回顾性比较Poser标准和McDonald标准

目的比较Poser标准和McDonald标准在多发性硬化诊断中的差异。方法对80例新诊断为多发性硬化患者的临床资料进行回顾性分析,分别应用Poser标准和McDonald标准对其进行诊断分类。结果80例患者中48例(60.00%)行脊髓MRI检查,阳性率为83.33%(40/48)。应用Poser标准,41例(51.25%)为临床确诊,10例(12.50%)为实验室确诊,17例(21.25%)为临床可能,余12例(15.00%)未能进行诊断分类。应用McDonald标准,45例(56.25%)为确诊多发性硬化,35例(43.75%)为可能多发性硬化。经Poser标准临床确诊的41例患者中,除4例不完全符合McDonald标准中确诊多发性硬化条件外,其余37例均符合;实验室确诊的10例患者中,4例符合McDonald标准中确诊多发性硬化条件,其余6例为可能多发性硬化。经McDonald标准确诊的45例患者中,37例符合Poser标准中临床确诊条件,4例为实验室确诊,1例为临床可能,余3例为原发进展型多发性硬化。18例患者(33.33%)脑脊液寡克隆区带检测阳性,26例(40.63%)IgG合成率升高。4例患者脑脊液寡克隆区带或IgG检测呈阳性反应且MRI病灶≥2个,经McDonald标准诊断为确诊多发性硬化。结论McDonald标准较Poser标准更为完善,尤其适用于原发进展型多发性硬化及临床孤立综合征患者。同时应用脊髓MRI检查以及脑脊液寡克隆区带和IgG检测有利于提高McDonald标准诊断的敏感性。     

多发性硬化的McDonald诊断标准评价情况

1983年,Poser等[1]提出了由临床、亚临床(诱发电位)和脑脊液(CSF)异常组成的多发性硬化(multiple sclerosis,MS)诊断标准(简称旧标准),在国内一直沿用至今。其中并没有把磁共振成像(MRI)异常作为诊断依据(直到1988年对该诊断标准的进一步完善),这很大程度上是因为当时MRI处于发展的早期,尚未普及。近年来,MRI已经成为诊断和鉴别诊断MS必不可少的手段之一。2002年国外研究表明,大约95%的临床确诊MS患者以及约2/3的临床孤立综合征(CIS)患者MRI的T2像或质子像会出现异常[2]。2001年国际MS诊断专家组在重新复习Poser诊断标准,并考虑…     

McDonald标准对多发性硬化的诊断意义

目的 研究McDonald标准对多发性硬化(MS)的诊断意义.方法 对47例MS患者,其中视神经脊髓型MS(OSMS)17例、传统型MS(CMS)30例,进行病史收集、查体及MRI,诱发电位、脑脊液检查.运用McDonald标准对MS患者进行诊断.采用Fisher精确检验对两亚型诊断结果 进行比较.结果 OSMS亚组符合McDonald标准确诊MS 16例(94.1%)、可能MS1例(5.9%),CMS亚组符合确诊MS 20例(66.7%)、可能MS 10例(33.3%),两亚组确诊比率的差异有统计学意义(OR=0.1250,95%CI:0.0144～1.083,P<0.05).其中,不需附加证据而符合临床确诊的OSMS患者为16例,而CMS患者为17例,另3例需要结合MRI和脑脊液证据后确诊.结论 McDonald标准诊断OSMS的准确性高于CMS,尤其在确诊MS时更明显.其原因可能与该标准确诊OSMS时主要依赖临床症状和体征,而对CMS需要严格的MRI证据的规定有关.     

颅脑多发性硬化的临床及MRI诊断

目的 探讨多发性硬化(MS)的临床及MRJ特征,提高对多发性硬化的认识及诊断水平.方法 对20例颅脑MS患者临床资料、病灶部位、形态、MR信号及强化特点、胼胝体改变进行回顾性分析评价.结果 MS以青、中年女性稍多见,急性、亚急性起病,多以视觉障碍或肢体感觉、运动障碍为首发症状.视觉诱发电位大多数异常.MRJ检查18例发现病灶,敏感性90%(18/20).病灶以双侧侧脑室旁、额叶皮层及皮层下、半卵圆中心多发.病灶大、小不等,多数为圆形、卵圆形."直角脱髓鞘征"及"白质变脏征"是两个较为典型的征象.T1WI上表现为等、低信号,T2WI及Flair序列上表现为高信号,Flair序列显示病灶更清晰.增强扫描病灶可呈结节状强化、环状强化、弧形强化或无强化.结论 MS的临床及MRI表现具有一定特征.MRI有助于脑部MS的诊断及鉴别诊断,是诊断MS最敏感的成像方法.     

2017修订版McDonald多发性硬化诊断标准解读

2010修订版McDonald多发性硬化(multiple sclerosis,MS)诊断标准被广泛应用于研究和临床实践。结合近年来的科学研究进展,MS诊断国际专家组在2010修订版McDonald MS诊断标准的基础上,提出了新的2017修订版McDonald MS诊断标准。主要修订内容包括:典型的临床孤立综合征(clinically isolated syndrome,CIS)患者,如已有临床或磁共振成像(magnetic resonance imaging,MRI)的空间多发证据,且脑脊液(cerebrospinal fluid,CSF)特异的寡克隆区带阳性,即允许MS诊断成立;在幕上和幕下病灶或脊髓综合征患者中,症状性病灶可用于空间和时间多发证据;皮质病灶可用于空间多发证据。2017修订版McDonald MS诊断标准更加简化和明晰,有利于MS的早期诊断;同时保留了2010修订版的特异性,旨在促进其在不同人群中的合理应用以降低误诊率。本文对2017修订版McDonald MS诊断标准的要点进行解读和评论。     

脊髓型多发性硬化临床诊断分析

目的 总结分析脊髓型多发性硬化(MS)的临床特点和MRI表现.方法 回顾性分析21例脊髓型MS的临床特点和MRI表现,所有患者行脊髓和颅脑MRI检查.结果 脊髓型MS除有脊髓病变的临床表现外,临床症状和体征的多样性是其特点,如感觉障碍、肢体无力、视力障碍等;脊髓内病灶的MRI特点是不规则斑片状和条带状异常信号,位于脊髓两侧和后部,在T:WI像上为高或稍高信号,在T1WI上为等信号或稍低信号;80.9%(17/21)脊髓型MS合并脑内病灶.结论 脊髓型MS临床表现呈多样性,MRI可以准确显示脊髓内病灶,颅脑MRI检查有助于脊髓型MS的诊断.     

多发性硬化临床与磁共振研究

目的：探讨多发性硬化（MS）的MRI表坎特点及与临床表现的相关性,以及MRI对MS的诊断价值。方法：对44例临床确诊的MS进行头部和／或脊髓MRI横断面,矢状面和冠状面扫描。结果：MS临床表现主要为视力减退,感觉障碍．肢体瘫痪,共济失调等。MRI表现为异常信号呈T1和／或等T1及长T2改变且分布广泛。病变分布以大脑皮质下白质、脑室周围、丘脑、胼胝体、脑干、小脑、颈胸段脊髓等明显、脑室旁椭圆形病灶与矢状位垂直及发现胼胝体脱髓鞘病灶为本病较具特征性的改变。MRI尚可发现无症状病变。结论：MRI不仅有助于MS诊断．尚能动态观察MS病情进展及评估疗效。     

多发性硬化MRI研究新进展

多发性硬化（multiple sclerosis，MS）是中枢神经系统最常见的脱髓鞘疾病，其诊断一直强调病史和体格检查的重要性。但是随着MRI技术的发展与应用．这一情况发生了改变。2000年7月制订的McDonald诊断标准第一次将MRj用于MS的诊断，并明确地说明了MS时间和空间上多发性的MRI证据．从而有利于对患者尤其是表现为临床孤立综合征（clinically isolated syndrome，CIS）的患者进行早期诊断。超微型超顺磁性氧化铁颗粒（ultrasmall superparamagnetic iron oxides，USPIOs）对比剂及MRI新技术在显示MS特异性征象、病变与病理变化及临床功能障碍之间的相关性、定量研究、早期诊断及疗效监测等方面提供了更多特异性信息。本文对此进行综述。     

McDonald多发性硬化诊断标准的应用性评价

多发性硬化（multiple sclerosis，MS）的诊断主要基于中枢神经系统病灶在时间上和空间上多发性的临床证据，且需除外可引起这些损害的其他疾病。目前被广泛应用的诊断标准有两种，即1983年提出的Poser标准和2001年提出的McDonald标准。McDonald标准 ^[1]是建立在Poser标准基础上，但更注意利用包括MRI在内的相关实验室检查来证明多发性硬化在时间和空间上的多发性，     

多发性硬化的磁共振成像研究进展

不断发展的磁共振成像(MRI)新技术,如磁化传递成像(MTI)、扩散加权成像(DWI)、扩散张量成像(DTI)、磁共振波谱(MRS)、MR灌注成像(PWI)、功能磁共振成像(fMRI)等,为多发性硬化(MS)研究提供了新的视角,在MS的早期诊断、鉴别诊断、病程进展监测、疗效评估、病理机制及神经心理变化等方面发挥越来越重要的作用。     

Diagnosis of multiple sclerosis: comparison of the Poser criteria and the new McDonald criteria

OBJECTIVES: A confident and accurate diagnosis of multiple sclerosis (MS) is important, but a specific diagnostic test for the disease does not exist. The traditional diagnostic criteria of Poser et al. were published in 1983, and recently, McDonald et al. recommended new criteria for the diagnosis of MS. PATIENTS AND METHODS: In this study these two diagnostic schemes were compared by prospectively applying both of them to 76 patients with clinical features suggesting a new diagnosis of MS. RESULTS: Using the Poser criteria, 29 patients (38%) were classified as clinically definite and 35 patients (46%) as laboratory definite MS. According to the new McDonald criteria, MS was diagnosed in 39 (52%) patients, 37 patients (48%) had 'possible MS'. All patients with a clinically definite MS with the Poser criteria were also given the diagnosis of MS as recommended by McDonald et al. Of those 35 patients with laboratory definite MS according to Poser et al., four patients could be classified as having MS with the McDonald criteria, 89% of them had 'possible MS'. Conversely, 75% of the 39 patients, who fulfilled the new McDonald criteria for MS were assigned to the category of clinically definite MS according to the Poser criteria, and 83% of the patients with a 'possible MS' using the McDonald criteria, had a laboratory definite MS with the Poser criteria. CONCLUSION: MS according to the McDonald criteria was diagnosed more often than 'clinically definite MS' according to Poser et al., but combining the categories of clinically and laboratory definite MS, the diagnosis of MS could clearly be established more frequently using the Poser criteria.     

多发性硬化患者的脑部磁共振成像表现

目的　寻找多发性硬化(multiplesclerosis,MS)患者有诊断及鉴别诊断意义的脑部磁共振成像(MRI)征象。方法　分析41例临床确诊MS患者的常规脑部MRI表现,内容包括病灶数目、分布、大小、形态、信号特征及增强表现等。结果　脑部的MS灶可以单发和多发,单发者幕下多见,多发者以4~15个病灶者最多。少数病例的病灶弥漫分布,无法计数,呈现“白质变脏征”。斑块分布以两侧脑室旁最多见,其次为额顶叶皮层下、胼胝体、脑干,伴发小脑内病灶仅2例。斑块直径约几个毫米至2cm不等,约占75%; 2cm以上病灶者少见,最大病灶约6~7cm。根据形态和信号,病灶可以分为急性和慢性。急性病灶呈卵圆形或圆形,有明显膨胀感,T1WI呈低或略低信号,周围可见等或略高信号;T2WI呈高信号,但增高程度不同,表现为中央呈“核心”而周围呈“晕环”。此类病灶均表现强化,最典型为环形强化、强化环完整或呈不完整弓形,即使病灶较大仍具有上述特点。慢性病灶也可分为两种,一种为大体对称性的病灶,分布于两侧脑室旁,另一种病灶分布较分散,额、顶叶,侧脑室旁及脑干等处都有,病灶呈小条、片状,部分融合成较大片状。慢性病灶有收缩感,边缘较锐利,信号较均匀,周围无晕环征象,增强后无强化。结论　脑部MS有多种MRI表现,部分征象具有特征性。     

Neuropsychological assessment in multiple sclerosis: a follow-up study with magnetic resonance imaging

Summary Nineteen moderately impaired patients with clinically definite multiple sclerosis and an initially relapsing-remitting course were included in a neuropsychological and magnetic resonance imaging (MRI) follow-up study. The average test/re-test interval was about 2 years. The neuropsychological findings were indicative of a very mild overall impairment; the patients, as a group, showed no evidence of cognitive deterioration in the follow-up period. A numerical estimation of the severity of cerebral demyelination shown by MRI did not indicate a significant change. No correlation between cognitive performance variations and MRI changes was found.     

Facial palsy in multiple sclerosis

Facial palsy occurred in 21 (19.6%) of 107 Japanese patients with multiple sclerosis (MS) during a mean follow-up period of 4.3 years. We observed residual signs of facial palsy in five other patients in whom acute onset was confirmed from medical records. Facial palsy began on average 7.6 years after the onset of MS but in five patients (4.7%) was the first symptom of MS, preceding the next MS symptom by 0.5–3 years. Facial palsy was usually associated with other brainstem signs, while two patients showed only facial palsy 1 and 3 years after the onset of MS. Twenty-one (84.0%) of the 25 patients who underwent brain magnetic resonance imaging (MRI) showed brainstem lesions in the pontine tegmentum ipsilateral to the facial palsy. However, the two patients without other symptoms or signs had no apparent causal lesion on MRI, which suggests difficulty in differentiating idiopathic Bell’s palsy from MS- associated facial palsy by MRI, although it has an excellent capacity to detect causal lesions of facial palsy associated with MS. Received: 6 March 1997 Received in revised form: 25 July 1997 Accepted: 12 August 1997     

多发性硬化患者的磁化传递及弥散张量成像特点

目的比较多发性硬化(MS)患者脑内病灶、表现正常脑白质(NAWM)及健康志愿者脑白质的磁化传递及弥散特性的差异,探讨MS患者的磁化传递率(MTR)与平均弥散率(MD)的相关性。方法分别对24例复发缓解型MS患者和24名健康志愿者行常规MRI、磁化传递成像(MTI)及弥散张量成像(DTI),经处理后得到相应的MTR、MD及各向异性分数(FA)图。测量MS病灶、对侧NAWM及健康志愿者相应脑白质区域的MTR、MD及FA值。结果MS患者病灶的平均MTR值(23.49%±5.16%)明显低于NAWM组(29.49%±3.38%)及健康对照组(32.78%±3.42%,F=101.44,P<0.01);病灶的平均FA值(0.32±0.09)也明显低于NAWM组(0.42±0.09)及对照组(0.51±0.09,F=95.41,P<0.01);而病灶的平均MD值[(1.10±0.17)×10-3mm2/s]明显高于NAWM组[(0.92±0.13)×10-3mm2/s]及对照组[(0.76±0.04)×10-3mm2/s,F=144.89,P<0.01]。与MS患者T1WI等信号病灶相比,低信号病灶的MTR值及FA值降低,而MD值升高。MS患者病灶的MTR值与MD值显著相关,而NAWM的MTR与MD值无明显相关性。结论MTI和DTI的指标可以反映MS患者脑内不同部位病理变化的不同,为常规MRI提供补充信息。     

A comparison of neuropsychological deficits in primary and secondary progressive multiple sclerosis

Neuropsychological deficits and the relationship to brain pathology were examined in 13 primary progressive (PP) and 12 secondary progressive (SP) multiple sclerosis patients with a similar duration of the progressive phase and comparable physical disability. A battery of neuropsychological tests to assess attention, short-term and working memory was administered to the patients, and their performance was compared to that of 20 healthy controls matched for age and premorbid IQ. Total cerebral lesion load on T2-weighted magnetic resonance imaging was measured in the patients. Both PP and SP patients performed significantly worse than controls in most of the neuropsychological tests. There were only subtle differences between SP and PP on the working memory task although magnetic resonance imaging lesion load was significantly higher in SP than in PP patients. In this exploratory study only subtle differences in cognitive impairment were detected between SP and PP patients matched for physical disability and relevant illness features. The results also suggest that the severity of cognitive impairment cannot be fully explained by the extent of abnormalities detected on conventional T2-weighted magnetic resonance images, and that other pathological abnormalities such as in normal-appearing white matter are likely to be involved. Received: 2 November 1998/Received in revised form: 4 August 1999/Accepted: 6 October 1999     

Randomized placebo-controlled trial of mitoxantrone in relapsing-remitting multiple sclerosis: 24-month clinical and MRI outcome

We designed a randomized, placebo-controlled, multicentre trial involving 51 relapsing-remitting multiple sclerosis patients to determine the clinical efficacy of mitoxantrone treatment over 2 years. Patients were allocated either to the mitoxantrone group (27 patients receiving IV infusion of mitoxantrone every month for 1 year at the dosage of 8 mg/m²) or to the placebo group (24 patients, receiving IV infusion of saline every month for 1 year) using a centralized randomization system. Disability at entry and at 12–24 months was evaluated by four blinded neurologists trained in the application of the Kurtzke Expanded Disability Scale (EDSS). In addition, the number and clinical characteristics of the exacerbations over the 24 months were recorded by the local investigators. MRI, at 0,12 and 24 months, was performed with a 0.2 T permanent unit. MRI data were analysed by two blinded neuroradiologists. All patients underwent a clinical evaluation. A statistically significant difference in the mean number of exacerbations was observed between the mitoxantrone group and placebo group both during the 1st and the 2nd year. Although there was no statistically significant benefit in terms of mean EDSS progression over 2 years, the proportion of patients with confirmed progression of the disease, as measured by a one point increase on the EDSS scale, was significantly reduced at the 2nd year evaluation in the mitoxantrone group. Forty-two (23 mitoxantrone, 19 placebo) patients underwent all MRI examinations during the 24-month period. We observed a trend towards a reduction in the number of new lesions on T2-weighted images in the mitoxantrone group. Our study suggests that mitoxantrone might be effective in reducing disease activity, both by decreasing the mean number of exacerbations and by slowing the clinical progression sustained by most patients after 1 year from the end of treatment. Received: 22 February 1996 Received in revised form: 20 August 1996 Accepted: 20 September 1996