发笑性癫痫的研究进展
The research advancements in gelastic epilepsy
林媛 1谢凌辉 1邱建敏2
作者信息
- 1. 福建医科大学临床医学部(福州 350001)
- 2. 福建医科大学临床医学部(福州 350001);福建省莆田市第一医院神经内科(莆田 351100)
- 折叠
摘要
发笑性癫痫(Gelastic seizure,GS)是一种不合时宜的以爆发性发笑为主要临床特点,伴或不伴其他癫痫性发作事件的癫痫发作类型,按症状出现时间、有无情绪改变及意识障碍情况分为单纯性和非单纯性发笑发作.笑的产生的神经通路主要分为情感和意志两大通路,其神经网络涉及了额叶、岛叶、边缘叶、颞叶、脑干等相关神经结构.GS最常见的病因为下丘脑错构瘤,立体定向脑电图可以记录到病变本身的放电,手术切除下丘脑错构瘤可以使大多数患者GS发作立即消失,少数患者症状得到部分改善其头皮脑电图仍可监测到癫痫样放电,尽早手术治疗可能改善预后.非下丘脑源性GS,影像学检查无明显异常者,脑电图多为局灶性放电并且对抗癫痫发作药物治疗效果较好,而影像学提示结构异常的患者多为多灶性放电且对药物治疗效果较差,少数药物难治性非下丘脑源性的GS手术治疗可以有效控制症状.本文对GS这一个较为少见的癫痫发作类型的病因、涉及的神经网络、脑电特点、治疗选择进行综述,以期提高对该病的认识.
Abstract
Gelastic seizure(GS)is a type of epilepsy characterized primarily by inappropriate bursts of laughter,with or without other epileptic events.Based on the timing of symptoms,the presence of emotional changes,and disturbances of consciousness,GS is classified into simple and complex types.The generation of laughter involves two major neural pathways:the emotional pathway and the volitional pathway.The neural network involved in GS includes structures such as the frontal lobe,insula,cingulate gyrus,temporal lobe,and brainstem.The most common cause of GS is a hypothalamic hamartoma,and stereotactic electroencephalography can record discharges from the lesion itself.Surgical removal of the hypothalamic hamartoma can result in immediate cessation of GS in the majority of patients,while some may experience partial improvement with persistent epileptic-like discharges detectable on scalp electroencephalography(EEG).Early surgical intervention may improve prognosis.In cases of non-hypothalamic origin of GS with no apparent imaging abnormalities,focal discharges are often observed on EEG and these cases respond well to antiepileptic drugs.Conversely,patients with structural abnormalities suggested by imaging studies tend to have multifocal discharges and a poorer response to medication.In a small subset of medically refractory non-hypothalamic GS,surgical intervention can effectively control symptoms.This article provides a comprehensive review of the etiology,neural networks involved,EEG characteristics,and treatment options for GS,with the goal of improving understanding of this relatively rare type of epileptic seizure.
关键词
癫痫/发笑性癫痫/脑电图/病理性的笑/笑的神经网络Key words
Epilepsy/Gelastic seizure/Electroencephalogram/Pathological laughter/Neural pathways in laughter引用本文复制引用
出版年
2024