自身免疫性癫痫的临床特征及预后

Autoimmune epilepsy:clinical features and prognosis

目的探讨自身免疫性癫痫(AE)的临床特征及免疫调节治疗的效果。方法回顾性分析5例AE患儿的临床资料,总结患儿的临床表现、辅助检查结果、治疗效果及随访复发情况。结果 5例患儿均急性起病,均有前驱感染病史,以频繁抽搐为主要临床表现,4例伴有精神症状或情绪改变。5例患儿头颅MRI均未见异常,外周血及脑脊液AE相关抗体检测均阴性,但4例患儿脑脊液白细胞轻度升高。5例患儿脑电图可见不同脑区出现尖波和尖慢波,其中5例脑电图背景均呈现慢波。给予常规抗癫痫药物治疗后抽搐难以控制,给予免疫调节治疗后癫痫均得以控制。随访5个月,有2例患儿复发。结论对于耐药性癫痫患儿,如存在精神、情绪、性格以及脑脊液炎性改变,即使AE抗体检测及MRI影像学正常,仍需考虑AE的可能性。抗癫痫药物联合免疫调节治疗AE效果佳,但可出现复发。

Objective To explore the clinical features of autoimmune epilepsy（ AE） as well as the efficacy of immunoregulation therapy for AE. Methods The clinical data of 5 AE children were retrospectively analyzed,and the clinical manifestations,results of auxiliary examination,therapeutic effects,and recurrence during follow-up were summarized. Results The 5 children suffered from acute attack of AE and experienced prodromic infection,with repeated hyperspasmia as common clinical symptom,among which 4 were complicated with psychiatric symptom or emotional changes. The results of head MRI revealed that no abnormality was found in the 5 cases,the AE-associated antibodies in peripheral blood and cerebrospinal fluid were negative,but slightly elevated WBC was observed in cerebrospinal fluid. The findings of electroencephalogram showed that sharp wave and sharp-slow wave were seen in various encephalic regions among the 5 cases,including 5 cases presented slow wave in electroencephalogram background. The routine therapy of anti-epilepsy drugs failed in controlling hyperspasmia,however,immunoregulation therapy achieved successful hyperspasmia control. During a 5-month follow-up,recurrent AE occurred in 2 cases. Conclusion For children with drug-resistant epilepsy,those who suffer the changes of psychology,emotion,personality as well as the inflammatory changes of cerebrospinal fluid,even in the case of having normal AE antibodies and MRI images,should still consider their risk of AE. Anti-epilepsy drugs combined with immunoregulation therapy have a favorable efficacy,but with a possibility of recurrent AE.