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小儿局灶性皮质发育不良 II 型的临床特征和手术结果。

Clinical features and surgical outcomes in young children with focal cortical dysplasia type II.

机构信息

Department of Pediatrics, Peking University First Hospital, Beijing, China.

Children Epilepsy Center, Peking University First Hospital, Beijing, China.

出版信息

CNS Neurosci Ther. 2020 Feb;26(2):270-277. doi: 10.1111/cns.13205. Epub 2019 Aug 1.

Abstract

AIMS

To investigate clinical characteristics and surgery outcomes of young children with focal cortical dysplasia (FCD) type II.

METHODS

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.

RESULTS

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.

CONCLUSION

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.

摘要

目的

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。

方法

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。

结果

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。

结论

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efdb/6978260/44b0cb21814f/CNS-26-270-g001.jpg

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