摘要
目的分析不同类型皮质发育障碍(malformation of cortical dysplasia,MCD)所致儿童癫痫的临床特征、术前评估和预后,探讨不同类型儿童MCD的外科干预策略。方法回顾性分析我中心采用外科手术治疗176例MCD伴儿童癫痫的临床资料,致痫灶定位情况和外科手术结果,并对不同类型MCD的结果进行比较分析。结果 MCD占同期儿童癫痫病因的34.2%。术后疗效按Engel分级,Ⅰ级106例,Ⅱ级31例,Ⅲ级21例,Ⅳ级14例,有效率89.8%,优良率77.8%,平均智商(IQ)从术前的66.5分提高到72.9分。在不同类型MCD中,半巨脑、结节性硬化、局灶性皮质发育障碍、无/巨脑回畸形、灰质异位和多小脑回的术后癫痫控制优良率依次为100%、90.9%、76.8%、75.0%、66.7%和62.5%,Ⅱ型局灶性皮质发育障碍(focal cortical dysplasia,FCD)疗效优于Ⅰ型FCD(84.2%vs 70.5%),合并肿瘤的FCD的优于单纯的FCD(86.4%vs 76.6%),伴海马硬化的患儿疗效较佳(85.7%vs 76.7%)。智力评估结果显示,FCD和结节性硬化患儿均得到显著提高,其他类型智力改善不明显。结论不同类型MCD具有不同的临床特点、影像学特点、电生理特点、功能学和外科预后,因此需要重视针对不同类型MCD,进行差异化、个体化的外科评估程序和手术处理。
Objective To investigate the clinical characters,preoperative evaluation,surgical procedures and outcomes of pediatric intractable epilepsy patients with malformation of cortical development（MCD）.Methods The clinical data,epilepsy focus localization and surgical outcomes of 176 pediatric patients with MCD between 2005-2010 were retrospectively analyzed.The results of different subtypes of MCD were compared.Results MCD was the most important pathology of pediatric intractable epilepsy（34.2%）.The postoperative follow-up on average of 3.3 years showed that 106 patients（60.2%） had an Engel Class Ⅰ outcome,31 patients（17.6%） had rare seizure（Engel Class Ⅱ）,21 patients had a decrease in seizure frequency（Engel Class Ⅲ）,and 14 patients had an Engel Class Ⅳ outcome.The mean intelligence quotient（IQ） improved from 66.5 to 72.9.The excellent rates of surgical outcomes in different subtypes of MCD were 100% in hemimegalencephaly,90.9% in tuberous sclerosis（TS）,76.8% in focal cortical dysplasia（FCD）,75.0% in pachygyria/agyria,66.7% in gray matter heterotopia and 62.5% in polymicrogyria.The outcomes of the patients with subtype Ⅱ FCD were better than those of the patients with subtype Ⅰ FCD（84.2% vs 70.5%）,the outcomes of the FCD patients complicated with tumor were better than those of the patients with FCD alone（86.4% vs 76.6%）,and the outcomes of the FCD patients complicated with hippocampal sclerosis were better than those of the FCD patients without hippocampal sclerosis（85.7% vs 76.7%）.Significant improvement of IQ was shown in patients with FCD and TS as compared with other patients.Conclusion Different subtypes of MCD show distinct characteristics in clinical symptoms,imaging,neurophysiology,brain function and surgical outcome,and individual preoperative evaluation and different surgical strategies should be conducted for different subtypes of MCD.
出处
《第三军医大学学报》
CAS
CSCD
北大核心
2012年第22期2244-2248,共5页
Journal of Third Military Medical University
关键词
儿童难治性癫痫
外科治疗
皮质发育障碍
局灶性皮质发育障碍
结节性硬化
pediatric intractable epilepsy
surgery
malformation of cortical development
focal cortical dysplasia
tuberous sclerosis
