57例局灶性皮质发育不良所致癫痫患儿的临床分析

Clinical analysis of 57 children with epilepsy caused by focal cortical dysplasia

目的总结局灶性皮质发育不良(FCD)所致癫痫患儿的临床特征,并初步筛选影响外科手术后患儿癫痫发作控制效果的相关因素。方法选择郑州大学第三附属医院神经外科自2019年7月至2023年11月手术治疗的57例FCD所致癫痫患儿为研究对象。所有患儿均进行规范的癫痫术前评估、外科手术、术后管理及随访。回顾性总结患儿的一般资料、影像学资料、视频脑电图资料、手术方式、病理资料及随访资料等,并初步分析外科手术后患儿癫痫发作控制效果的影响因素。结果57例患儿中男29例(50.88%),女28例(49.12%);首次癫痫发作年龄为30.00(8.00,74.50)个月,外科手术年龄为95.00(50.00,138.50)个月;42例(73.68%)的癫痫发作类型为局灶性泛化至全面性强直-阵挛发作,13例(22.81%)为癫痫性痉挛发作。34例(59.65%)患儿头颅MRI阳性,常见表现为脑沟/脑回形态异常(17/57,29.82%)。接受PET-CT检查的43例患儿中40例(93.02%)检测出低代谢部位,40例(93.02%)的PET/MRI融合显像上病灶范围与实际手术范围一致。FCD病理分型Ⅰ型16例(28.07%)、Ⅱ型39例(68.42%)、Ⅲ型2例(3.51%)。截至2023年12月,44例(77.19%)患儿的预后为EngelⅠ级、4例(7.02%)为Ⅱ级、4例(7.02%)为Ⅲ级、5例(8.77%)为Ⅳ级。预后良好(EngelⅠ+Ⅱ级)与预后不良(EngelⅢ+Ⅳ级)患儿在首次癫痫发作至外科手术时间、MRI阳性或阴性、术后是否规律服用抗癫痫发作药物等方面的差异均有统计学意义(P<0.05)。结论局灶性泛化至全面性强直-阵挛发作是FCD所致癫痫患儿最常见的癫痫发作类型,脑沟/脑回形态异常是其最常见的MRI阳性表现,PET/MRI融合显像在致痫灶确定方面具有较大优势。首次癫痫发作至外科手术时间长、头颅MRI阴性及术后不规律服用抗癫痫药物患儿术后癫痫发作控制效果较差。

Objective To summarize the clinical characteristics of patients with epilepsy caused by focal cortical dysplasia(FCD),and identify the influencing factors for postoperative seizure controls.Methods Fifty-seven patients with epilepsy caused by FCD admitted to Department of Neurosurgery,Third Affiliated Hospital of Zhengzhou University from July 2019 to November 2023 were chosen;standard preoperative evaluation,surgery,postoperative management and follow-up were performed.A retrospective study of clinical data,imaging and video electroencephalogram(VEEG)data,surgical approaches,pathological findings,and follow-up data was performed;influencing factors for postoperative seizure controls were analyzed.Results In these 57 patients with epilepsy caused by FCD,29 were males(50.88%)and 28 were females(49.12%).Onset age was 30.00(8.00,74.50)months,and surgery age was 95.00(50.00,138.50)months.Focal to bilateral tonic-clonic seizures(42/57;73.68%)and epileptic spasms(13/57;22.81%)were common seizure types.Cranial MRI was positive in 34 patients(59.65%),mainly manifested as abnormal cortical gyri/sulci morphology(17/57;29.82%).In 43 patients accepted PET-CT,hypometabolic sites were detected in 40(93.02%),and complete agreement between PET/MRI fusion results and actual lesion sites was noted in 40(93.02%).FCD type I was noted in 16 patients(28.07%),type II in 39(68.42%),and type III in 2(3.51%).By December 2023,44(77.19%)had Engel grading I,4(7.02%)had grading II,4(7.02%)had grading III,and 5(8.77%)had grading IV.Children with good prognosis(Engel grading I+II)and those with poor prognosis(Engel grading III+IV)showed significant differences in terms of time from first seizure to surgery,positive/negative MRI,and regularity of postoperative ASMs(P<0.05).Conclusions Focal to bilateral tonic-clonic seizure is the most common seizure type in patients with epilepsy caused by FCD,and abnormal cortical gyri/sulci morphology is the most common MRI manifestation;PET/MRI fusion imaging is superior to PET-CT or MRI in identifying epileptogenic foci.Poor seizure control can be noted in patients with long onset time to surgery,with negative cranial MRI results,or with irregular postoperative ASMs.