14例小儿EB病毒脑炎临床分析

Clinical study of children with Epstein-barr virus encephalitis

目的探讨EB病毒(EBV)脑炎的临床表现、发病机制、诊断方法及预后。方法回顾性分析2006年7月至2009年10月我院收治的14例EBV脑炎临床特点。结果 14例患儿脑脊液和血清EBVCA(衣壳抗原)-IgM均阳性。其中6例进行脑脊液EBVDNA检测,3例阳性。2例具有传染性单核细胞增多症的典型症状,淋巴结及肝肿大2例,单纯淋巴结肿大3例,脾肿大1例,皮疹3例。14例均有发热,抽搐7例,嗜睡7例,昏迷1例,共济失调及构音障碍1例;2例出现EBV相关噬血细胞综合征。8例脑电图异常。7例头颅MRI异常,病变包括额叶、颞叶、丘脑、基底节、小脑等部位,3例诊断中枢神经系统脱髓鞘病变。14例给予抗病毒治疗。5例(包括3例脱髓鞘病及2例噬血细胞综合征)给予糖皮质激素治疗。出院后随访6个月至1年,12例痊愈,存在基底节及丘脑病变的2例患儿出现后遗症,其中1例遗留癫痫,1例左侧肢体活动障碍。结论 EBV脑炎可以缺乏传染性单核细胞增多症的典型症状,临床表现多样,临床上对合并淋巴结肿大、肝脾肿大、皮疹等表现的病毒性脑炎患儿,以及传染性单核细胞增多症治疗中出现神经系统症状的患儿,要警惕EBV脑炎可能。EBV脑炎可累及额叶、颞叶、丘脑、基底节、小脑等部位,亦可出现中枢神经系统脱髓鞘病变。大多数EBV脑炎预后良好,存在基底节及丘脑病变的患儿易出现后遗症。

Objective To summarize the clinical characteristics,pathogenesis,diagnostic methods and prognosis of Epstein-barr virus（EBV） encephalitis.Methods Fourteen EBV encephalitis patients registrered in Beijing Children Hospital from Jul 2006 to Sep 2009 were studied retrospenively.Results Fourteen patients had evidence of convincing EBV serology and positive cerebrospinal fluid（CSF） EB VCA-IgM.CSF EBV PCR was done in 6 patients,3 of whom had positive results.Two cases manifested typical features of infectious mononucleosis（IM）.Two cases had lymphadenectasis and hepatomegaly;three cases had lymphadenectasis;only one case had splenomegaly;three cases had rash.Fourteen cases had fever;seven cases had seizures;senven cases had lethargy;one case had coma;one case had ataxia and dysarthria.Two cases had EBV associated hemophagocytic syndrome.Seven cases had abnormalities on MRI;Demyelination disease was diagnosed in 3 cases.Fourteen cases were treated with antiviral drugs.Corticosteroids were used in 5 cases.Followed up study was done within 6~12 months.Twelve cases had full recovery;two patients with thalamus or basal ganglia involvement developed sequela.Conclusions Features of IM might be absent in patients with EBV encephalitis.The clinical manifestations were varied.For those viral encephalitis cases with lymphadenectasis,splenohepatomegalia and rashes,or patients with IM developed manifestations such as headache,vomiting,EBV encephalitis should be suspected.EBV encephalitis involvements were quite diverse,including frontal lobe,temporal lobe,thalamus,basal ganglia and cerebellum.It may have demyelination as well.Majority of patients with EBV encephalitis had favorable prognosis,while patients with thalamus or basal ganglia involvement were more likely to suffer from neurologic sequelae.