摘要
由生后早期(3个月内)癫痫发作导致的严重精神运动发育迟缓可被诊断为早发性婴儿发育性癫痫性脑病(Early-infantile developmental and epileptic encephalopathies,EIDEE),其原发病因包括结构性、遗传性、代谢性等,主要的发病机制可能与大脑正常生理活动被异常电活动抑制和大脑神经网络受损有关。Ohtahara综合征和早期肌阵挛性脑病(Early myoclonic encephalopathy,EME)是EIDEE的典型类型。治疗原则主要是通过控制频繁的癫痫发作,进而改善患儿认知与发育功能。药物难以控制发作时应尽早行手术评估,对可以进行手术治疗的患者首选手术治疗,手术类型可分为切除/离断性手术、神经调控手术等。本文针对EIDEE的手术治疗现状进行叙述,探索手术治疗的疗效,以利于临床医师选择合适的治疗方式。
Severe psychomotor developmental delay resulting from early postnatal(within 3 months)seizures can be diagnosed as Early-Infantile Developmental and Epileptic encephalopathies(EIDEE).Its primary etiologies include structural,hereditary,metabolic and etc.The main pathogenesis may be related to the inhibition of normal physiological activity of the brain by abnormal electrical activity and the damage of the brain neural network.Ohtahara syndrome and Early Myoclonic Encephalopathy(EME)are typical types of EIDEE.The principle of treatment is to improve the cognitive and developmental function by controlling frequent seizures.When the seizure is difficult to control with drugs,surgical evaluation should be performed as soon as possible,and surgical treatment is the first choice for patients suitable for surgery.The types of surgery can be divided into excision surgery,dissociation surgery,neuromodulation surgery and etc.The current status of surgical treatment of EIDEE was described,and the curative effect of surgical treatment was explored,so as to help clinicians choose appropriate treatment methods.
作者
刘一迪
操德智
LIU Yidi;CAO Dezhi(Epilepsy Center,Shenzhen children’s Hospital,Shenzhen 518038,China)
出处
《癫痫杂志》
2023年第6期482-486,共5页
Journal of Epilepsy
基金
深圳市儿童医院高水平医院疑难疾病精准诊治攻关项目(LCYJ2022094)
广东省高水平医院建设专项经费资助。
关键词
早发性
发育性癫痫性脑病
发育迟缓
手术治疗
Early-infantile
Developmental and Epileptic Encephalopathies
Psychomotor developmental delay
Surgical treatment
作者简介
通信作者:操德智,Email:caodezhi888@aliyun.com。
