多发性硬化226例临床分析

目的　总结国人多发性硬化 ( MS)的临床、影像及病理学特点。方法　从临床、影像学及组织病理学等多方面对 MS病例进行回顾性分析。结果　 2 2 6例 MS病例中 ,临床确诊 1 96例 ,实验室支持确诊 1 6例 ,临床或实验室可能 1 4例 ;男性 85例 ,女性 1 41例 ;发病年龄 4岁至 67岁 ,平均 3 2 .46岁 ,<1 5岁的早发型病例占 9.3 % ;3 0 .1 %病例病前有诱发因素 ,其中 77.9%与感染有关 ;首发症状以感觉异常、肢体无力及视力减退最为多见 ;88.1 %病例呈急性或亚急性起病 ,复发 -缓解型占 49.1 % ;病变部位中脊髓 1 5 1例 ,脑干 74例 ,小脑 2 4例 ,大脑半球 95例 ,视神经 98例 ;66.8%病例出现脊髓受累 ,5 0 .4%病例曾出现过尿便障碍 ,其中视神经脊髓炎占2 9.2 %。结论　国内 MS的发病年龄较西方国家早 ,起病急 ,病程短 ,视神经及脊髓易受侵犯 ,小脑、脑干较少受累 ,临床表现中以视神经受损及尿便障碍较为突出 ,致残率较高。诱发电位及 MRI检查有助于发现亚临床病变。急性、亚急性期应用激素疗效显著。     

东北地区204例多发性硬化患者临床特征分析

目的 总结东北地区多发性硬化(MS)的临床特点.方法 对204例MS患者的临床表现、病变部位、影像学特点、实验室检查及激素治疗进行分析总结.结果 (1)性别:男69例(33.82％),女135例(66.18％)；发病年龄:20 ～40岁95例(46.57％).(2)起病形式:急性或亚急性为主(65.69％),25例患者(12.25％)首次发病前存在明显诱因.(3)常见症状:肢体无力为主(70.59％),尿便障碍56例(27.45％)；以复发-缓解型多见.(4)脑脊液IgG增高占60.19％；脑干视、听觉诱发电位阳性率为69.77％；体感诱发电位阳性率为2.94％.(5)病灶部位:脑部异常73例(35.78％),脊髓异常53例(25.98％),脑与脊髓均有病灶78例(38.24％).(6)均无MS家族史,自身免疫性疾病史者8例,3例为产后发病.(7)150例患者接受激素治疗,并采用扩展残疾状态量表(Expanded disability status scale,EDSS)评价其疗效,有效率为78.67％.结论 我国北方地区MS患者仍以女性为多；发病年龄以20～40岁为主；临床特点表现为发病较晚、起病急,以复发-缓解型为主；大多数患者发病前无明显诱因；神经系统症状以肢体运动及感觉障碍为主,尿便障碍较常见；本组患者颅内病灶主要见于皮质下白质、侧脑室旁及半卵圆中心,小脑受累较少；脊髓病灶主要以颈段受累为主；激素冲击治疗对本组患者有较好疗效.     

多发性硬化413例患者的临床表现特点

目的 总结多发性硬化(MS)患者的临床特点。方法 用临床病例分析统计方法，对413例MS患者的发病规律和临床特点进行归纳、分析。结果 MS好发于青壮年，以急性和亚急性起病为主；首发症状以视力障碍(128例，31．O％)最常见；肢体无力(325例)、感觉障碍(246例)、视力障碍(243例)是MS患者最常见的症状，Lhermitte征较多见；发作性症状多见，以痛性痉挛发作和癫疴发作常见；可合并周围神经系统损害；临床定位以脊髓(256例)和视神经(244例)受累最多见。结论本组MS临床特点不同于西方人。     

多发性硬化38例临床分析

目的分析38例多发性硬化(MS)患者临床特点,以提高对MS认识.方法回顾性分析38例患者的发病年龄、起病形式、首发症状、临床症状、体征以及诱发电位、核磁共振检查结果及治疗用药情况,并与相关文献对照.结果患者中男女比例1:1.1,有复发-缓解病史者占42.1%;初发年龄16～49岁者占68.4%;无明显诱因者占52.6%,急性、亚急性发病者占76.3%;首发症状中以单发症状起病者占60.5%,临床症状及体征中感觉异常占65.8%、肢体无力占60.5%、视力下降占34%、大小便异常占26.3%,脑干和小脑症状占15.8%;核磁共振、诱发电位异常率为80.0%和86.4%;急性期应用激素治疗好转率达91 9%.结论MS临床表现具有特征性,MRI、诱发电位对亚临床MS的诊断具有重要意义;早期应用激素治疗效果显著.     

多发性硬化的临床特点分析

目的探讨多发性硬化(MS)的临床表现特点。方法回顾性分析2007-01—2010-06于我院确诊的68例多发性硬化病例,总结其一般资料、临床表现、病变部位、重要辅助检查及治疗转归等。结果 68例患者中,首发症状以肢体无力(28例,41.2%)最常见;肢体无力、感觉障碍、视觉损害是MS患者最常见的症状;MS好发于青壮年,以急性和亚急性起病为主;视、听、体感诱发电位(VEP、BAEP、SEP)有助于发现亚临床病变;脑脊液(CSF)检查可有异常;磁共振(MRI)检查阳性率高;临床定位以大脑半球、脊髓和视神经受累最多见。糖皮质激素及免疫球蛋白治疗有效。结论根据临床特点,结合神经电生理脑脊液及影像学等检查能大大提高MS的临床确诊率。     

多发性硬化62例分析

随着电生理、影像检查技术进步,CT及MRI在临床上广泛应用,多发性硬化(multiple sclerosis,MS)临床诊断率得到了提高,现将我们收集资料完整的MS患者62例分析如下.1临床资料1.1 一般资料 62例MS患者中,男26例,女36例;年龄6～10岁2例,＞10～20岁2例,＞20～25岁6例,＞25～30岁18例,＞30～40岁24例,＞40～50岁6例,＞50岁4例.首次发病8例,2次以上54例,最多复发5次;复发时间最短2个月,最长22 a,2 a内复发35例(56.3％).急性起病16例,亚急性起病42例,慢性起病4例.临床诊断52例,实验室支持10例.     

多发性硬化118例临床分析

目的探讨多发性硬化(MS)的临床特征及MRI、视觉诱发电位(VEP)、脑干听觉诱发电位(BAEP)、脑脊液(CSF)检查在诊断中的价值。方法从流行病学、首发症状、起病形式、病程、累积部位、各项辅助检查情况等方面对作者医院1998—2004年收住的118例MS患者进行回顾性分析。结果118例MS患者中,临床确诊68例,实验室支持确诊21例,临床可能29例;男、女比为1.46∶1;平均首发年龄:男(31.1±13.5)岁,女(34.9±10.5)岁;起病形式:急性37.0%,亚急性40.0%,慢性23.0%;病程:复发-缓解型占67.0%;首发症状:肢体无力58例,肢体麻木34例,视力障碍29例。累及部位:视神经80例,脊髓72例,脑干61例,大脑半球50例,小脑26例。MRI:行MRI检查82例,阳性67例,其中累及脊髓32例,脑室旁34例(占MRI检查阳性者的50.7%)。VEP:异常但无症状体征者占47.0%;BAEP:异常但无症状体征者占81.5%。CSF:24 h鞘内合成率阳性占81.6%,寡克隆区带阳性占23.0%。结论MS多无明显诱因,多以急性或亚急性起病,复发率高,首发症状以肢体无力多见,脊髓、视神经易受累,MRI检查阳性者中半数左右大脑白质有异常信号,VEP、BAEP检查可发现许多视神经及脑干亚临床病灶,CSF 24 h鞘内合成率阳性者较多,可提示病变的炎性本质。     

肾上腺脑白质营养不良的临床特点

目的探讨肾上腺脑白质营养不良(ALD)的临床特点。方法回顾性分析14例ALD患者的临床资料。结果本组14例ALD患者均为男童,起病年龄1～13岁,缓慢起病,临床上均有不同程度的智力障碍和肢体活动障碍,其中9例视力下降,6例听力下降,13例言语不清,5例有抽搐发作,6例皮肤色素沉着;5例血浆极长链脂肪酸(VLCFA)不同程度增高,2例头颅CT、12例头颅MRI显示双侧脑室三角区周围白质对称分布的蝶形病灶,3例增强扫描示部分病灶周围花边样强化条带。结论ALD临床特点为进行性智力及肢体运动障碍、视力及听力下降、肾上腺皮质功能减低、血浆VLCFA水平增高及特征性的头颅影像学改变。     

27例成人烟雾病的临床特点分析

目的探讨成人烟雾病患者的临床特点,以提高对烟雾病的认识。方法回顾徐州医学院附属医院神经内科2010年1月～2011年12月收治的27例初次诊断烟雾病住院患者的临床资料,分析烟雾病的发病年龄、性别和伴发疾病的特点。结果本组患者发病平均年龄39．2岁,其中男性患者为33．6岁,女性为41．5岁,不同性别患者组发病年龄比较差异无显著性;不同性别患者间临床表现分布无显著性差异;男女患者比例1：2．38,发病频数分布拟合优度检验差异具有显著性（x2=4．481,P=0．034）;患者起病形式为缺血性卒中表现者占59．25％;本组中3例累及椎基底动脉系统,占11．11％;40．74％患者合并高血压。结论本组成人烟雾病患者中女性患者比例高于男性,多数患者临床表现为缺血性脑卒中。该病受累血管可以合并椎一基底动脉系统。     

无肢体瘫痪性脑出血86例分析

脑出血患者无肢体瘫痪时易误诊、漏诊。我院自 1995 0 8～2 0 0 1 12共收治脑出血患者 60 9例 ,其中无肢体瘫痪者 86例 ,占同期脑出血患者的 14 1%。为提高对无肢体瘫痪性脑出血的认识 ,就本病一些特点加以讨论。1　临床资料1 1　一般资料　男 47例 ,女 3 9例。年龄 2 1～ 83岁 ,平均 5 5 2岁 ,其中 >5 5岁者 5 9例。既往有高血压病病史者 68例 (79% ) ,有脑卒中病史者 11例 (12 8% ) ,有糖尿病史者 8例 (9 3 % )。起病至入院时间 :6ｈ内 12例 ,6～ 2 4ｈ 2 0例 ,2 4～ 72ｈ 3 7例 ,超过72ｈ 17例。1 2　临床表现　本组病例均呈急性起病…     

Clinical features of Japanese pediatric patients with anti-aquaporin 4 antibody

Recently, a disease-specific antibody was found in serum from patients with neuromyelitis optica (NMO), and its target antigen was identified as aquaporin 4 (AQP4) water channel protein. There is no clinical picture of pediatric cases with anti-AQP4 antibody, except one report from North America. Here, we report the clinical features of 18 Japanese anti-AQP4-antibody-positive patients with childhood-onset of NMO. Of the 2000 patients who had been examined for anti-AQP4 antibody at Tohoku University Hospital up until 2008, 60 were under 15 years of age at onset, and 18 of them were positive for anti-AQP4 antibody. We analyzed the clinical information on those patients. There were 14 girls and 4 boys (M:F ratio = 1:3.5). The age of onset ranged from 3 to 15 years old (median 13). The clinical diagnoses of the 18 patients before the anti-AQP4 antibody tests were: NMO in 8 (44%), MS in 7 (39%), and opticospinal MS (OSMS) in 3 (17%). Nine patients developed only optic neuritis at onset. At the last follow-up, brain magnetic resonance imaging (MRI) was abnormal in 14 patients, nine had monocular or binocular blindness, and the expanded disability status scale score was 6.0 or higher (cannot walk without support) in eight patients. NMO has a poor prognosis, as seen in adult cases, suggesting the importance of an early diagnosis using the anti-AQP4 antibody test to institute effective immunosuppressive treatment.     

9例原发性中枢神经系统血管炎临床、影像及病理特点研究

目的  总结原发性中枢神经系统血管炎临床、磁共振成像（magnetic resonance imaging,MRI）和病理学特点。 方法  收集2012年3月～2014年12月首都医科大学附属北京天坛医院神经病学中心收治的原发性中枢神经系统血管炎患者资料,采用描述性方法对其临床表现、MRI及病理学特点进行分析。 结果  共收集9例患者,其中男性5例（55.56%）,女性4例（44.44%）,年龄范围10～47岁,中位年龄30岁。痫性发作4例（44.44%）,行为认知异常3例（33.33%）,局灶性感觉运动异常5例（55.56%）,头晕2例（22.22%）,钝性头痛2例（22.22%）,面部疼痛1例（11.11%）,视物模糊1例（11.11%）,行走不稳1例（11.11%）。腰穿异常4例（44.44%）。MRI表现为双侧病灶6例（66.67%）,单侧病灶3例（33.33%）,其中受累部位分别为额叶9例（100%）,顶叶5例（55.56%）,颞枕叶4例（44.44%）,合并皮层下白质受累6例（66.67%）,合并脑膜/脊膜受累3例（33.33%）,合并基底节受累1例（11.11%）,合并脊髓受累1例（11.11%）;病变边界不清8例（88.89%）,边界清1例（11.11%）;皮层萎缩伴脑室扩大3例（33.33%）;病灶及脑脊膜强化6例（66.67%）;7例患者行磁共振T2*或磁敏感加权序列（susceptibility weighted  imaging,SWI）,其中病灶表现低信号4例（57.14%）。2例行脑病理学检查,表现为脑实质水肿,血管周围炎性细胞浸润,血管壁坏死和胶质增生。 结论  原发性中枢神经系统血管炎临床表现及影像学多样,病灶合并脑膜和（或）脊膜强化,以及磁共振T2*或SWI序列低信号是其重要影像学特征,脑活检仍是目前重要的确诊手段。     

Acute transverse myelitis with normal brain MRI

Objective To investigate the long-term risk of developing MS in patients presenting with acute transverse myelitis (ATM) and normal brain MRI scans at onset. Methods We studied 58 ATM patients with normal brain MRI at presentation for up to 5 years with serial neurologic and imaging studies. All patients underwent CSF analysis at onset which was defined positive if two or more IgG oligoclonal bands and/or elevated IgG index were present. Brain and spinal cord MRI scans were obtained every 6 months for the first 2 years, and annually thereafter unless the patient experienced a second neurologic attack different from the initial episode to confirm CDMS or there was demonstration of MRI lesions confirming dissemination in time and space to fulfill McDonald imaging criteria to diagnose MS. Results Seventeen of 58 (29%) patients developed MS of which 7 (41%) patients developed CDMS and 10 (59%) developed MS using McDonald Imaging Criteria. Mean time to CDMS by a second clinical attack was 11. 1 months compared to 19. 2 months by MRI lesions (P = 0. 03). None of the patients developed MS after 24 months of onset. All 17 patients who developed MS had positive CSF although 15 patients who had positive CSF did not develop MS during the 5 years of follow-up. Conclusions The majority of patients with ATM and normal brain MRI do not develop MS after 5 years of follow-up confirming the relatively low risk compared to patients with abnormal brain MRI scans. CSF is helpful in distinguishing patients more likely to develop MS. Compared to clinical attacks, serial imaging may not lead to an earlier diagnosis in ATM patients with normal brain MRI.     

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.     

Late onset multiple sclerosis

Multiple sclerosis (MS) with clinical onset after 50 years old is unusual and frequently misdiagnosed. Clinical presentation and course seem also to be different that in MS occurring between 20 and 50 years old. The aim of this study was to evaluate the clinical and paraclinical characteristics of late onset MS. We respectively studied MS patients older than 50 years at the onset of the disease. We evaluated demographical data, clinical symptoms, cerebrospinal fluid (CSF), visual evoked potentials (VEPs) and MRI of these patients. We also studied clinical data during the follow-up with the occurrence of new symptoms and the evolution of the disease by the index of progression (EDSS unit per year). In a population of 1 417 MS, 3.4 p.cent had their first symptoms at 50 years old or older and 0.45 p.cent after 59 years old. At the time of the study patients had more frequently a progressive form: 37 p.cent had a primary progressive form and 35 p.cent a secondary progressive MS. None of the patients with onset after 60 years old had relapsing remittent MS. Motor symptoms were the most common neurologic presentation (54 p.cent of patients). Very few patients had a clinical optic nerve involvement during the follow-up. The mean progression index was 1 suggesting a most severe evolution in this subgroup of MS patients. 76 p.cent of patients had oligoclonal banding detected by CSF electrophoresis. The VEPs were abnormal in 81 p. cent of patients tested. 71 p.cent of the brain MRI were consistent with the diagnosis of MS. 60 p.cent of patients had spinal cord MRI abnormal. This study highlights the differences between the late onset MS and earlier onset. As previously reported, our study underlines the high frequency of progressive course, motor function involvement and poor prognosis.     

同心圆硬化12例临床及影像学分析

目的 探讨同心圆硬化的临床表现、影像学特征及其诊断价值.方法 回顾12例同心圆硬化患者(门诊2例,住院10例)的临床表现、实验室检查结果、影像学特点及病理结果,并对患者进行随访.结果 12例患者(男4例,女8例)发病年龄8～54岁(平均36.8岁),多以淡漠少语、反应迟钝起病(7例),病程中其他临床表现以智能减退(10例)、肢体活动障碍(8例)、大小便失禁(7例)、肌力减退(7例)、锥体束征阳性(6例)较多见.8例患者行脑脊液髓鞘碱性蛋白检查,其中7例增高.6例行脑活体组织检查,均可见分层脱髓鞘改变,有炎性细胞浸润.病灶数量1例为单发,11例为多发.4例患者行头颅CT检查,病灶均呈低密度影,增强后无强化.MRI检查均可见圆或类圆形影像,增强扫描8例可见环状或半环状强化,易被误诊为脑肿瘤;3例呈同心圆样强化.结论 同心圆硬化常亚急性或慢性起病,首发症状多为认知功能障碍,易被误诊,头颅MRI有特征表现,有诊断价值,激素治疗有效,预后良好,呈良性临床过程.

Abstract：

Objective To explore the clinical features, imaging and their diagnosis vaule of Baló's concentric sclerosis (BCS). Methods The clinical manifestation, laboratory examination, neuroimaging characteristics, pathology and follow-up of 12 (2 outpatients and 10 inpatients) cases of BCS were analyzed retrospectively. Results The onset age of 12 patients (male 4, female 8) ranged from 8 to 54 years old (mean 36.8 years). Unconcerned and slowness response were common in the disease onset (7 cases).Other symptoms and signs such as hypophrenia ( 10 cases), limitation of limb or hand movement (8 cases),urinary and fecal incontinence (7 cases), hypo-myodynamia (7 cases), positive pyramidal sign (6 cases)were not rare in course of disease. Eight cases underwent the examination of cerebrospinal fluid myelin basic protein, which increased in 7 cases. Demyelinating change and perivascular inflammatory infiltration were shown in 6 brain biopsies. The involvements of multi-lesions were found in 11 cases. Four patients underwent CT scanning and all the cases showed with hypodense lesions and couldn' t be enhanced. On MRI, all cases appeared round or round-like in shape. Enhanced lesions showed ring-like or half-ting-like shape (8 cases) and concentric ring (3 cases). It's easy to be misdiagnosed as brain tumor (8 cases).Conclusions BCS is a kind of demyelinating disease of subacute or chronic onset accompanied with cognitive disorder being the most common initiating symptoms. It's prone to be misdiagnosed. Diagnosis depends on MRI and corticosteroid treatment is effective. BCS has a good prognosis and presents with benign clinical course.     

线粒体脑肌病伴乳酸血症和卒中样发作综合征的临床、EEG、影像学及肌肉病理特点分析

目的 探讨线粒体脑肌病伴乳酸血症和卒中样发作(MELAS)综合征的临床症状、EEG、影像学及肌肉病理特点,以提高对其认识及诊断的正确率.方法 回顾性分析6例基因确诊的MELAS综合征患者的临床资料,分析其临床表现、EEG、影像学、肌肉病理特点.结果 6例患者中男性4例,女性2例,年龄最大者43岁,最小者4岁,平均起病年...     

Multiple sclerosis: report on 200 cases from Iran

Clinical findings of 200 patients in Iran with definite multiple sclerosis (MS) according to Poser et al.'s criteria and positive findings on magnetic resonance imaging (MRI) have been reviewed. The clinical course was relapsing-remitting (RR) for 88%, primary progressive (PP) for 7% and secondary progressive (SP) for 5% of cases. The mean age of onset was 27 +/- 7.4 years for the whole group and 37.1 +/- 8.8 years for PPMS. The gender ratio was 2.5:1 female:male. Involvement of the pyramidal system was the most common mode of presentation. Five per cent of patients had positive family history for the disease, 14% of patients had benign MS and 12% with disease duration longer than five years had an Expanded Disability Status Scale < or = 2. The optico-spinal form was not a common form of presentation in the group.     

Clinical characteristics, course and prognosis of relapsing Devic’s Neuromyelitis Optica

Abstract.Objectives: To evaluate the clinical characteristics, course and prognosis of Devics neuromyelitis optica (DNO), to evaluate the prognostic role of demographic and clinical features, to evaluate the current DNO diagnostic criteria.Methods: Demographic, clinical, CSF and MRI data of patients affected by DNO were collected from fifteen Italian MS centres. Inclusion criteria were: 1) two or more acute episodes of neurological dysfunction indicating involvement of the optic nerve and spinal cord, in a simultaneous or subsequent temporal relationship; 2) no evidence of lesions beyond the optic nerve or the spinal cord; 3) brain MRI at onset negative or non-specific for multiple sclerosis (MS) (white matter lesions 2). Disability was scored by means of Kurtzkes Expanded Disability Status Scale (EDSS).Results: 46 patients with relapsing DNO were included, 37 females and 9 males, with mean age at onset of 40.1 ± 16.3 years (range 12–77 years). The follow up duration was 8.8 ± 3.5 years, the mean annualised relapse rate was 1.3 ± 1.2. After 5, 10 and 15 years EDSS 3.0 was reached respectively by 65%, 82 % and 86% of cases, EDSS 6.0 respectively by 42%, 53 % and 69% of cases, EDSS 10 respectively by 8%, 12% and 23% of cases. The probability of reaching EDSS 3 was statistically correlated with age at onset, interval between the first and 2^nd attack, and relapse rate. The probability of reaching EDSS 6.0 was correlated with the residual EDSS at onset and to relapse rate.During the follow up, brain white matter lesions appeared in 8 subjects. Spinal cord MRI showed lesions extending across 3 or more segments in 39 subjects, only 1 lesion involving 1 segment in 4 subjects, and was normal in 3 subjects. One or more CSF abnormalities were found at least once in 29/44 patients (65.9 %), the most frequent findings being pleocytosis (38.6 %), oligoclonal bands (34.1 %), high protein level (25 %), and high albumin ratio (20.5 %).Conclusions: DNO has a poor prognosis in most cases. Compared with MS, DNO patients have a higher age at onset, females are more frequently affected, the course is more severe. Brain and spinal cord MRI permit the differentiation of DNO from MS. CSF supports the probability of DNO if it shows increased cells and proteins.* participants in the Italian Devics Study Group are listed in the Appendix.