EB病毒感染后眼阵挛-肌阵挛-共济失调综合征一例并文献复习  

Opsoclonus-myoclonus-ataxia syndrome caused by the infection of Epstein-Barr virus:A case report and review of the literature

作　　者：丁朋 陈国娟 相蕾[3] 张隽 崔菀真 李文霞[1] 岳伟[3] DING Peng;CHEN Guojuan;XIANG Lei;ZHANG Jun;CUI Wanzhen;LI Wenxia;YUE Wei(不详;Department of Neurology,Clinical College of Neurology,Neurosurgery,and Neurorehabilitation,Tianjin Medical University,Tianjin Huanhu Hospital,Tianjin 300000,China)

机构地区：[1]天津医科大学神经内外科及神经康复临床学院,300000 [2]天津市人民医院神经内科,300121 [3]天津市环湖医院神经内科,天津市脑血管与神经变性重点实验室,300350 [4]唐山市工人医院神经内二科,063000 [5]广州中医药大学第二临床医学院,510006

出　　处：《中国神经免疫学和神经病学杂志》2024年第4期265-270,共6页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的探讨眼阵挛-肌阵挛-共济失调综合征(opsoclonus-myoclonus-ataxia syndrome,OMAS)与EB病毒(Epstein-Barr virus,EBV)感染的关系、治疗及预后。方法报告1例OMAS患者的临床表现、辅助检查、治疗及预后,并对相关文献进行回顾分析,总结EBV感染后OMAS病例特点。结果在中英文数据库中共检索出6篇符合要求文献中报道的7例患者,合并本文报告病例,共8例患者纳入分析。8例患者中男女各4例,发病年龄20个月至71岁,4例为儿童。5例患者在出现OMAS前约10 d至2周出现一系列前驱症状,如发热、呕吐、咽喉痛、皮疹以及淋巴结肿大。8例患者中仅1例在病程中无明显的眼阵挛症状,其余7例均出现眼阵挛-肌阵挛-共济失调三联征。全部8例患者中,4例患者血清中病毒衣壳抗原(VCA)IgM阳性,2例患者早期抗原(EA)IgG阳性,1例患者行PCR检测到EBV DNA。6例患者行脑脊液检查,其中1例患者EA IgG阳性,1例患者行PCR检测到EBV DNA,6例患者脑脊液检查糖均在正常范围,2例患者出现蛋白升高〔(0.53~0.73)g/L〕,3例出现白细胞升高〔(11~20)×106/L〕。8例患者均行头部CT和(或)MRI检查,2例行全身PET-CT检查,均无阳性发现。8例患者中,甲泼尼龙治疗3例,免疫球蛋白治疗2例,促肾上腺皮质激素治疗1例及未予治疗2例,所有患者均在发病2~11周完全恢复,接受促肾上腺皮质激素治疗的1例患儿(年龄20个月,未行全身PET-CT检查)在发病5个月后发现神经母细胞瘤。结论EBV感染后可继发OMAS。结合前驱症状及针对EBV进行特异性的血清学及脑脊液检查有助于明确病因。免疫抑制治疗对EBV感染后OMAS有效。EBV感染后OMAS一般预后良好。Objective To explore the relationship,treatment and prognosis of opsoclonus-myoclonus-ataxia syndrome(OMAS)with Epstein-Barr virus(EBV)infection.Methods The clinical manifestations,auxiliary examinations,treatment and prognosis of one patient with OMAS were reported,and the relevant literature was reviewed and analysed to summarize the characteristics of OMAS cases after EBV infection.Results A total of 7 patients reported in 6 eligible publications were retrieved from Chinese and English databases,and 8 patients were included in the analysis when combined with the case reported here.Four of the 8 patients were of each sex,with onset ages ranging from 20 months to 71 years,and 4 were children.Five patients presented with a series of prodromal symptoms,such as fever,vomit,sore throat,rash,and lymph node enlargement,about 10 d to 2 weeks before the onset of OMAS.Only one of the eight patients did not have significant ophthalmoplegia during the course of the disease,and the remaining seven patients had the ophthalmoplegia-myoclonus-ataxia triad.Of the 8 patients,4 had positive serum viral capsid antigen(VCA)IgM,2 had positive early antigen(EA)IgG,and 1 had EBV DNA by PCR.Six patients underwent cerebrospinal fluid(CSF)examinations,of which 1 had positive EA IgG,1 had EBV DNA by PCR,and 6 showed normal sugar level.Protein was elevated in 2 patients[(0.53-0.73)g/L],and leukocytes were elevated in 3 patients[(11-20)×106/L].Eight patients underwent head CT and/or MRI,and 2 patients underwent whole-body PET-CT,with no positive findings.Of the 8 patients,3 were treated with methylprednisolone,2 with immunoglobulin,1 with adrenocorticotropic hormone,and 2 without treatment.All patients recovered completely within 2-11 weeks after onset.Neuroblastoma was found 5 months after the onset of illness in one patient(20 months of age,without whole-body PET-CT)who was treated with adrenocorticotropic hormone.Conclusions OMAS can occur secondary to EBV infection,and imaging studies are usually normal in patients in the acute phase.T

关 键 词：眼阵挛-肌阵挛-共济失调综合征 EB病毒 脑炎 

分 类 号：R741.02[医药卫生—神经病学与精神病学]