关于急性播散性脑脊髓炎的新认识

急性播散性脑脊髓炎是一种中枢神经系统的特发性炎症脱髓鞘疾病,临床表现多样,可有复发病程.目前其病因与发病机制尚不清楚,病理研究有限,诊断标准不一.本文综述了其发病机制、病理表现、临床特点、诊断等近几年的研究进展.     

47例急性播散性脑脊髓炎临床研究

目的评价急性播散性脑脊髓炎(ADEM)的诊断与治疗.方法对47例符合诊断标准的ADEM的临床特征、实验室及MRI,以及单用甲基强的松龙(MPPT)组和甲基强的松龙+丙种球蛋白(IVIG)组之间的疗效关系进行分析.结果 47例ADEM中有29例(61.7%)有先驱感染性疾病,服驱虫药4例(8.51%),不明原因14例(29.79%),症状和体征数天之内进展达高峰.认知功能损害为最常见的症状,有26例(55.32%).MRI最常见双侧脑白质不对称类圆形病灶,而深部脑灰质核团亦见累及.单用甲基强的松龙治疗与甲基强的松龙联用丙种球蛋白两组之间的疗效有显著差异.结论 ADEM是一种急性中枢神经系统脱髓鞘疾病.MRI是诊断ADEM的重要手段,大剂量应用皮质激素与丙种球蛋白可取得较好的疗效.     

急性播散性脑脊髓炎的护理体会

急性播散性脑脊髓炎（ADEM）是一种少见的脑脊髓弥散性损害的炎症脱髓鞘疾病，常与病毒感染、免疫接种及某些非特异性因素有关。临床主要表现为脑和脊髓弥散性损害为症状和体征。可分为脑型、脊髓型和脑脊髓型。主要症状为脑膜刺激征、脑神经麻痹、偏瘫、截瘫、大小便障碍等。因本病起病急，进展迅速，重者可危及生命，做好护理工作对降低病死率，减少后遗症十分重要。我院自1997年以来共收治急性播散性脑脊髓炎6例，现将护理体会总结如下。     

急性播散性脑脊髓炎、多发性硬化及视神经脊髓炎脑深部灰质病灶MRI影像比较

目的 探讨并比较急性播散性脑脊髓炎(ADEM)、多发性硬化(MS)及视神经脊髓炎(NMO)脑深部灰质病灶的MRI影像学特征. 方法 自2004年8月至2012年10月在中山大学附属第三医院神经内科住院的ADEM、MS、NMO患者共353例,筛选出其中MRI显示有脑深部灰质病灶者95例(包括ADEM 12例,MS 60例,NMO 23例),对这些病灶的大小、数量、部位等特征进行分析. 结果 3组患者丘脑、尾状核、苍白球受累的病例数比例比较差异均无统计学意义(P=0.154,P=0.438,P=0.697).ADEM组壳核受累的病例数比例明显高于MS组、NMO组,差异有统计学意义(P=0.002,P=0.013).NMO组下丘脑受累的病例数比例则明显高于ADEM组、MS组,差异有统计学意义(P=0.033,P=0.001).ADEM组丘脑的病灶直径明显大于NMO组,差异有统计学意义(P=0.027),但和MS组相比差异无统计学意义(P=0.116),而MS组和NMO组丘脑的病灶直径比较差异亦无统计学意义(P=0.209).3组尾状核、壳核、苍白球、下丘脑的病灶直径比较差异均无统计学意义(P＞0.05).3组的病灶分布对称性比较差异无统计学意义(P=0.335). 结论 丘脑受累对于ADEM和MS的鉴别诊断可能意义不大,壳核受累可能是将ADEM区别于MS和NMO的一个鉴别点,下丘脑受累是NMO的特异性表现.病灶直径大小在这三种疾病的鉴别诊断中价值不大.     

急性播散性脑脊髓炎与经典多发性硬化的临床对比分析

目的 寻找临床上鉴别急性播散性脑脊髓炎(acute disseminated encephalumyelitis,ADEM)与经典多发性硬化(classical multiple sclerosis,CMS)的方法.方法 回顾性分析20例ADEM和24例CMS患者的流行病学特点、临床症状、实验室检查和MRI,对各定性资料进行卡方检验,定量资料进行两独立样本的Wilcoxon秩和检验.结果 ADEM患者起病年龄[(27±15)岁]较CMS患者[(37±13)岁,Z=-2.218,P=0.027]小.ADEM患者通常有前驱感染史(75%),发热(65%)、脑膜刺激征(40%)、癫痫(25%),较CMS者常见(x2=23.652、18.609、9.189、4.514,均P＜0 05),脑病更多见于ADEM患者.ADEM患者血白细胞[(11 9±5.8)×109/L,Z=-2.030,P=0.042]、C反应蛋白(2.74 mg/L,Z=-3.028,P=0.002)、红细胞沉降率(11.00 mm/h,Z=-2 406,P=0.016)、脑脊液白细胞(9×106/L,Z=-2.781,P=0.005)较CMS患者[上述指标分别为(8.0±3.2)×109/L、0.49 mg/L、7.00 mm/h、2 ×106/L]高,脑脊液蛋白(ADEM组0.19 g/L,CMS组0.17 g/L)及寡克隆带(OCB)阳性率(ADEM组4/20,CMS组11/24)在两者间差异无统计学意义.在MRI上,ADEM患者更多见皮质灰质病灶(14/20,x2=15.213,P=0.000)、基底节区灰质病灶(14/20,x2=8.910,P=0.003)和脑干病灶(14/20,x2=5.867,P=0.015),脊髓病灶多近中央分布(83%,x2=11.542,P=0 001),病灶边界模糊(95%,x2=21.787,P=0.000);CMS患者更多见近皮质白质病灶(21/24,x2=17.628,P=0.000)、侧脑室旁病灶(21/24,x2=15.213,P=0.000)和胼胝体病灶(14/24,x2=8.640,P=0.003),脊髓病灶多呈偏心分布(85%),病灶边界清楚(75%).结论 ADEM与CMS无论在流行病学特点、临床症状,还是在脑脊液和MRI检查方面都有一定差异.

Abstract：

Objective To improve differential diagnosis between acute disseminated encephalomyelitis ( ADEM) and classical multiple sclerosis ( CMS).Methods All 20 cases of ADEM and 24 cases of CMS were examined.Their epidemiological and clinical findings,laboratory features and magnetic resonance imaging ( MRI) data were analyzed using x2 test for categorical variables,Wilcoxon Rank-Sum tests for continuous variables.Results ADEM and CMS showed no sex predominance.Patients with ADEM ((27 ±15) years) were younger than CMS ((37 ±13) years,Z= -2.218,P =0.027).The following findings were more commonly seen in ADEM compared with CMS:predemyelinating infectious disease (75% vs 4%,x2 =23.652,P = 0.000),fever (65% vs 4%,x2 =18.609,P = 0.000),meningeal irritation sign (40% vs 0,x2 = 9.189,P =0.002),seizure (25% vs 0,x2 =4.514,P = 0.034),and encephalopathy.ADEM patients were more likely to present with blood leucocytosis ( (11.9 ± 5.8) ×109/L vs (8.0±3.2) ×109/L,Z= -2.030,P=0.042),high C-reactive protein (2.74 mg/L vs 0.49 mg/L,Z = - 3.028,P = 0.002),increased erythrocyte sedimentation rate (11.00 mm/h vs 7.00 mm/h,Z= -2.406,P =0.016),and cerebrospinal fluid leucocytosis (9 × 106/L vs 2×106/L,Z =- 2.781,P = 0.005).There were no differences in cerebrospinal fluid protein and oligoclonal band between the two groups.The following MRI lesions were more commonly seen in ADEM patients:cortical gray matter lesions (14/20,x2=15.213,P=0.000),basal ganglia gray matter lesions (14/20,x2 =8.910,P = 0.003),and brainstem lesions ( 14/20,x2 = 5.867,P = 0.015).In contrast,lesions in subcortical white matter (21/24,x2 = 17.628,P =0.000),periventricular area (21/24,x2 =15.213,P=0.000) and corpus callosum ( 14/24,x2 = 8.640,P = 0.003 ) were more common in the MRI image of CMS patients.The lesions in spinal cord were usually centrally distributed in ADEM (83% ),while peripherally in CMS (85%,x2 = 11.542,P = 0.001).The lesions had poorly defined margins in ADEM (95%),but well defined margins in CMS (75%,x2 =21.787,P = 0.000).Conclusion There are differences in epidemiological and clinical findings,laboratory features and MRI appearances between ADEM and CMS.     

儿童急性播散性脑脊髓炎21例临床分析

目的分析儿童急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)的临床特点及辅助检查特点,以提高对此病的早期诊断及治疗。方法对2008-01—2013-05我院21例ADEM患儿的临床表现、辅助检查、治疗经过及预后进行回顾性分析。结果 21例患儿中有前驱感染病史者17例(80%),接种疫苗史2例。起病方式呈急性或亚急性起病,首发症状多样。MRI主要表现为白质受累。13例患儿脑脊液检查异常(62%),主要表现为细胞数及蛋白轻度升高。所有病例予以大剂量甲泼尼龙,12例合用大剂量免疫球蛋白,病情大多明显好转。结论儿童ADEM临床症状多样,结合MRI、脑脊液检查等多种辅助检查有助于及时明确诊断,患儿预后较好。     

急性播散性脑脊髓炎12例临床分析

目的探讨急性播散性脑脊髓炎临床特点及治疗原则。方法回顾1990年5月-2010年12月住院治疗且诊断明确的急性播散性脑脊髓炎患者临床资料,结合文献分析其临床表现、实验室检查及影像学特点。结果共12例患者符合入组条件,男性10例,女性2例,年龄6～69岁(中位年龄34.50岁)。其中9例发病前出现前驱感染症状如上呼吸道感染(6例)或无诱因发热(3例),以及诱发因素如麻疹疫苗接种史(1例)或麻疹病史(1例)。临床主要表现为发热,恶心、呕吐,头痛,神经系统症状与体征以脑神经受累(展神经常见),瘫痪(偏瘫、截瘫、四肢瘫),感觉异常,膀胱功能障碍[尿失禁和(或)尿潴留],脑膜刺激征阳性,以及不同程度意识障碍为主。大剂量糖皮质激素冲击疗法或人血丙种球蛋白静脉注射治疗有效。结论尽管急性播散性脑脊髓炎发病凶险,但早期明确诊断及鉴别诊断,及时治疗,可明显改善患者预后。     

急性播散性脑脊髓炎临床特征及文献复习

急性播散性脑脊髓炎(ADEM)是一种累及中枢神经系统脱髓鞘性疾病,临床表现为多灶性中枢神经功能缺损症状,致死率高达20%,临床误诊率较高。目前其发病机制尚未明确,患者发病前常有病毒、细菌感染或疫苗接种史[1]。该文总结了5例ADEM患者的临床及影像学特征,旨在为临床诊治提供依据。1对象和方法     

成人急性播散性脑脊髓炎误诊分析

目的 分析急性播散性脑脊髓炎(ADEM)的误诊原因,以提高对本病的认识.方法 对5例误诊为其他病的ADEM患者的临床表现、实验室检查、影像学表现、治疗与转归进行回顾性分析.结果 5例ADEM患者分别被误诊为急性吉兰-巴雷综合征、病毒性脑炎、急性脑梗死、脑囊虫、颅内多发转移瘤,经对其发病诱因、临床表现、实验室检查、影像学表现等进行综合分析后明确ADEM的诊断,予以激素和(或)丙种球蛋白治疗,临床症状均有不同程度好转.结论 ADEM临床表现多样,MRI检查有助于早期诊断,提高对本病的认识,有助于减少误诊.     

急性播散性脑脊髓炎的临床、MRI表现及治疗

目的研究急性播散性脑脊髓炎（ADEM）的临床特点。方法对2例临床确诊为ADEM患者进行回顾及随访．并对临床和影像学表现等进行分析。结果神经系统症状多样,影像学表现：主要累及脑白质的脱髓鞘病变,应用激素治疗有效。结论ADEM是一种中枢神经系统炎性脱髓鞘疾病,临床表现多样。MRI表现有一定的特征性,MRI检查有利于早期诊断,也是评价疗效的重要手段。     

Acute disseminated encephalomyelitis after bee sting

Acute disseminated encephalomyelitis (ADEM) usually follows a viral infection or an immunization and is thought to be an immunomediated disease. We describe a patient with ADEM after multiple yellow jacket bee stings. The patient recovered after treatment with a high dose of methylprednisolone. Although the pathologic mechanism exact remains unclear, potential cross-reactivity between bee toxins and the central nervous system myelin could induce demyelination. ADEM should be considered a rare complication of bee stings. Received: 15 April 2002 / Accepted in revised form: 5 December 2002 Correspondence to C. Boz     

神经节苷脂治疗吉兰-巴雷综合征疗效及肌电图变化的临床评价

目的:评价神经节苷脂在促进吉兰-巴雷患者恢复方面上的临床疗效及肌电图变化。方法:将符合入选标准的36例患者随机分为2组,分别采用神经节苷脂和普通B族维生素治疗。治疗前后对两组患者定期进行MRC、Hughes评分以及肌电图检查,测定运动神经传导速度及F波平均潜伏期。结果:两组MRC、Hughes评分与治疗前相比均有显著或极显著差异(P<0.05-0.01),半年后两组MRC、Hughes评分比较有显著差异(P<0.05),半年后两组运动神经传导速度、F波潜伏期比较有显著差异(P<0.05),两组无明显不良反应。结论:神经节苷脂是促进吉兰-巴雷患者恢复的有效药物,无论是临床评分还是肌电图改善均优于普通B族维生素。     

A comparative study of acute and chronic diseases induced by two subgroups of Theiler’s murine encephalomyelitis virus

Theiler’s murine encephalomyelitis viruses (TMEV) are divided into two subgroups on the basis of their different biological activities. The GDVII strain produces acute polioencephalomyelitis in mice, whereas the DA strain produces demyelination with virus persistence in the spinal cord. A comparative study of GDVII and DA strains suggested that low host immune responses are responsible for the development of acute GDVII infection and that the persistence of infected macrophages plays a crucial role in the development of chronic white matter lesions in DA infection. All 78 mice infected with GDVII died or became moribund by day 13, while none of 54 mice infected with DA died. In the acute stage, the distribution of viral antigens in the central nervous system (CNS) tissue was similar in both GDVII and DA infections, although the virus titer was higher in GDVII infection. In DA infection, a substantial number of T cells were recruited to the CNS on day 6 when they were virtually absent in GDVII infection. The titer of neutralizing antibody was already high on day 6 in DA infection but was negligible in GDVII infection. Development of chronic paralytic disease from day 35 of the DA infection was accompanied by focal accumulation of viral antigen-positive macrophages in the spinal white matter. In addition, white matter lesions comparable to those in chronic DA infection were induced in the spinal cord within 7 days after intracerebral injection of DA-infected murine macrophages. Received: 26 June 1995 / Revised, accepted: 27 December 1995     

Tau protein concentrations in the cerebrospinal fluid of children with acute disseminated encephalomyelitis

Background: Acute disseminated encephalomyelitis (ADEM) is clinically characterized by the acute onset of neurological symptoms after a viral infection or immunization, and is thought to represent an autoimmune disease directed against myelin. Tau protein is a phosphorylated microtubule-associated protein, primarily located in neuronal axons. Increased levels of tau protein in cerebrospinal fluid (CSF) are found in various pathological conditions. Methods: We used tau protein as a marker of axonal damage and examined its concentration in the CSF of 27 children with ADEM. Results: CSF tau protein concentration in children with ADEM was significantly higher than that in the CSF of control subjects (P = 0.008). There were no significant differences in CSF tau protein concentrations in the ADEM patients with and without encephalopathy. The CSF tau protein concentration in patients with partial lesion resolution in follow-up brain MRI was significantly higher than in patients with complete lesion resolution (P = 0.014). Conclusions: In conclusion, we demonstrated that CSF tau protein concentration was significantly increased in ADEM patients. Our findings suggest that axonal damage may occur in addition to demyelination in children with ADEM.     

Acute disseminated encephalomyelitis in adults: a reappraisal of clinical, CSF, EEG, and MRI findings

Objectives To establish an adequate definition of acute disseminated encephalomyelitis (ADEM) in adults, based on our clinical observations of a case-series. Methods Over a period of three years 10 adult patients with a para- or postinfectious disseminated (diffuse or multifocal) syndrome of the CNS fulfilling predefined strict criteria for the diagnosis of ADEM were encountered and systematically followed. Results The age ranged from 21 to 62 years, two were men. MRI was normal in 5 patients and only mildly abnormal in the remaining patients. CSF was normal in 5 patients and mildly abnormal in the remainder, EEG was abnormal in 7/8 patients. All patients survived and were followed over a period of 30 months (range: 8 to 48 months). Nine patients were left with some residual defects, consisting most often of a mild cognitive impairment. Conclusions The EEG as an investigation of brain function can be crucial in establishing the organic nature of disease. MRI is important to exclude other diffuse or multifocal encephalopathies. However, in contrast to previous reports in the literature abnormal MRI should not be considered mandatory in adult ADEM. Difficulties in the diagnosis of ADEM are discussed and the importance of clinical and paraclinical findings for establishing the diagnosis is outlined. Received: 20 April 2001, Received in revised form: 19 July 2001, Accepted: 25 July 2001     

急性播散性脑脊髓炎的临床特点

目的探讨急性播散性脑脊髓炎（ADEM）的临床特点。方法对3例ADEM患者的临床表现、辅助检查、治疗及预后等进行回顾性分析。结果 3例ADEM中2例有前驱感染史,1例有手术史,首发症状分别为意识障碍、头晕和癫癇发作,头颅磁共振（MRI）见颅内多发长T1、长T2信号,呈环形或脑回样强化,累及双侧大脑、小脑和脑干,以双侧侧脑室旁和丘脑常见,经糖皮质激素治疗后病情迅速缓解。结论 ADEM是一种急性炎性脱髓鞘疾病,认识该病的临床及MRI特点可使患者得到及时诊治,改善预后。