105例多发性硬化临床特点及治疗分析

目的 探讨多发性硬化的临床发病特点及临床治疗疗效.方法 从2005-01至2010-10月间收治的多发性硬化患者105例为研究对象.结果 105例患者中以20-40岁女性多见,以急性、亚急性起病,以肢体无力为首发症状最常见,病变部位多以脑和脊髓多见,脑脊液蛋白升高、IgG增高及寡克隆区带阳性常见,核磁共振较计算机断层扫...     

脑脊液寡克隆区带在多发性硬化和视神经脊髓炎中的应用

目的探讨脑脊液寡克隆区带在多发性硬化和视神经脊髓炎2组疾病中的应用价值。方法通过等电聚焦电泳加免疫固定染色的实验室方法检测多发性硬化和视神经脊髓炎2组患者的脑脊液寡克隆区带,比较2组疾病的脑脊液寡克隆区带特点。结果多发性硬化患者共120例,其中脑脊液寡克隆区带阳性39例,阳性率为32.5%;视神经脊髓炎患者共45例,其中脑脊液寡克隆区带阳性4例,阳性率为8.9%。多发性硬化组脑脊液寡克隆区带阳性率明显高于视神经脊髓炎组,差异有统计学意义(P=0.002)。结论本结果对2组疾病的鉴别诊断具有一定的指导意义,且支持多发性硬化和视神经脊髓炎发病机制不同的观点。     

经病理证实的以瘤样脱髓鞘病为首发表现的多发性硬化临床分析

目的研究经病理证实的以瘤样脱髓鞘病变(tumefactive demyelinating lesions,TDL)为首发表现的多发性硬化(multiple sclerosis,MS)患者的临床、影像学及病理学特点。方法收集2011-01-01-2013-08-05就诊于海军总医院以TDL为首发表现的MS患者6例。6例患者均在首次发病时行脑立体定向活检术,分析所有患者的临床资料及诊治经过,并定期随访24个月(最后1例患者末次随访日期为2015-10-20)。结果 6例患者中男4例、女2例,年龄12~44岁,平均(33.5±12.4)岁。6例患者中:(1)首次发病(TDL表现)表现为头痛3例、肢体无力2例、头晕伴耳鸣1例;实验室检查:IgG合成指数3例升高;病灶部位:分布于侧脑室旁6例、基底节区3例、额叶4例、延髓3例、颞叶2例、胼胝体2例。(2)复发(MS确诊后表现)表现为肢体麻木伴感觉异常3例、伴肢体无力2例,头晕伴耳鸣1例,1例随访仅见2次影像学复发,无神经系统功能缺损症状;实验室检查:脑脊液寡克隆区带(OCB)3例阳性,血清MBP升高4例,脑脊液MBP升高3例;病灶部位:多累及额叶4例、侧脑室旁4例、颞叶3例、基底节区3例、胼胝体2例、延髓3例、大脑脚2例、桥脑2例。6例均以TDL起病,TDL出现距MS确诊的时间约6~24个月,平均(12.0±7.6)个月。所有患者均有≥2次临床或影像学发作史,平均复发次数为(3.2±1.0)次,年平均复发次数约(1.5±0.5)次/年。结论以TDL首发的MS临床表现多样,多以头痛起病,首次发病与复发期的脑MRI病灶多位于脑室旁,复发期脑脊液OCB可能由阴性转为阳性。重视复发期脑脊液OCB和脑MRI的动态复查有助于判断TDL的临床转归,减少MS的临床漏诊。     

脑脊液寡克隆区带和核磁共振检查对多发性硬化的临床诊断价值

目的 :探讨脑脊液寡克隆区带CSFOB和头颅MRI的变化对诊断多发性硬化 (MS)的临床价值。方法 :对 2 5例怀疑多发性硬化 (MS)的患者进行脑脊液的寡克隆区带和头颅MRI的检查 ,研究两者和MS间的关系。结果 :确诊多发性硬化的患者均有脑脊液的寡克隆带和头颅核磁共振 (头MRI)异常。结论 :脑脊液寡克隆带和头MRI两者结合对诊断发硬化 (MS)有重要临床价值。     

多发性硬化脑脊液寡克隆区带及24小时鞘内IgG合成率检测的比较

采取垂直平板聚丙烯酰胺凝胶电泳法、火箭免疫电泳法对30例多发性硬化病人进行脑脊液寡克隆区带及24小时鞘内IgG合成率的检测,结果发现寡克隆区带、合成率与多发性硬化的发病年龄、病程、病程进展类型、病残程度无统计学上的相关性,寡克隆区带阳性率46.7％,合成率增高占60％,两者合计异常率可达73.3％,提示我国多发性硬化寡克隆区带阳性率、合成率增高均较国外低,因而在我国对多发性硬化的实验室诊断两者同时检测更有意义。     

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。     

脑脊液寡克隆区带和核磁共振检查对多发性硬化的临床诊断价值

目的：探讨脑脊液寡克隆区带CSFOB和头颅MRI的变化对诊断多发性硬化（MS）的临床价值。方法：对25例怀疑多发性硬化（MS）的患者进行脑脊液的寡克隆区带和头颅MRI的检查，研究两者和MS间的关系。结果：确诊多发性硬化的患者均有脑脊液的寡克隆带和头颅核磁共振（头MRI）异常。结论：脑脊液寡克隆带和头MRI两者结合对诊断发硬化（MS）有重要监床价值。     

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。     

视神经脊髓炎水通道蛋白4抗体分布特征及其与脑脊液寡克隆区带间关系的临床研究

目的观察视神经脊髓炎患者水通道蛋白4(AQP4)抗体在血清和脑脊液的分布特征,探讨血清AQP4抗体与脑脊液寡克隆区带之间的关系。方法采用酶联免疫吸附试验和间接免疫荧光法检测视神经脊髓炎和多发性硬化患者血清和脑脊液AQP4抗体,动态定时散射比浊法检测白蛋白和IgG,等电聚焦电泳联合免疫固定法检测脑脊液寡克隆区带,免疫印迹法检测寡克隆区带阳性视神经脊髓炎患者脑脊液电泳条带中AQP4抗体。结果视神经脊髓炎组患者血清AQP4抗体滴度[8.94(5.41,11.93)ng/ml]与多发性硬化组[7.34(4.76,12.00)ng/ml]相近(Z=-0.510,P=0.610),脑脊液AQP4抗体滴度[0.45(0.42,0.47)ng/ml]高于多发性硬化组[0.41(0.40,0.41)ng/ml;Z=-2.359,P=0.018],而且血清水平高于脑脊液(Z=-3.702,P=0.000)。视神经脊髓炎组患者脑脊液AQP4抗体阳性检出率高于多发性硬化组(5/7对1/5),但差异未达到统计学意义(Fisher确切概率法:P=0.242);复发期血清AQP4抗体滴度[8.54(5.32,11.42)ng/ml]与缓解期[9.97(5.41,13.28)ng/ml]相近(Z=-0.347,P=0.728);寡克隆区带阳性检出率低于多发性硬化组(3/13对10/14)且差异有统计学意义(Fisher确切概率法:P=0.021)。未在寡克隆区带阳性视神经脊髓炎患者的IgG电泳条带中检出AQP4抗体。结论视神经脊髓炎患者血清AQP4抗体滴度高于脑脊液,行脑脊液AQP4抗体检测具有一定临床意义。视神经脊髓炎患者鞘内合成IgG能力低于多发性硬化患者,且无针对AQP4抗原的成分。     

多发性硬化42例临床分析

目的 探讨多发性硬化(MS)的临床特点、诊断和治疗.方法 分析42例多发性硬化患者的一般资料、病变部位、主要症状、重要辅助检查及其治疗方法和效果.结果 42例MS患者中青壮年女性多见,以急性、亚急性起病为主,首发症状以肢体无力最常见,病变部位以脊髓和视神经最常见,实验室检查以脑脊液蛋白水平和IgG指数增高常见.MRI异常率高达85.96%.结论 MS是一种临床表现复杂、累及中枢神经系统白质,多部位、多时相的自身免疫性疾病.根据临床特点、神经电生理、脑脊液免疫学及磁共振成像检查能提高临床确诊率,激素治疗对大多数病人有效.     

多发性硬化的临床特点与预后的分析

目的分析多发性硬化(MS)的临床特点与预后。方法回顾性分析117例MS患者的临床资料,从临床、影像学等方面对视神经脊髓型(OSMS)和经典MS型(CMS)进行比较。结果117例MS患者中,OSMS型42例(35.9%),CMS型75例(64.1%)。临床表现中,OSMS患者出现肢体无力(88.1%)、感觉缺失(85.7%)、感觉异常(57.1%)、视物模糊(76.2%)、尿便障碍(73.8%)明显多于CMS患者(70.7%、56.0%、20.0%、45.3%及26.7%)(均P<0.05);出现共济失调(7.1%)、复视(0)及构音不清(0)明显少于CMS患者(42.7%、10.7%及16.0%)(P<0.05～0.01)。OSMS患者出现长节段融合性脊髓病变的比例(94.1%)显著多于CMS患者(48.2%)(P<0.01)。脑脊液检查和诱发电位检查结果OSMS组和CMS组差异无统计学意义。发病5年后扩大的残疾状态量表(EDSS)评分OSMS组(2.42±0.90)显著高于CMS组(1.50±0.88)(P<0.05)。在发病的第1年和第3年,年平均复发次数复发缓解型OSMS[(1.92±1.05)次、(1...     

Balo''s concentric sclerosis diagnosed intravitam on brain biopsy

A 28-year-old woman presented with acute right hemiparesis evolving to bilateral hemiplegia and a mute state within 1 week on a background of paranoid psychosis of 8 years duration. CT scan revealed multiple bilateral hypodense enhancing lesions in the central white matter. Biopsy of the lesion showed lamellar alternating zones of demyelination and remyelination, typical of Balo's concentric sclerosis, an acute variant of multiple sclerosis. Immunohistochemical staining for neurofilament showed greater damage to the axis cylinders by the disease than generally perceived by silver stains. Steroid therapy produced a gratifying and sustained improvement. Follow up CT scans and psychometry revealed regression of the demyelinating lesions and improvement of the cognitive state.     

The frequency of relapse in multiple sclerosis

Summary The authors analyze the course of 245 cased of multiple sclerosis. The mean annual frequency of attacks is 0.66 for all the patients (remittent forms and progressive forms). Although it is usually suggested that this frequency decreases with the years, this has not been found in our study. Our results also indicate that we would have to follow 590 patients over 1 year or 190 over 2 years before being able to attest the effectiveness of a treatment decreasing the frequency of attacks by 25%.Ingénieur I.N.S.E.R.M.     

Western and Asian features of multiple sclerosis in Mexican Mestizos

OBJECTIVES: The objective of this study was to compare the clinical expression of MS in Mexican Mestizos with that of patients of European or Asian descent; as well as to compare the annual frequency of new cases with that observed in the previous decades. PATIENTS AND METHODS: All patients with diagnosis of definite MS seen at the National Institute of Neurology and Neurosurgery of Mexico from January 1993 to December 2003 were studied (n=312). Sociodemographic and clinical characteristics were compared with reports of patients from either Western or Asian origin; the long-term disability score was analyzed according to gender, age of onset of MS and the initial symptom. RESULTS: The clinical expression of MS in Mexican Mestizos shares some characteristics with both, Asian and Western forms of MS indicating that the genetic composition of Mexican Mestizos participates in the clinical expression of the disease. Also, at the prevalence date, the mean age of patients and the duration of the disease were lower in our patients than in MS patients from endemic countries suggesting a true increasing incidence in recent times, rather than only improved case ascertainment. CONCLUSIONS: Clinical expression of MS in Mexican Mestizos shows the coexistence of some features common in European and in Asian cases.     

The risk of motor neurone disease and multiple sclerosis is different in Estonians and Russians. Data from South Estonia

Epidemiological studies were performed in South Estonia to establish the prevalence rate of multiple sclerosis (MS) and motor neurone disease (MND). The case finding method included information from the hospital records of the central hospital in the region-the University Hospital (for MS from 1942 to 1989), from all neurologists in the region, from the Estonian MS Society and Association of Muscular Disorders, and from nursing homes in the region. The prevalence day was 31 December 1989. MND incidence was established for the period of 1986-1995. The results demonstrated high prevalence rates of MS among native Estonians (55.3 per 100 000), somewhat lower prevalence among native-born representatives of other nationalities (43.6 per 100 000) and the lowest prevalence rate of MS among non-Estonian immigrants (26.6 per 100 000). The differences were not statistically significant. The results for MND demonstrated the opposite pattern. The mean annual incidence rate of MND for 10 years was statistically significantly higher among people of other nationalities (2.5 per 100 000) and Russians (2.6 per 100 000), and lower in native-born Estonians (1.1 per 100 000). No differences in health care or clinical picture were established. The reasons for the demonstrated differences in MND incidence remain unclear.     

Proton magnetic resonance spectroscopy of normal appearing white matter in familial and sporadic multiple sclerosis

Objective To assess metabolite levels in normal–appearing white matter in sporadic and familial multiple sclerosis (MS). Methods Using H–MRS and applying one voxel measure we assessed NAA/Cho,NAA/Cr and Cho/Cr ratios in 26 patients with sporadic and in 25 with familial MS and compared them with healthy subjects. Results In both MS groups NAA/Cho and NAA/Cr ratio were significantly lower than in healthy individuals whereas Cho/Cr ratio was higher in MS patients. In sporadic MS patients NAA/Cho and NAA/Cr ratios were lower, although not significantly, than in familial cases. The Cho/Cr ratio was similar in both MS groups. Conclusion These results suggest that subtle differences in H–MRS measures corresponding to axonal rather than to myelin pathology might occur in sporadic and familial MS.     

Balo's concentric sclerosis. Evolution of active demyelination demonstrated by serial contrast-enhanced MRI

We report a case of Balo's concentric sclerosis with peculiar MRI findings. Unlike previously published cases all concentric rings in our case showed marked enhancement, supporting the view of synchronous active demyelination in the lesion. Follow-up MRI disclosed a change of the lesions into a confluent pattern more resembling a typical large MS plaque. There were no oligoclonal bands or intrathecal Ig-G synthesis. The hypothesis concerning the pathophysiology of the lesions' typical and peculiar morphological appearance and its relationship to multiple sclerosis are briefly discussed. Received: 18 August 2001, Received in revised form: 9 November 2001, Accepted: 21 November 2001     

Clinical picture of multiple sclerosis with late onset

A group of 47 patients (multiple sclerosis (M.S.) diagnosed according to Schumacher's criteria) with age at onset over 40 years, was studied by statistical analysis and compared with a control group (100 M.S. patients with onset between ages 18 and 38).
The following features appear peculiar in this group:
– the most frequent initial symptoms are motor disturbances
– the onset is generally monosymptomatic
– the course is generally progressive and more severe than that of controls, as seen by Kurtzke's Disability Scale, relapse rate, interval between first and second attack
– the course is more severe when the onset is progressive and characterized by motor disturbances.     

Balo's Concentric Sclerosis–A Case Report

This is a report of Balo's concentric sclerosis in a 23-year-old Filipino male, who presented behavioral abnormalities, gait disturbance, double incontinence and generalized paratonia with the entire course of a six-month duration. A review of literature on this rare form of demyelinating disease is discussed.