Mean NBV values for each center decreased significantly after manual editing (overall mean NBV = 1605.3 cm(3) vs. 1651.1 cm(3) without manual editing; t = -4.58, P < 0.001). Total variance in PBVC decreased significantly by a factor of 1.8 after manual editing (sigma(2) = 2.82 before, and sigma(2) = 1.54 after manual editing, P < 0.05). CONCLUSION: Substantial intercenter agreement was found for manually-edited SIENAX and SIENA, suggesting that measurements from multiple centers may be pooled. Manual editing reduces overestimation of NBV, and is likely to increase statistical power for PBVC.     

Contrast-enhanced MR imaging in the evaluation of treatment response and prediction of outcome in multiple sclerosis

MR imaging is becoming increasingly important in the evaluation of multiple sclerosis based on its sensitivity to acute, often subclinical events in the brain and because it provides a basis for measuring the accumulation of disease over time. Contrast-enhanced MR imaging in particular evaluates disease at the fundamental level of events affecting the blood-brain barrier. This review emphasizes (a) recent developments in the use of contrast-enhanced MR imaging as a measure of disease in patient groups and individuals and (b) its emerging role in evaluating new therapies.     

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。     

多发性硬化与视神经脊髓炎影像对比研究

目的 比较多发性硬化(MS)与视神经脊髓炎(NMO)的影像特点,以提高对MS与NMO的临床鉴别诊断能力.方法 回顾性分析60例MS及48例NMO患者的头颅及脊髓MRI的影像特点,包括对发病部位、形态特点以及增强扫描表现等进行对比分析,采用SPSS 13.0软件包对所有数据进行统计学处理.统计学方法采用t检验和x2检验.结果 (1)发病部位:MS脑内好发部位依次为:侧脑室旁(34/60)、皮层下白质(27/60)、脑干(23/60),还可累及基底节区、小脑、胼胝体与丘脑,而皮层受累(9/60)也较为常见;59.4％ (19/32)的NMO患者脑内发现病灶,而NMO好发部位依次为:脑干(13/19)、侧脑室周围(12/19)、皮层下白质(7/19),特别是NMO第三脑室周围受累(6/19)以及脑干被盖导水管周围(8/19)病灶在MS未见类似影像,而脑干及丘脑病灶NMO也较MS更为常见(x2值分别为5.267、6.004,P值均＜0.05).(2)形态特点:脊髓MR扫描发现,MS病灶形态以卵圆形居多,并多靠周边且不对称,而NMO则以纵向融合及居中病灶多见;同时在脊髓MS的受累平均节段数为2.2个,显著少于NMO的7.3个(t=-9.288,P＜0.01);而其脊髓病灶数为2.0个,显著多于NMO的1.3个(t=4.565,P＜0.01),差异均有统计学意义;58.3％ (28/48)的NMO脊髓发生肿胀,较MS(21.9％,7/32)更为多见(x2=10.370,P＜0.01).(3)增强扫描表现:头部MS主要表现为环状强化(7/42)、卵圆形强化(6/42)、不规则边缘强化(4/42),而NMO则主要表现为浅淡片状强化(5/11);脊髓MS主要表现为卵圆形强化(16/26)与线状强化(8/26),而NMO病灶较长,以条索状强化(26/35)、淡线状强化(12/35)较常见.结论 NMO与MS影像特点差异显著,有助于两者的临床鉴别.     

A rare presentation of multiple sclerosis: Schilder's disease

Schilder''s disease is a rare form of multiple sclerosis. It concerns mostly teenagers and young adults. The Clinical signs and symptoms might be atypical for early multiple sclerosis which often mimics intracranial neoplasm or abscess. Their coursemay be either progressive or relapsing and remitting, with a high sensitivity to steroids. The knowledge of this rare form ofmultiple sclerosis may help radiologists in assessing a precise diagnosis. We report the case of a young 22-year-old patientadmitted to the emergency room with an array of headache, vomiting and frontal syndrome. Magnetic resonance imaging shows2 bilateral demyelinating frontal areas. The patient was put under corticosteroids bolus with discreet improvement in her clinicalcondition. After 6 months of follow-up, we did not notice any real clinical improvement. Although Schilder''s disease isconsidered to be a variant of Multiple Sclerosis, its clinical and imaging features behaves more like a demyelinating conditionwith its monophasic course with, however, serious clinical consequences if the treatment is delayed.     

Evaluation of Gd-enhancement in brain MR of multiple sclerosis: image subtraction with and without magnetization transfer

The aim of our study was to test the possibility of using image subtraction in detecting enhancing lesions in brain MR scans with and without magnetization transfer (MT) in multiple sclerosis (MS). Ten MS patients underwent 1.5-T MR imaging of the brain with spin-echo T1-weighted sequences with and without MT, repeated after 0.1 mmol/kg of an usual two-compartment paramagnetic contrast agent (Gadoteridol, Gd-HP-DO3A). Precontrast images were subtracted from postcontrast. Enhancing lesions were counted on the postcontrast images only (post-Gd), comparing pre- and postcontrast images by direct visual control (pre/post-Gd), and on the subtracted images (SI) only. Without MT, 36 enhancing lesions were counted on post-Gd, 36 on pre/post-Gd, and 59 on SI; using MT, 69, 52, and 50, respectively. Significant differences were found for pre/post-Gd without MT vs SI without MT ( p=0.028) and vs pre/post-Gd with MT ( p=0.012) as well as for pre/post-Gd with MT vs post-Gd with MT ( p=0.028). With pre/post-Gd, MT allowed the detection of 1.6 enhancing lesions per patient more than without MT. Whereas the SI without MT allow the detection of an increased number of enhancing lesions, SI with MT do not. An off-site final assessment allowed calculation of sensitivity and positive predictive value as follows: without MT were 63 and 94% (post-Gd), 67 and 100% (pre/post-Gd), 96 and 88% (SI); and with MT were 93 and 73% (post-Gd), 96 and 100% (pre/post-Gd), 91 and 98% (SI), respectively. Thus, SI seem to increase the sensitivity without MT; moreover, they could be used to correct the pseudoenhancement that impair post-Gd images with MT.