Department of Pediatrics, Peking University First Hospital, Beijing, China.
Children Epilepsy Center, Peking University First Hospital, Beijing, China.
CNS Neurosci Ther. 2020 Feb;26(2):270-277. doi: 10.1111/cns.13205. Epub 2019 Aug 1.
To investigate clinical characteristics and surgery outcomes of young children with focal cortical dysplasia (FCD) type II.
Young children (onset age ≤6 years) with FCDII who underwent epileptic surgery in Children Epilepsy Center of Peking University First Hospital in 2014-2018 were followed up for at least 6 months after surgery.
One hundred and twelve children with FCDII were included, with median age of onset 0.9 years (0.01-5.9), who underwent surgery at 4.1 years old (0.8-16.2). Focal seizures were most frequent (90.2%) and epileptic spasms presented in 23 (20.5%) cases. Epileptic encephalopathy was not uncommon (12.5%), associated with earlier epilepsy onset and higher rate of bilateral onset on ictal EEG (OR = 0.213, 9.059; P = .041, .004). At the last follow-up, 88.4% achieved seizure-free. Before surgery, 49.1% showed moderate/severe developmental delay, associated with earlier seizure onset and higher rate of history of epileptic encephalopathy (OR = 0.740, 5.160, P = .023, .042). For 48 children with preoperatively moderate/severe developmental delay, DQ rank at 6 months postsurgery was improved in only four cases.
For young children with FCDII, they tend to present with epileptic encephalopathies and show moderate/severe developmental delay before surgery. The seizure outcome was favorable after surgery. For children with preoperatively moderate/severe developmental delay, developmental outcome at 6 months after surgery was not satisfactory.
研究儿童局灶性皮质发育不良(FCD)Ⅱ型的临床特征和手术治疗效果。
回顾性分析 2014 年至 2018 年于北京大学第一医院小儿癫痫中心行手术治疗的 FCDⅡ型患儿的临床资料,入组标准为发病年龄≤6 岁。
共纳入 112 例 FCDⅡ型患儿,发病年龄中位数为 0.9 岁(0.01-5.9 岁),手术年龄中位数为 4.1 岁(0.8-16.2 岁)。最常见的发作类型是局灶性发作(90.2%),23 例(20.5%)患儿出现痉挛发作。癫痫脑病并不少见(12.5%),癫痫脑病患儿的发病年龄更早,发作间期脑电图双侧起源比例更高(OR=0.213,9.059;P=0.041,P=0.004)。末次随访时,88.4%的患儿达到无发作。术前 49.1%的患儿存在中重度发育迟缓,癫痫脑病患儿的发病年龄更早,有癫痫脑病病史的患儿比例更高(OR=0.740,5.160,P=0.023,P=0.042)。48 例术前中重度发育迟缓的患儿中,仅 4 例在术后 6 个月时的 DQ 评分有所提高。
儿童 FCDⅡ型常伴有癫痫脑病,术前存在中重度发育迟缓。手术治疗后发作控制效果良好,但对于术前存在中重度发育迟缓的患儿,术后 6 个月的发育结局并不理想。