按照ILAE分类标准回顾性分析440例难治性癫痫病理学特征

Retrospectively analysis on the clinicopathologic features of intractable epilepsy of 440 cases according to the ILAE classification standard

目的探讨难治性癫痫相关局灶性皮质发育不良的临床病理特征。方法收集2016年1月—2018年12月北京丰台医院神经外科功能组临床诊断为癫痫、经药物治疗无效且手术切除致痫灶440例患者的临床资料,按照2011年ILAE分类标准回顾性分析其临床病理特征。结果440例患者平均发病年龄11.975岁,平均病程9.0192年,发作形式以复杂部分型发作为主。病理组织分型:轻微皮质发育不良15例(3.4%),局灶性皮质发育不良369例(83.86%),及其他56例(单纯性海马硬化23例、囊肿8例、结节性硬化5例、巨脑回多小脑回畸形2例、灰质异位7例、错构瘤2例,无显著病理改变9例)。FCD中,FCDⅠ型49例(FCDⅠa型14例,FCDⅠb型30例,FCDⅠc型5例),FCDⅡ型94例(FCDⅡa型43例,FCDⅡb型51例),FCDⅢ型226例(FCDⅢa型61例,FCDⅢb型111例,FCDⅢc型17例,FCDⅢd型37例)。结论局灶性皮质发育不良(FCD)是难治性癫痫的一个重要的致病原因,其中以FCDⅢb型最为多见,其次是FCDⅢa型及FCDⅡ型。2011年ILAE分类标准能更清晰反映出难治性癫痫的致病原因,进而可以更好的指导临床治疗及评估预后。

Objective To investigate the clinicopathologic characteristics of focal cortical dysplasia(FCD)in intractable epilepsy.Methods The clinical data of 440 patients those diagnosed as epilepsy and have undergone epilepsy surgery in Beijing Fengtai hospital between January 2016 and December 2018 due to ineffective drug therapy were collected.According to the classification criteria proposed by the International League Against Epilepsy(ILAE),the clinicopathologic characteristics were reviewed retrospectively.Results The mean age of epilepsy onset was 11.975 years,mean duration of epilepsy was 9.0192 years.The disease often presented as complex partial seizures.The histopathology types were identified:mild cortical malformation in 15 cases(3.4%),focal cortical dysplasia in 369 cases(83.86%),and other types in 56 cases(including 23 pure hippocampal sclerosis,8 cyst,5 tuberous sclerosis complex,2 cases of giant gyrus multiple cerebellar gyrus malformation,7 gray matter heterotopia,2 hamartoma and 9 without significant pathological changes).Among FCD cases:FCD type I were 49 cases(FCDIa:14,FCDIb:30,FCDIc:5),FCD typeⅡwere 94 cases(FCDⅡa:43,FCDⅡb:51),and FCD typeⅢwere 226 cases(FCDⅢa:61,FCDⅢb:111,FCDⅢc:17,FCDⅢd:37).Conclusions FCD is a common cause of the intractable epilepsy.FCD typeⅢb is the most common pathologic sub-type causing intractable epilepsy,the followed sub-types are FCD typeⅢa and FCD typeⅡ.The etiology of the intractable epilepsy could be distinguished according to the classification criteria of ILAE,and well guide the treatment and estimate the prognosis.