儿童中央区局灶性皮质发育不良继发癫痫的临床特点和手术疗效  

Clinical characteristic and surgical outcomes of children＇s epilepsy caused by rolandic focal cortical dysplasia

作　　者：张诏[1] 马延山 李俊驹[3] 胡文瀚[4] 莫嘉杰 张建国[1,4] 张凯[1,4] Zhang Chao;Ma Yanshan;Li Junju;Hu Wenhan;Mo Jiajie;Zhang Jianguo;Zhang Kai(Department of Functional Neurosurgery,Beijing Tiantan Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京天坛医院功能神经外科,100050 [2]北京大学第一医院丰台医院神经外科 [3]海南省人民医院神经外科 [4]首都医科大学、北京市神经外科研究所

出　　处：《中华神经外科杂志》2018年第9期873-877,共5页Chinese Journal of Neurosurgery

基　　金：首都卫生发展科研专项（2016-1-1071）;海南省应用技术研发与示范推广专项（ZDXM2015068）;首都医科大学基础-临床科研合作课题（17JL05）;北京市委组织部北京市优秀人才项目（2016000021469G214）;中国抗癫痫协会UCB基金（2017002）

摘　　要：目的探讨儿童中央区局灶性皮质发育不良（FCD）继发癫痫的临床特点和手术疗效。方法回顾性纳入2015年5月至2017年6月首都医科大学附属北京天坛医院功能神经外科癫痫中心收治的8例中央区FCD继发癫痫的患儿。术前对所有患儿行头颅MRI、正电子发射断层显像术（PET）-CT和视频脑电图检查，其中3例患儿接受立体定向脑电图（SEEG）电极植入并行1—7d的长程视频脑电监测。根据患者的癫痫症状学、影像学和头皮或颅内脑电图结果定位致痫灶，行手术切除。术后对所有患儿行门诊或电话随访，采用Engel分级评估手术疗效，同时评估患儿的肢体肌力情况。结果8例患儿中，2例致痫灶的切除范围局限于中央区，其余6例累及中央区周围结构，包括盖部、辅助运动区、运动前区和岛叶。术后病理学提示：FCD Ⅱ型7例，FCDⅢd型1例。8例患儿的随访时间为0．3～2．5年，中位时间为0．6年。Engel分级均为Ⅰ级。术后1周内7例患儿出现不同程度的对侧肢体肌力下降，最严重的可降至0级。直至末次随访，7例患儿的肢体肌力均有不同程度的改善。结论儿童中央区FCD继发癫痫的症状复杂，切除性手术后预后良好，术后短期肌力下降，之后肌力会逐渐恢复。此类患儿早期手术可能获益更大。Objective To investigate the clinical features, evaluation methods and value of resective surgery in children with epilepsy caused by rolandic focal cortical dysplasia （FCD）. Methods Eight patients with epilepsy related to rolandic FCD were admitted to Epileptic Center, Department of Functional Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from May 2015 to June 2017 and enrolled into this retrospective study. All patients underwent head MRI, positron emission tomography （PET）-CT and video-EEG. Three patients underwent stereo-electroencephalography （SEEG） electrode implantation and EEG monitoring lasting for 1 - 7 days. According to the patient＇s epileptic symptoms, imaging, and scalp or intracranial EEG resuhs, the epileptogenic zone was identified and surgical resection was performed. All patients were followed up through outpatient visits or telephone. The Engel scale was used to evaluate the surgical outcome and the limb strength was also assessed. Results Among 8 patients, the epileptogenic zone was confined to the central area in 2 and surrounding areas of the central area in the other 6 cases, which included the operculum, secondary motor area, premotor area and insula. Postoperative pathological findings showed FCD type Ⅱ in 7 and FCD type Ⅲ d in 1. Image post-processing techniques and SEEG techniques facilitated the determination of epileptogenic zone. The follow-up duration for the 8 patients was 0.3 - 2.5 years, with the median of 0.6 year. All patients showed epilepsy remission （Engel Ⅰ ）. At 1 week post surgery, 7 patients had varying degrees of decline in contralateral limb muscle strength and grade 0 was reported in the most severe case. Until the last follow-up, the limb muscle strength of 7 patients improved with varying degrees. Conclusions Children with rolandic FCD have complicated epilepsy symptoms. The prognosis could be satisfactory following resection surgery. The muscle strength might decrease during a short term post operation and gradually rec

关 键 词：癫痫 儿童 随访研究 预后 局灶性皮质发育不良 

分 类 号：R742.1[医药卫生—神经病学与精神病学]