儿童光敏性枕叶癫痫的电临床特征与治疗分析

Electroclinical characteristics and treatment analysis of photosensitive occipital lobe epilepsy in children

目的探讨光敏性枕叶癫痫(POLE)的临床及脑电图特征。方法收集2006年1月至2019年12月北京大学第一医院儿科22例POLE的患儿资料,对相关临床资料、脑电图、治疗和预后进行回顾性分析。结果22例患儿中,女12例,男10例;3例合并轻度智力发育落后。癫痫发作症状中,仅3例主诉有视觉先兆的主观症状,常见的症状为眼部运动(11例次)及呕吐(6例次)、头痛(3例次)、头晕(3例次)等客观症状。病程中均有局灶性发作,20例有继发双侧强直-阵挛发作,5例兼有全面性肌阵挛发作。22例患儿脑电图均监测到发作间期癫痫样放电,其中单纯局灶性放电6例,单纯广泛性放电10例,广泛及局灶性放电共存6例;19例诱发了光阵发反应,其中单纯局灶性放电4例,单纯广泛性放电6例,局灶及广泛性放电共存9例;16例诱发光惊厥反应,其中15例为枕叶起始局灶性发作或局灶继发双侧强直-阵挛发作,另1例仅诱发肌阵挛发作。18例使用了抗癫痫发作药物(ASMs)治疗,并进行了随访,常用的前3位药物为丙戊酸(12例)、左乙拉西坦(8例)、拉莫三嗪(4例),13例癫痫发作得到了控制。结论POLE发作期的视觉先兆不显著,临床医师应有意识地询问日常生活中癫痫发作与光刺激的关系,避免该综合征的误诊或漏诊。POLE脑电图常出现局灶性放电和广泛性放电共存,且可伴随全面发作,在治疗过程中选择ASMs时应兼顾共存现象,慎重选择钠离子通道阻滞剂。

Objective To investigate the clinical and electroencephalographic characteristics of photosensitive occipital lobe epilepsy(POLE)in children.Methods The clinical data,electroencephalogram(EEG),treatment and prognosis of 22 children with POLE in the Department of Pediatrics,Peking University First Hospital from January 2006 to December 2019 were retrospectively analyzed.Results Among the 22 patients,12 cases were female and 10 cases were male.There were 3 cases combined with mild mental retardation.Classified by seizure symptoms,only 3 patients complained of subjective symptoms of visual aura,and the common symptoms were eye movement in 11 patients,headache in 3 patients,vomiting in 6 patients and dizziness in 3 patients.All patients had focal seizures during the course of disease.Twenty patients had secondary bilateral tonic-clonic seizures,and 5 patients also had generalized myoclonic seizures.Interictal epileptiform discharges were monitored in EEG of all children,including pure focal discharges in 6 patients,pure generalized discharges in 10 patients,and coexistence of generalized and focal discharges in 6 patients.Photoparoxysmal responses were induced in 19 patients,including pure focal discharges in 4 patients,pure generalized discharges in 6 patients,and coexisting focal and generalized discharges in 9 patients.Photoconvulsive responses were induced in 16 patients,including focal seizures with occipital lobe onset or focal secondary to bilateral tonic-clonic seizures in 15 patients,and myoclonic seizures in 1 patient.Eighteen patients were treated with anti-seizure medications(ASMs)and followed up.The top 3 commonly used drugs were Valproic acid(12 patients),Levetiracetam(8 patients),and Lamotrigine(4 patients),and 13 cases had controlled seizures.Conclusions The visual aura of POLE is not obvious,and the relationship between epilepticseizures and light stimuli in daily life should be actively inquired to avoid misdiagnosis or underdiagnosis of the syndrome.The EEG of POLE often visualizes the coexistence of focal and generalized discharges,which may be accompanied by generalized seizures.The coexistence phenomenon should be considered when ASMs are medicated during treatment,and odium channel blockers should be selected carefully.