我国北方地区多发性硬化164例的临床特点

目的通过分析总结我国北方地区164例多发性硬化(MS)的临床资料,探寻MS的临床特点。方法从临床表现和影像学等多方面对164例MS病例进行回顾性分析。结果164例MS病例中,男48例,女116例;发病年龄4．5个月-66岁,平均29．2s岁;<15岁的早发型占9．8％,发病高峰在20-40岁;39％的患者有明确诱因;主要以急性或亚急性起病(73．1％);首发症状以肢体无力(36．6％)、肢体感觉障碍(25．6％)和视力减退(22．6％)最常见;复发缓解型占58．5％。本组头部MRI阳性率为93．5％,脊髓MRI阳性率为87．5％,以半卵圆中心、脑室周围、基底节和脑干等处多见。结论本组MS患者的临床特点表现为发病早,起病急,病程短,视神经易受侵犯,小脑较少受累。MRI对于发现MS病灶有很高的敏感性。     

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。     

65例多发性硬化患者的临床分析

目的总结多发性硬化(MS)患者的临床特点(精神情感障碍)以及脑脊液、诱发电位、影像学改变进行分析。方法回顾分析65例MS患者的有关临床和实验室资料。结果65例中,男17例,女48例;年龄16~68岁,平均(36.5±14.1)岁,男女比为1∶2.8。MS首发及常见症状为感觉异常、肢体无力、视力减退及括约肌功能障碍,病变部位以脊髓受累最多见,部分患者存在精神情感障碍。结论出现感觉异常有助于MS早期诊断,诱发电位及MRI有助于发现亚临床病变;括约肌功能异常多见,可能与脊髓受累较多有关;MS患者的精神情感应受人们关注。     

早发型多发性硬化的临床特点

目的总结中国早发型多发性硬化(EOMS)的临床特点。方法从临床、辅助检查等方面对32例EOMS病例进行回顾性分析。结果EOMS好发于10岁后儿童,无性别差异,急性起病为主,首发症状以肢体无力多见,视力、运动和感觉障碍是EOMS患者最常见的症状,可合并周围神经系统损害,诱发电位检查阳性率较高,影像学改变以大脑半球和脊髓受累多见。结论EOMS是多发性硬化的一个亚型,其起病急、症状多样但预后较好,诱发电位和影像学检查对EOMS诊断有一定意义。     

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。     

多发性硬化35例临床、诱发电位及磁共振成像分析

目的　探讨多发性硬化 (MS)的临床特点以及诱发电位 (EP)、磁共振成像 (MRI)对MS的诊断价值。方法　分析 35例MS患者的临床表现及视觉诱发电位 (VEP)、体感诱发电位 (SEP)、脑干听觉诱发电位 (BAEP)和MRI的检查结果。结果　MS多见于青壮年 ,女性多见 ,呈急性或亚急性起病 ,视神经、脊髓、脑干受累多见 ,病程多缓解复发。VEP、SEP、BAEP、头颅MRI、脊髓MRI检查的异常率分别为 81.5 %、76 .2 %、5 2 %、77.8%、87.5 %。结论　MS的诊断主要依靠临床 ,EP和MRI检查对MS患者临床病灶的确定和亚临床病灶的发现均具有较高的敏感性 ,二者结合应用可显著提高MS的诊断正确率     

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158例临床特点分析

目的提高对多发性硬化(multiple sclerosis,MS)的诊断水平。方法回顾性分析158例MS患者的临床资料。结果MS好发于中年人(125,79.12%),女性(109,68.99%)病前多有诱因,首发症状多样,常见症状为肢体无力(145,91.77%)、感觉障碍(130,82.28%)、视力障碍(70,44.30%);MS侵犯部位:脊髓(110,69.62%)、视神经(90,56.92%)、大脑(85,53.79%)、脑干(70,44.30%)、小脑(65,41.14%)、周围神经(25,15.83%);病灶多呈斑片状,在T1加权像上可为低、稍低或等信号,在T2加权像上多为高信号,病灶多为斑片状或斑点状强化;MS多伴有诱发电位和脑脊液的异常。结论MS临床表现复杂多样,综合临床、诱发电位、脑脊液、影像学资料分析,提高对MS诊断的准确性,改善患者的预后。     

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。     

Assessing the value of spinal cord lesions in predicting development of multiple sclerosis in patients with clinically isolated syndromes

The purpose of this study was to determine the value of spinal cord lesions as a predictive factor for conversion in clinically isolated syndrome (CIS) patients. Patients with CIS and without immunomodulatory treatment were prospectively included. Age at onset, sex, clinical syndrome at onset, oligoclonal bands, and presence, number and location of lesions on brain and spinal MRI were analyzed. Conversion to multiple sclerosis (MS) was the primary endpoint. Cox regression was used to compare outcomes between groups. A total of 75 patients were included: 53 (71%) women, mean age at onset 32.7 years (SD ± 7.5), mean follow-up time 72.5 months (SD ± 9; range 17-104 months). There were 11 (14.6%) patients with one focal spinal cord lesion, while 13 (17%) patients had two or more spinal cord lesions at the first scan during the onset of the disease. Of the 23 patients (30.6%) who converted to clinically definite MS (CDMS), 2 had a normal spinal cord MRI, 8 patients had one spinal cord lesion, and 13 had more than one lesion on MRI (p < 0.001). In multivariable analyses, one focal spinal cord lesion was significantly associated with increased risk of conversion to MS (p = 0.01, HR 3.5, CI 95% 2.1-6.9), while the presence of two or more focal spinal cord lesions was independently associated with a higher risk of conversion to MS (p < 0.001, HR 5.9, CI 95% 3.2-10.8). CIS patients with an abnormal baseline spinal cord MRI have a higher risk for developing clinically definite MS, independent of brain lesions as well as the presence of cerebrospinal fluid oligoclonal banding (OSF-OB) .     

多发性硬化的MRI特征

目的 探讨多发性硬化(MS)患者脑及脊髓的MRI特征.方法 回顾性分析110例临床确诊的MS患者的MRI检查资料.结果 MS患者脑部病灶以侧脑室旁白质多见(55.8%),其次是额叶深部白质(54.7%)、顶叶深部白质(44.2%)、脑干(25.6%)、基底节(23.3%)、丘脑(11.6%)等,灰质也可受累;病灶大小不一,形态可为斑片状、斑点状、圆形、类圆形.脊髓病灶以颈、胸髓多见,分别占75.0%和68.8%,形态可为斑片状、条片状、类圆形,脊髓灰白质可同时受累,10.0%的患者出现脊髓形态改变,如增粗、萎缩.MS患者脑及脊髓内病灶在影像学上因病程不同可表现为长T1、长T2或等T1、长T2信号.结论 MS的MRI特点主要是以脑和脊髓白质出现多个大小、形状不同的病灶.     

糖皮质激素不同给药方式治疗急性期多发性硬化的疗效观察

目的探讨糖皮质激素3种不同给药方式治疗急性期多发性硬化(multiple sclerosis，MS)的疗效及安全性。方法将1998～2002年于我科治疗的48例MS患者按主要治疗方法的不同分为鞘内注入地塞米松(DXM)组、甲基强的松龙(MPS)冲击组及常规使用激素组。比较治疗后各组不同时间的Kurtzke扩充致残量表(Kurtzke expanded disability status scale，EDSS)评分减少数及副作用的发生率。结果鞘内注入DXM组治疗早期EDSS评分减少较其它两组显著，尤其对于脊髓受累为主的MSEDSS评分改善更为明显。结论鞘内注入DXM与两种常规方法比较见效快，建议用于脊髓受累为主的急性期MS。     

视神经脊髓炎患者33例脑部磁共振分析

目的 探讨视神经脊髓炎(neuromyelitis optica,NMO)患者脑部MRI影像学表现.方法 收集满足最新NMO诊断标准且脑部MRI表现不符合多发性硬化诊断标准的患者33例,均行脑部和脊髓MRI检查,分析其MRI影像学特点.结果 33例NMO中,脑部正常表现者5例(15.2%),异常表现28例(84.8%),其中脑内实质有明确病灶22例(66.7%),另6例(18.2%)脑内虽未见明确病灶,但深部脑白质显示了肉眼可视的对称性弥漫性脱髓鞘高信号影.22例明确病灶中,15例病灶数≥2个,7例为单个病灶.幕上近皮质、皮质下和深部脑白质区的点状非特异性病灶最多,少数为非典型的斑片状融合病灶.幕下脑干是易受累的部位(14/33,42.4%),特别是延髓(7/33,21.2%).结论 NMO患者出现脑内异常较为常见,有脑部的异常不能排除NMO的诊断.认识NMO脑内病灶对完善NMO诊断标准有帮助.     

Magnetic resonance imaging of Asians with multiple sclerosis was similar to that of the West

Magnetic resonance imaging (MRI) of the brain is the most important paraclinical diagnostic test in multiple sclerosis (MS). The appearance of MRI in Asians with MS is not well defined. We retrospectively surveyed the first brain and spinal cord MRI in patients diagnosed to have MS, according to Poser's criteria in seven regions throughout Asia to define the MRI changes among Asians with MS. There were 101 patients with first brain, and 86 with first spinal cord MRI, 66 of whom had both. The brain MRI showed a mean of 17 lesions per patient in T2 weighted images, mostly asymptomatic. Almost all the lesions were in the white matter, particularly in the juxtacortical, deep and periventricular white matter. A third of the lesions were greater than 5 mm, 14% enhanced with gadolinium. There were more supratentorial than infratentorial lesions at a ratio of 7.5: 1. Ninety five percent of the spinal cord lesions were in cervical and thoracic regions, 34% enhanced with gadolinium. The lesions extended over a mean of 3.6 +/- 3.3 vertebral bodies in length. Fifty (50%) of the brain and 54 (63%) of the spinal MRI patients had the optic-spinal form of MS. The MRI of the optic-spinal and classical groups of patients were similar in appearance and distribution, except that the optic-spinal MS patients have fewer brain but longer and more severe spinal cord lesions. In conclusion, the brain and spinal cord MRI of Asian patients with MS was similar to that of the West, although, in this study, Asian MS patients had larger spinal cord lesions.     

Spinal-cord MRI in multiple sclerosis

The potential of MRI of the spinal cord as a diagnostic tool in MS has recently gained much interest. Dual echo spin echo MRI is most sensitive for the detection of spinal-cord abnormalities, which range from multiple focal lesions to confluent areas of high signal intensity. In some patients, commonly those with primary progressive disease, diffuse areas of slightly increased signal intensity are found. Disappointingly, the relation between MRI findings and clinical disability is weak. Studies relating MRI findings with histopathology have revealed substantial axonal loss in the spinal cords of patients with MS, whether focal lesions are present of not. Further, diffuse cord atrophy is found in advanced MS, which may reflect axonal loss. In the diagnostic setting, spinal-cord imaging is valuable. First, asymptomatic spinal-cord lesions are very rare in disorders other than MS. For example, in a patient with equivocal brain findings such as an elderly patient with vascular-ischaemic lesions, a normal spinal-cord examination can help rule out MS. Second, presence of asymptomatic spinal lesions may help confirm a diagnosis of MS when few or no brain lesions are present.     

Multisequence MRI in clinically isolated syndromes and the early development of MS.

OBJECTIVE: To apply multisequence MRI techniques to patients with clinically isolated syndromes, to document the pattern and frequency of abnormalities at baseline and early follow-up, and to determine their predictive values for the early development of clinical MS. BACKGROUND: Disseminated lesions on T2-weighted brain MRI confer an increased risk of progression to clinically definite MS. Newer MRI techniques increase detection of lesions in both brain and spinal cord, and clarify further their pathology. The predictive value of such techniques for the development of clinical MS needs to be defined. METHODS: Brain and spinal MRI were performed on 60 patients after their first demyelinating event. A total of 50 patients were followed for 1 year, and 49 underwent repeat brain MRI 3 months after the initial scan. RESULTS: At baseline, 73% of patients had lesions on T2-weighted fast spin-echo (FSE) brain images and 42% had asymptomatic spinal cord lesions. Fast fluid-attenuated inversion-recovery brain did not improve detection of brain lesions. Repeat brain MRI demonstrated new FSE lesions in 43% of patients. After 1 year, 26% of patients developed MS. The MRI features that provided the best combination of sensitivity and specificity for the development of MS were the presence of new FSE lesions at follow-up and enhancing lesions at baseline. The frequency of developing clinical MS was higher for those with both brain and spinal cord lesions at baseline (48%) than brain lesions alone (18%). CONCLUSIONS: The combination of baseline MRI abnormalities and new lesions at follow-up, indicating dissemination in space and time, was associated with a high sensitivity and specificity for the early development of clinical MS. These data suggest a potential role for new diagnostic criteria for MS based on early MRI activity. Such criteria may be useful in selecting patients for therapeutic trials at this early clinical stage.     

Gadolinium-enhanced brain lesions in multiple sclerosis relapse

ObjectiveTo study the clinico-radiological paradox in multiple sclerosis (MS) relapse by analyzing the number and location of gadolinium-enhanced (Gd+) lesions on brain MRI before methylprednisolone (MP) treatment.MethodsWe analyzed brain MRI from 90 relapsed MS patients in two Phase IV multicenter double-blind randomized clinical trials that showed the noninferiority of different routes and doses of MP administration. A 1.5- or 3-T brain MRI was performed at baseline before MP treatment and within 15 days of symptom onset. The number and location of Gd+ lesions were analyzed. Associations were studied using univariate analysis.ResultsSixty-two percent of patients had at least 1 Gd+ brain lesion; the median number was 1 (interquartile range 0–4), and 41% of patients had 2 or more lesions. The most frequent location of Gd+ lesions was subcortical (41.4%). Gd+ brain lesions were found in 71.4% of patients with brainstem-cerebellum symptoms, 57.1% with spinal cord symptoms and 55.5% with optic neuritis (ON). Thirty percent of patients with brain symptoms did not have Gd+ lesions, and only 43.6% of patients had symptomatic Gd+ lesions. The univariate analysis showed a negative correlation between age and the number of Gd+ lesions (p = 0.002).ConclusionMost patients with relapse showed several Gd+ lesions on brain MRI, even when the clinical manifestation was outside of the brain. Our findings illustrate the clinico-radiological paradox in MS relapse and support the value of brain MRI in this scenario.     

脊髓型多发性硬化的MRI表现

目的总结分析脊髓型多发性硬化的MRI表现。方法搜集经临床证实的脊髓型多发性硬化11例,均行MRI检查,对其临床及MRI资料进行回顾性分析。结果脊髓型多发性硬化的特征性MRI表现为,11例患者的病灶以颈髓多见,病变脊髓在T1WI像为低或等信号,T2WI像为高信号,病灶位于脊髓两侧和后部,病灶活动期呈斑片状或边缘强化,应用糖皮质激素试验性治疗对脊髓出现的可疑脱髓鞘病灶者有一定的帮助。结论脊髓型多发性硬化有其特征性MRI表现,MRI有助于脊髓型多发性硬化的诊断,是目前诊断脊髓型多发性硬化最敏感的影像学方法 。     

中枢神经脱髓鞘性疾病病理组织学与免疫组化研究

目的　研究中枢神经脱髓鞘性疾病的病理组织学及免疫反应。方法　多发性硬化（ＭＳ）２ 例,视神经脊髓炎（ＤＤ）、急性播散性脑炎（ＡＤＥ）及急性出血性白质脑病（ＡＨＬＥ）各１ 例进行脑及脊髓病理组织学观察,以抗ＩｇＧ、ＩｇＡ、ＩｇＭ、Ｇ３ 补体、ＣＤ２０、ＣＤ４５ＲＯ抗体组化染色。结果　各种脱髓鞘性疾病脑部以白质脱鞘为主,脊髓则灰白质同时受累,坏死常见。小血管周围有不同程度炎细胞浸润。组化染色见较多Ｔ细胞浸润,ＩｇＧ、ＩｇＡ、ＩｇＭ、Ｃ３ 补体多见于细胞内,主要是巨噬细胞,亦见于星形细胞、浆细胞,在ＡＨＬＥ坏变血管壁、血管周围间隙、软膜及室管膜沉积较多。结论　ＭＳ与ＤＤ病变性质相同而部位有异,ＡＨＬＥ突出表现为小血管壁坏死、灶性出血,ＡＤＥ介于二者之间。脱髓鞘性疾病与免疫反应密切相关,各型间免疫反应类型与程度有所不同。星形胶质细胞在脱髓鞘性疾病免疫应答中可能发挥了重要作用。