21例Rasmussen脑炎的临床特征、治疗及预后

Clinical features,treatment and prognosis of Rasmussen encephalitis in 21 children

目的总结经手术治疗的Rasmussen脑炎(Rasmussen encephalitis,RE)的临床表型、电生理特点、影像学特点、治疗及预后,提高对此疾病的认识。方法回顾性分析2014年10月—2019年10月于北京大学第一医院儿童癫痫中心行半球离断术的21例RE患者资料。收集人口学数据、起病年龄、起病方式、癫痫发作形式、脑电图特点、头颅影像学特点、手术及预后。结果共收集21例患者,其中男8例、女13例,起病年龄(5.0±2.0)岁,手术时年龄(6.9±2.7)岁,手术时病程(1.7±1.3)年。20例(20/21,95.2%)患者以局灶运动性发作起病,10例(10/21,47.6%)患者有2~3种表现形式的局灶运动性发作。15例(15/21,71.4%)患者有部分性癫痫持续状态(Epilepsia partialis continua,EPC),出现于病程(0.7±0.6)年。所有患者均有偏瘫,偏瘫出现于(0.9±0.6)年。脑电图显示所有患者受累半球节律减慢,其中有4例患者健侧半球随着病情的进展节律亦减慢。所有患者发作间期均有受累半球侧癫痫样放电,其中6例患者健侧亦有独立的癫痫样放电。21例患者均行半球离断术。术后随访时间2~7年,所有患者均达到EngelⅠ级。神经功能损害均较术前有不同程度恢复。结论RE多发生于学龄期前后。局灶运动性发作是首发且最为常见的症状。随着病情的进展,发作形式呈现多样化,出现EPC、偏瘫及认知功能下降。脑电图以患侧半球慢波及放电为主要表现,虽然部分患者亦可双侧受累,但明显不对称。通过手术治疗,患者在癫痫发作及发育方面均获得了良好的疗效。

Objective To summarize the clinical phenotype,electrophysiological characteristics,imaging characteristics,surgical treatment and prognosis of Rasmussen encephalitis(RE),so as to deepen the understanding of the disease.Methods The clinical data of patients with RE who underwent surgical therapy from October 2014 to October2019 at Children’s Epilepsy Center in Peking University First Hospital were retrospectively reviewed.Demographic characteristics,seizure forms,electroencephalogram(EEG),cranial nuclear magnetic resonance(MRI),operative methods as well as surgical outcomes evaluated by Engel classification during follow-up of the subjects were collected and analyzed.Results Totally 21 pediatric patients were enrolled,including 8 males and 13 females.The age at onset was(5.0±2.0)years old,the age at the time of surgery was(6.9±2.7)years old,and the disease duration at the time of surgery was(1.7±1.3)years.Twenty(20/21,95.2%)patients had focal motor seizures,and 10(10/21,47.6%)patients had 2 or 3 forms of focal motor seizures.Fifteen patients(15/21,71.4%)had epilepsia partialis continua(EPC),which occurred(0.7±0.6)years after the onset.All patients had hemiplegia,which appeared at(0.9±0.6)years after the onset.All patients showed a slow rhythm at their affected hemispheres during the EEG monitoring and 4 of them also showed slow rhythm at the contralateral hemispheres as the disease progressed.All patients had epileptiform discharges at the involved hemisphere,and 6 patients also had independent epileptiform discharges on the contralateral side.All 21 patients underwent hemispheric disconnection.The duration of follow-up was 2 to 7 years,and all patients achieved Engel class I after the surgery.The neurological dysfunction recovered to varying degrees during the postoperative period.Conclusion RE mostly occurs around the school age.Focal motor seizures are the main manifestations and the most common onset symptoms.With the progress of the disease,the condition of patients worsened gradually.The EEG of patients was mainly characterized by broad slow wave and spike wave in the affected hemisphere.Some patients can also have bilateral involvement,which was obviously asymmetrical.Through surgical treatment,the patients all achieved good results in terms of seizures and development.