儿童急性播散性脑脊髓炎16例临床及实验室分析

目的 分析儿童急性播散性脑脊髓炎的临床和实验室特点。方法 对1999年4月～2003年12月间住院的16例急性播散性脑脊髓炎患儿的临床表现、实验室检查及预后进行回顾性分析。结果 15例有前驱病史(93．75％)。7例为脑脊髓型,8例脑型,1例脊髓型。10例脑脊液常规、生化异常,10例IgG指数增高(66．67％),7例脑脊液寡克隆带(OB)阳性(46．67％)。11／15例MRI异常(73．33％),多灶性(72．72％);1例CT异常。15／16例脑电图异常(93．75％)。所有病例均予激素治疗,疗效显著。结论 急性播散性脑脊髓炎是中枢神经系统常见的炎性脱髓鞘病变,神经系统临床表现多样,脑脊液、MRI检查有助于诊断,激素治疗有效,预后良好。     

儿童急性播散性脑脊髓炎22例临床分析

目的　总结急性播散性脑脊髓炎 (ADEM)患儿的临床特征 ,以提高对此病的认识。方法　对 2 0 0 1年10月至 2 0 0 3年 10月中国医科大学第二临床学院儿科 2 2例ADEM患儿的临床表现、辅助检查及治疗资料进行回顾性分析。结果　 2 2例中有前驱感染史者 19例 ,疫苗接种史 1例 ,无明显诱因 2例。患儿均呈急性起病 ,3例呈亚急性进展。首发症状表现为肢体疼痛、麻木、活动障碍、嗜睡、阵发性头痛、呕吐及抽搐。临床主要表现为脑型7例 ,脑脊髓型 9例 ,脊髓型 6例。 16例患儿脑脊液蛋白和 (或 )细胞数升高。MRI表现为脑皮质下或脊髓T1加权像呈等、低混合信号 ,T2加权像呈不均质信号或高信号 ;增强后病灶无强化或条状强化灶。地塞米松、丙种球蛋白治疗ADEM有效 ,丙种球蛋白疗效更好。结论　ADEM是一种急性脱髓鞘性疾病 ,早期诊断、尽早合理选用激素、丙种球蛋白治疗可取得较好的疗效     

急性播散性脑脊髓炎的临床特征及实验室检查

目的探讨急性播散性脑脊髓炎(ADEM)患儿的临床和实验室特点。方法对22例有典型临床表现,MRI异常,通过激素治疗明显有效的ADEM患儿的临床表现、实验室检查及预后进行回顾性分析。结果有前驱病史14例(63.6%),以出疹性病毒感染为主;临床表现为共济失调17例(77.3%),意识障碍16例(72.7%);脑脊液异常11例,蛋白轻度升高9例,淋巴细胞升高2例。22例MRI均异常,中丘脑病变占40.9%。16/18例脑电图(EEG)异常(88.9%)。2/4例视神经诱导电位双侧异常。病例均予激素治疗,2例使用静脉丙种球蛋白。死亡率4.5%,后遗症发生率27.2%(6/22例)。结论ADEM临床表现多样,MRI检查对ADEM诊断具有特征性,激素治疗有效,严重病例常有后遗症。应结合病史、年龄、病理组织学、临床表现、MRI病灶位置、范围及形态、预后等与病毒性脑炎、MS加以鉴别。     

疫苗接种后急性播散性脑脊髓炎2例

例 1,男 ,1岁 5个月。注射流脑疫苗 3d后出现步态不稳 ,双手及头部震颤 ,双上肢不能准确抓物 ,双下肢站立不稳 ,不能行走。无发热、抽搐、意识障碍及大小便失禁。院外给予能量合剂静滴 7d无效 ,于发病d10入院。查体 :体温正常 ,神清 ,偶见眼球、头及双手轻微震颤 ,双下肢不能站立。上肢肌力正常 ,下肢肌力 3级 ,浅反射存在 ,腱反射亢进。脑电图及头颅CT检查正常。脑脊液检查 :压力 2 30mmH2 O ,无色透明 ,糖、蛋白、氯化物正常 ,血象、血电解质正常。诊断 :流脑疫苗接种后急性脑脊髓炎。静滴人血丙种球蛋白2 0 0mg/ (kg·d)× 5d ,地塞米…     

小儿急性播散性脑脊髓炎八例临床及预后分析

目的研究小儿急性播散性脑脊髓炎(ADEM)的临床特点,提高临床诊治水平。方法对8例临床初诊为ADEM的患儿进行回顾性及随访研究,对临床特点、治疗方案及预后进行分析。结果(1)临床表现:全部病例可询及病前感染史或疫苗接种史;病程均为单相;神经系统症状多样,其中包括不典型的双侧对称性弛缓性瘫痪症状,并可伴1~3周的发热。(2)辅助检查:头颅MRI检查异常,白质病变为主,灰质亦可受累。全部病例脑电图出现背景慢化,提示灰质受累;脑脊液可为轻微异常,亦可正常。(3)治疗和预后:6例予甲泼尼龙冲击,1例予地塞米松治疗,1例仅口服泼尼松,其中6例合用大剂量IVIG,全部患儿的神经系统症状明显缓解。(4)2个月~4年随访8例均未发现新病灶,3例遗留轻度后遗症。结论应注意ADEM的临床特点,及早诊治。糖皮质激素和IVIG治疗有效。需长期随访。     

儿童急性播散性脑脊髓炎24例临床分析

目的 研究儿童急性播散性脑脊髓炎(ADEM)的临床和实验室特点,以提高诊治水平.方法 对1998年5月-2008年12月收治的24例ADEM患儿的临床特点、实验室检查结果 、治疗方案及预后进行回顾性分析及随访研究.结果 14例有前驱感染病史,2例有疫苗接种史.神经系统症状多样.15/24例(62.5%)脑脊液常规、生化异常.20例行脑脊液寡克隆带及免疫指数检查,其中13例(65%)鞘内IgG合成增加、免疫球蛋白指数增高0.84～2.5;8例(40%)脑脊液寡克隆带(+).22例行头颅MRI检查,20例(90.91%)异常,以白质病变为主,呈多灶性.3例头颅CT未发现异常而MRI示阳性结果 .24例均行脑电图检查,20例(83.33%)异常,其中5例EEG见到棘慢波.24例均予激素治疗,14例同时合用大剂量人血丙种球蛋白,疗效显著,所有患儿均好转.经6个月～9年随访,2例确诊为多发性硬化,15例无复发,7例失随防.结论 ADEM是中枢神经系统常见的急性脱髓鞘性疾病,应及早诊治,脑脊液、MRI检查有助于早期诊断,糖皮质激素及大剂量丙种球蛋白有一定疗效,预后良好.     

急性播散性脑脊髓炎12例临床研究

目的　研究急性播散性脑脊髓炎 (ADEM )的临床特点 ,以提高其诊治水平。方法　对 12例临床初诊为ADEM患儿进行回顾及随访研究 ,并对临床特点、治疗方案及预后进行分析。结果　 1.临床表现 :75 %病前有前驱感染或疫苗接种史。 83%为单相性 ,17%为多相性病程。神经系统症状多样。 2 .辅助检查 :18%脑脊液常规、生化异常 ,5 6 %鞘内IgG合成率增高 ;头颅MRI均异常 ,以白质病变为主。电生理检查中以脑电图异常多见。 3.治疗及预后 :11例予甲泼尼龙冲击 ,5例同时合用大剂量人血丙种球蛋白 ,所有患儿均好转。 11例经3个月～ 6年随访 ,1例确诊为多发性硬化 ,10例无复发 ,其中 6例 (6 0 % )遗留后遗症。结论　ADEM具有区别于中枢神经系统感染的临床特点 ,致残率高 ,应及早诊治 ,糖皮质激素及大剂量丙种球蛋白有一定疗效。ADEM与多发性硬化的关系、如何改进治疗方法、改善预后尚需进一步研究     

儿童急性播散性脑脊髓炎临床特征及转归

目的探讨儿童急性播散性脑脊髓炎（ADEM）的临床经过及随访结果，以期在诊治水平上有所提高。方法对10例临床诊断为ADEM患儿进行为期6个月～3年的临床随访，对其临床经过、治疗及预后加以分析。结果学龄前及学龄儿童起病季节以春季为多见（占50％）；临床首发神经系统症状各有不同，运动障碍多见（40％）。80％为单相性病程。脑脊液常规、生化异常占40％，脑电图检查异常70％，头颅MRI均表现为颅内多发异常信号（T1低信号、T2及Flair像高信号）。全部病例均用糖皮质激素治疗，9例同时用大剂量人血丙种球蛋白静滴，临床均有显著好转。经6个月～3年的随访，2例有复发。全部病例均未留神经系统后遗症，1例发生继发性癫痫，用抗癫痫药物治疗后控制良好。结论ADEM早期应用糖皮质激素及大剂量丙种球蛋白治疗疗效显著，临床恢复良好。激素的给药剂量和持续时闯可能影响本病的预后。头颅MRI表现侧脑室扩大与颅内其他病灶的恢复的不一致性值得关注和进一步随访。     

儿童急性播散性脑脊髓炎14例临床分析

目的：急性播散性脑脊髓炎（ acute disseminated encephalomyelitis,ADEM）的早期诊断并积极治疗对疾病的预后影响很大,通过对ADEM病例的临床资料进行分析,提高对ADEM的认识,为早期诊断提供参考。方法参照2013年国际儿科多发性硬化研究组的 ADEM修订诊断标准,对2013年1月至2015年5月间在本院住院诊断为ADEM并至少随访3个月以上的病例进行再评价,对其中14例的临床症状、体征、实验室检查结果、影像学特征及诊断过程进行回顾性分析。结果14例诊断病例就诊时平均年龄（7.1±4.3）岁,男女比例10∶4。初发症状多种多样：惊厥发作4例,头痛4例,共济失调4例,运动障碍2例,视力改变2例,眼球运动障碍1例,眼睑下垂1例,意识改变1例。79.6％的病例首次中枢神经系统MRI显示只有脑内多发病变,21.4％显示同时存在脑及脊髓病变。57.1％的病例首次就诊诊断为其他疾病,其中诊断为脑炎3例,视神经炎、热性惊厥、颅内占位、重症肌无力、吉兰-巴雷综合征各1例。结论 ADEM容易诊断为具有相同症状的疾病,中枢神经系统影像学MRI检查是除外其他疾病、早期诊断ADEM最有效的手段。     

急性播散性脑脊髓炎68例临床特征分析及预后研究

目的 探讨儿童急性播散性脑脊髓炎(ADEM)的临床特征与预后.方法 分析2001年2月至2007年3月68例复旦大学附属儿科医院ADEM患JLO-院病史资料及近远期疗效随访.结果 发病年龄l岁至15岁4个月,中位数6.5岁;住院患儿逐年增加.有前驱感染史者占57.4%;预防接种史者占4.4%;38.2%无明确病因.临床分型:脑型42例(61.8%),脑脊髓型18例(26.4%),脊髓型8例(11.8%);主要症状表现发热、瘫痪、惊厥、精神异常、锥体外系症状、意识障碍、认知障碍、括约肌障碍、共济失调等,部分惠儿出现语言功能障碍、颅神经麻痹.脑脊液免疫指标检查57例,44例(77.2%)阳性[IgC指数增加和(或)寡克隆带阳性].皮质激素近期疗效:起效时间55.9%≤3d、26.5%≤7 d、17.6%>1周;好转时间48.5%≤1周;8例自然缓解,死亡1例;远期疗效:病程≥1 48例;病程<1年20例.随访结果:痊愈38例;后遗症9例(痛程1年以上尚未恢复者);7例复发;随访中8例;失访5例.结论 儿童ADEM多发生于3～12岁,临床特征复杂多样,病情轻重和病程长短不一;临床有多灶脑病的表现时应重视颅脑MRI检查以早期发现本病;多数远期预后良好,部分严重者完全恢复时间超过6个月,少数惠儿留有神经系统后遗症.     

Consecutive cerebral MRI findings of acute relapsing disseminated encephalomyelitis

A seven year old girl with acute relapsing disseminated encephalomyelitis (ARDEM) was observed with serial magnetic resonance imaging (MRI) examination. She had the first encephalomyelitis episode at the age of 2 years 9 months. She entered remission with steroid therapy. She suffered a recurrence at the age of 7. Steroid therapy was instituted and consecutive magnetic resonance imaging (MRI) was performed. In spite of rapid improvement of neurological abnormality with steroid and steroid pulse therapy, disseminated high intensity lesions in T₂-weighted imaging which were considered as demyelination remained for a long time. Magnetic resonance imaging seems to be most suitable for evaluating the clinical condition and long-term follow-up of ARDEM.     

Acute disseminated encephalomyelitis associated with Mycoplasma pneumoniae infection

An 8 year old girl with acute disseminated encephalomyelitis (ADEM) is described. Elevated serum antibody titers suggested recent Mycoplasma pneumoniae infection. T2-weighted image of magnetic resonance imaging (MRI) disclosed multiple lesions of high signal intensity in bilateral basal ganglia and thalami as well as in the white matter. Postcontrast T1-weighted image revealed an enhanced lesion in the deep white matter. She showed rapid clinical improvement in response to corticosteroid therapy. The lesions had disappeared completely on MRI performed 10 weeks after the onset. ADEM is believed to be a demyelinating disorder of probable autoimmune etiology. MRI findings in this case may support the hypothesis that the primary pathological event is vascular injury and demyelination occurs only as a secondary phenomenon.     

急性播散性脑脊髓炎与多发性硬化的诊断及鉴别诊断

目的 根据急性播散性脑脊髓炎（ＡＤＥＭ）和多发性硬化（ＭＳ）临床及实验室检查特点,探讨二者的诊断和鉴别诊断。方法 制定较严格的诊断标准,对符合标准的３４例ＡＤＭ和１７例ＭＳ的临床表现及实验室检查分析比较。结果 ＡＤＥＭ３４例,男１９例,女１５例;ＭＳ１７例,男７例,女１０例。ＡＤＥＭ与ＭＳ起病发热（６４．７％;２９．４％）;头痛（５８．８％;２３．５％）;意识障碍（６４．７％;５．９％）,其中昏迷     

Cyclosporine in the Treatment of a Case of Fulminant and Refractory Acute Disseminated Encephalomyelitis

Background

Acute disseminated encephalomyelitis (ADEM) is a rare, monophasic, demyelinating disease of the CNS which sometimes could be refractory to traditional treatment.

Case Presentation

We present a case of fulminant ADEM which is treated with combination of corticosteroid, intravenous immunoglobulin and cyclosporine.

Conclusion

Immunosuppressive agents such as cyclosporine may be effective especially in fulminant form of the disease.     

Acute disseminated encephalomyelitis: a review of 18 cases in childhood

OBJECTIVE: Acute disseminated encephalomyelitis (ADEM) is a treatable inflammatory demyelinating disorder seen more commonly in children than in adults. It typically presents to general paediatricians, often, like encephalitis, with non-specific cerebrospinal fluid findings. The brain computerized tomography scan is usually normal, so is falsely reassuring and delays the diagnosis, which might result in considerable morbidity. The present study was initiated to report on the various modes of presentation and raise the awareness of the diagnosis of ADEM among general paediatricians. METHODS: A retrospective review of the case notes of 18 children with a diagnosis of ADEM established in a tertiary referral centre from 1995 to 2000 was undertaken with particular reference to clinical features, investigations and treatment. RESULTS: The most common presenting features were ataxia (10 cases), followed by headache (eight cases) and weakness (five cases). Magnetic resonance imaging (MRI) of the brain was needed to confirm the diagnosis in all 18 children. Treatment usually included a course of intravenous methylprednisolone followed by a tapering dose of oral prednisolone over several weeks. Although the outcome for most of the children was generally good, two relapsed after cessation of steroids and five children had ongoing disabilities. CONCLUSIONS: The investigation of choice for establishing the diagnosis of ADEM was MRI of the brain. Other investigations were seldom helpful in reaching the diagnosis. Early diagnosis and prompt treatment of ADEM will probably reduce morbidity.     

血浆置换治疗儿童急性播散性脑脊髓炎2例报告并文献复习

目的探讨血浆置换治疗儿童急性播散性脑脊髓炎(ADEM)的疗效及安全性。方法回顾分析2例ADEM患儿的临床资料,并检索1998年1月至2018年1月万方和PubMed数据库的相关文献进行分析。结果 2例男性患儿,分别为5岁和2岁,临床有发热伴有抽搐、意识障碍等多种脑病表现;影像学提示白质多发病变,符合ADEM诊断标准。予大剂量激素、免疫球蛋白治疗后,病情仍进展,肌无力、意识障碍加重,出现呼吸衰竭。予呼吸机辅助呼吸、血浆置换(于病程2周左右)等,症状快速缓解,神志转清,肌力逐渐恢复,脱离呼吸机。分别随访1年及6个月,均未遗留神经系统症状。结论在ADEM患儿对大剂量激素及丙球治疗反应不佳且病情迅速进展时,血浆置换可作为挽救性治疗措施。     

Fulminant form of acute disseminated encephalomyelitis in a child treated with mild hypothermia

We describe the case of a 3‐year‐old boy diagnosed with the fulminant form of acute disseminated encephalomyelitis (ADEM). He developed general fatigue, fever, drowsiness and difficulty in walking. He had extensive multiple high‐intensity lesions in the white matter of the cerebrum and cerebellum, which are typical findings of ADEM. He became comatose and developed decerebrate rigidity with severe brain edema despite high‐dose methylprednisolone therapy, and then was subjected to mild hypothermia therapy, and given i.v. immunoglobulin. The patient recovered remarkably with the sequela of only mild action tremor. The patient was considered to have acute hemorrhagic leukoencephalitis (AHLE), an extremely severe form of ADEM, in terms of the rapidly deteriorating clinical course and neuroimaging features. It was speculated that AHLE and ADEM might be a continuous disease spectrum. It is considered that the severe brain edema associated with ADEM or AHLE is a suitable indication for mild hypothermia therapy.