诊断多发性硬化的Poser标准与McDonald新标准的比较

目的 比较诊断多发件硬化(multiple sclerosis,MS)的Poser标准和McDonald新标准.方法 将Poser标准和McDonald新标准回顾性应用于临床表现提示为MS的67例患者,采用Fisher精确枪验对两种诊断标准进行比较分析.结果 符合Poser临床和实验室确诊者分别为34例和24例,可能MS者9例,符合McDonald标准的MS确诊者36例,可能MS者31例,两种标准的诊断阳性率差异有统计学意义(OR=5.549,95%CI 2.37～13.00,P<0.01).结论 两种标准住诊断MS,尤其在确诊MS时有明显差异,这可能主要与Poser标准更多地依赖各种亚临床证据,而McDonald标准采用了更为严格的MRI规定有关,脑脊液分析可能在一定程度上有助于提高MS的确诊率和MRI异常的病理特异性.     

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，     

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.     

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。     

诊断为脊髓型多发性硬化的脊髓疾病附68例临床分析

目的 应用新的多发性硬化和视神经脊髓炎诊断标准,回顾分析以往被诊断为“脊髓型多发性硬化”的病例,探讨与主要累及脊髓的脱髓鞘疾病相鉴别的重要疾病类型.方法 应用2010年新修订的McDonald多发性硬化诊断标准,以及2006年Wingerchuk视神经脊髓炎的诊断标准,回顾分析我院1994年～2012年之间曾被诊断为“脊髓型多发性硬化”的患者68例,并进行随访.结果 仅17.65％的患者完全符合McDonald标准,多数脊髓型多发性硬化患者最终转变为视神经脊髓炎,其它疾病如脊髓血管病和系统性自身免疫病也易被误诊为脊髓型多发性硬化.结论 对于孤立的脊髓综合征应该按照一定的诊疗规范进行诊断、鉴别和随访,不建议再使用脊髓型多发性硬化的名称.     

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。     

多发性硬化诊断标准的进展

2005年新修订的McDonald诊断标准在多发性硬化（MS）诊断方面强调了MRI检查T2相病变能更早地提示病变在时间上的多发性，在距第1次发病至少30d后进行MRI扫描所发现的新T2相病变均可用于影像学标准说明病变在时间上的多发性；将脊髓病变整合进影像诊断要求中，对MS鉴别诊断具有极大的实用性。新研究结果显示，在缺乏阳性脑脊液检查结果时仍然可对原发进展型MS（PPMS）做出可靠诊断（至少要有典型头颅MRI改变出现），2005年新修订标准基于此研究结果简化了PPMS诊断标准，并对MRI所见的脑部和脊髓病理变化进行了更为详细的说明。     

脊髓型多发性硬化一附11例临床分析

报告11例脊髓型多发性硬化（MS），占同期多发性硬化住院病人18.6％。其临床表现为脊髓损害的症状和体征。病程中有缓解与复发，或呈慢性进展。颈段脊髓2例，胸段脊髓9例，均作了磁共振扫描（MRI），其特点是：脊髓内症灶的间断分布与其长轴走行一致的椭圆形或条索状病灶，病灶呈偏心分布。本资料及文献报道有一些MS患者以脊髓受损为首发部位，并在较长时间内表现为脊髓损损的临床病征，MRI的使用对早期脊髓型MS诊断有很大价值，但脊髓型MS是独立疾病，还是MS病程中脊脶受损的表现之一，在研究中应引起重视。     

放射学孤立综合征的研究进展

放射学孤立综合征(RIS)是近年来神经免疫学领域的研究热点, 国内文献目前对该疾病的研究报道和综述较少。随着影像学技术的发展和2017年多发性硬化(MS)改良McDonald诊断标准的提出, RIS的诊断标准、临床研究、功能影像学研究及诊疗策略均有新的进展。本综述通过对上述内容进行梳理和总结, 旨在提高国内同行对RIS的认识。     

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。     

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.     

Evaluation of multiple sclerosis diagnostic criteria in Suzhou, China – risk of under-diagnosis in a low prevalence area

Objective –  To evaluate the discharge diagnosis of demyelinating diseases in the central nervous system (CNS) and analyze the predictive value of the new diagnostic criteria in Suzhou, China.
Materials and methods –  We collected clinical information and data of laboratory examinations for all cases with a diagnosis of various demyelinating diseases in the CNS. All data were reviewed individually by four senior neurologists, and a diagnosis was finally given to each patient according to the McDonald criteria and the Poser criteria for multiple sclerosis (MS).
Results –  In the analysis, 176 patients with a diagnosis of demyelinating diseases in the CNS at discharge were included. In 82 patients with a diagnosis of MS at discharge, the MS diagnosis was confirmed for 74 patients according to the McDonald criteria for MS, and the positive predictive value for the discharge diagnosis of MS was 90.2% (74/82). According to the Poser criteria, 61 patients were diagnosed as MS. The consistency of the two diagnostic criteria for MS was 78.4%, based on the results of the evaluation.
Conclusions –  Under-diagnosis of MS could be one of the explanations for the low prevalence of MS in China. Compared to the Poser criteria, the McDonald criteria had a higher sensitivity for the diagnosis of MS.     

Review of the diagnosis and clinical features of multiple sclerosis in China

This review focused on the diagnosis and clinical features of multiple sclerosis (MS) in China. We have identified the published researching information from 1976 to 2008 in China. The key issues related to the diagnosis and clinical features of MS in China were summarized. The first patient with MS in China was reported in 1926 from Xiehe hospital. Case reports on MS has been increasing during recent decades. Almost all the patients with MS were confirmed by the McDonald criteria (1977) before 1984. After the year of 1992, even to this day, the Poser criteria were widely used in China. Although the new diagnostic criteria, McDonald criteria (2001), were presented in 2001, only few papers published in Chinese were reported. The most frequent initial symptoms or signs of the patients with MS were optic nerve, motor weakness and sensory symptoms. The most frequent location of MS lesions over the course was found to be the spinal cord, followed by the cerebrum and optic nerves. Almost all patients had been treated with corticosteroids. This review supported previous observations in Chinese patients with MS. However, further studies are needed to understand epidemiologic features of MS in China.     

Diagnosis of multiple sclerosis

Multiple sclerosis (MS) is an inflammatory demyelinating autoimmune disease of the central nervous system. The disorder displays marked clinical heterogeneity. In certain cases, making diagnosis can be challenging. Diagnosis of MS has become more important in the era of treatments that change the natural history of the disease. Several general diagnostic principles are useful to guide the diagnostic approach to MS. Clinically, MS requires neurological problems associated with objective abnormalities. Certain basic principles, first outlined by Schumacher et al. (1965) are still pertinent. Poser et al. (1983) have further modified the criteria using data derived from clinical evaluation and laboratory studies, including cerebrospinal fluid analysis, evoked potentials, and imaging studies. Poser criteria have long been familial for most neurologists. The most recent addition to our diagnostic armamentarium are the McDonald criteria (2001), which are the first attempt to incorporate standardized MRI criteria into the MS diagnostic process. The most innovative use of MRI to support an MS diagnosis is dissemination of demyelination can be demonstrated by MRI alone, in the absence of any new clinical attacks. Diagnosing MS by such sensitive MRI criteria will occur more quickly than waiting for a second clinical event. This has added some sensitivity, some controversy, and a lot of confusion. The application of the new criteria on Asian MS patients remains to be validated. Each of the criteria will be discussed, with major emphasis on the McDonald criteria.     

Application of the new McDonald criteria to patients with clinically isolated syndromes suggestive of multiple sclerosis

Traditionally, multiple sclerosis (MS) has been diagnosed on the basis of clinical evidence of dissemination in time and space. Previously, it could not be diagnosed in patients with single clinical episodes of demyelination known as clinically isolated syndromes. New diagnostic criteria from the International Panel of McDonald and colleagues incorporate MRI evidence of dissemination in time and space to allow a diagnosis of MS in patients with clinically isolated syndromes. From clinical and MRI examinations performed prospectively at baseline, 3 months, 1 year, and 3 years of follow-up, the frequency of developing MS was ascertained by the application of both the new McDonald criteria and the Poser criteria for clinically definite MS. The specificity, sensitivity, positive and negative predictive value, and accuracy of the new criteria for the development of clinically definite MS were assessed. At 3 months, 20 of 95 (21%) patients had MS with the McDonald criteria, whereas only 7 of 95 (7%) had developed clinically definite MS. After 1 year, the corresponding figures were 38 of 79 (48%) and 16 of 79 (20%), and after 3 years, they were 29 of 50 (58%) and 19 of 50 (38%). The development of MS with the new MRI criteria after 1 year had a high sensitivity (83%), specificity (83%), positive predicative value (75%), negative predictive value (89%), and accuracy (83%) for clinically definite MS at 3 years. Use of the new McDonald criteria more than doubled the rate of diagnosis of MS within a year of presentation with a clinically isolated syndrome. The high specificity, positive predictive value, and accuracy of the new criteria for clinically definite MS support their clinical relevance.     

Validation of the NARCOMS registry: diagnosis

The North American Research Committee on Multiple Sclerosis (NARCOMS) Registry is a patient registry, wherein the diagnoses of multiple sclerosis (MS) are unverified. We compared self-reported diagnoses of registry participants to physician-reported diagnoses, and with diagnoses based on medical records review. Registry participants with more than one of the following: age of onset <10 or >50 years, no bladder symptoms or fatigue, or zero or more than four relapses in the last year, were considered atypical. All others were considered typical. We sent letters to participants describing the study, surveyed treating physicians regarding the participants' diagnosis, and reviewed medical records. Diagnosis was classified by the McDonald and Poser criteria. Of the 240 participants sampled, 109 were in active registry status with accurate contact information. Of these, 52 consented, 29 refused and 28 did not respond (weighted response rate 76.3+/-4.5%). Some 37 of 38 physician surveys confirmed the diagnosis of MS (98.8+/-1.2%). After reviewing 41 medical records, we classified 53.2+/-8.9% of participants as definite MS, 16.9+/-6.8% as possible MS, while the remainder had insufficient data for diagnostic confirmation. We confirmed a diagnosis of MS in 98.7+/-1.3% of participants based on records review, physician survey or telephone interview, supporting the validity of the diagnoses reported by NARCOMS participants.     

The reliability of specific primary progressive MS criteria in an ethnically diverse population

Three different diagnostic criteria for primary progressive MS were recently proposed for Caucasian population of Western European region. OBJECTIVE: The objective of the study was to apply these criteria to a series of Brazilian patients with high ethnic diversity background to evaluate reproducibility and reliability. METHODS: 52 patients classified as form of the disease that is progressive from onset and followed between 2000 and 2006 were included. Thompson, McDonald and Polman criteria were applied based in clinical date and complementary exams. Results: 72% fulfilled all three criteria with moderate agreement (p<0.001). Ten patients fulfilled at least one criterion and four failed to fulfill any of the three criteria. Strong agreement was found between Thompson and McDonald criteria (p<0.001), agreement was moderate between Thompson and Polman criteria (p<0.001) and weak agreement occurred between McDonald and Polman criteria (p=0.042). CONCLUSION: The main difference between these criteria is the change in the role of CSF, previously a prerequisite for diagnosis. Rigid diagnostic criteria as Thompson have higher specificity, should be used in clinical research protocols, while more flexible criteria as Polman facilitate the diagnosis of PPMS in neurological practice, particularly in initial stages of the disease, because of their potentially higher sensitivity.     

Revision of McDonald's new diagnostic criteria for multiple sclerosis

In 2001, an international panel suggested new diagnostic criteria for multiple sclerosis (MS). These criteria integrate clinical, imaging (MRI), and paraclinical results in order to facilitate diagnosis. Since then, these so-called McDonald criteria have been broadly accepted and widely propagated. In the meantime a number of publications have dealt with the sensitivity and specificity for MS diagnosis and with implementing these new criteria in clinical practice. Based on these empirical values and newer data on MS, an international expert group recently proposed a revision of the criteria. Substantial changes affect (1) MRI criteria for the dissemination of lesions over time, (2) the role of spinal cord lesions in the MRI and (3) diagnosis of primary progressive MS. In this article we present recent experiences with the McDonald and revised criteria.