摘要
目的探讨Sturge-Weber综合征继发药物难治性癫痫的手术治疗方法和疗效。方法回顾性连续纳入2014年9月至2018年9月首都医科大学宣武医院神经外科手术治疗的Sturge-Weber综合征继发药物难治性癫痫患者4例(男3例,女1例)。术前均行MRI、CT及视频脑电图检查,评估患者颅内病变范围;术中应用皮质脑电图监测确定异常放电范围,手术方式包括大脑半球切除、多脑叶皮质切除或局灶性皮质切除联合皮质电凝热灼;肢体功能采用英国医学研究委员会肌力评级进行评定(0~Ⅴ级);术后定期复查头部MRI、CT及视频脑电图,随访采用Engel分级评估癫痫控制情况,并复习相关文献,分析手术治疗的适应证、手术方式和治疗效果。结果4例Sturge-Weber综合征患者均有颜面部血管瘤,术前均有药物难治性癫痫,伴有不同程度的偏瘫、言语障碍及认知功能减退等神经功能缺损症状;截至末次随访时间为12~55个月,Engel分级Ⅰ级3例,Ⅳ级1例。术后无出血、感染、梗阻性脑积水等手术并发症,2例术后出现受累侧肢体肌力下降1级,1例术后受累侧肢体肌力增加1级,1例术后肢体功能无明显变化。结论结合文献回顾,外科手术是治疗Sturge-Weber综合征继发药物难治性癫痫安全、有效的方法。根据患者的年龄、神经功能和病变程度,选择合适的术式能显著改善患者预后。
Objective To explore the surgical method and efficacy in patients with drug-refractory epilepsy secondary to Sturge-weber syndrome.Methods Four patients(3 males and 1 female)with drug-refractory epilepsy secondary to Sturge-Weber syndrome were continuously enrolled in the Neurosurgery Department of Xuanwu Hospital,Capital Medical University from September 2014 to September 2018 for surgical treatment.Magnetic resonance imaging(MRI),computed tomography(CT)and video electroencephalogram(EEG)were used to evaluate the extent of intracranial lesions before surgery.The electrocortical electroencephalography(ECoG)monitoring was used to determine the range of epileptic discharge during operation.The surgical methods included hemispherectomy,multi-lobular resection,or focal cortex resection combined with the thermal aspiration of focal cortex.Motor function was evaluated referred to the Medical Research Council muscle strength grading(grade 0 toⅤ).MRI,CT and video EEG were timely performed after operation.Seizure outcome was accessed referred to Engel classification.With a review of these cases and the literature,we explored the surgical indications,surgical methods and treatment effects.Results Four patients who had port-wine stains on the face were diagnosed as Sturge-Weber syndrome accompanying drug-refractory epilepsy as well as various degrees of hemiplegia,speech disorder and cognitive dysfunction.The follow-up time was 12 to 55 months.Three cases were Engel gradeⅠ,1 case was Engel gradeⅣ.There were no complications such as hemorrhage,infection and obstructive hydrocephalus after the operation.The muscle strength of the involving limb was decreased by 1 grade in 2 patients and increased by 1 grade in another patient.The motor function of the other one patient was stable.Conclusions Given these cases and the literature,surgery is a safe and effective method for the treatment of drug-refractory epilepsy secondary to Sturge-Weber syndrome.The appropriate surgery could significantly improve the prognosis of patien
作者
李沐阳
张华强
安阳
魏鹏虎
单永治
赵国光
Li Muyang;Zhang Huaqiang;An Yang;Wei Penghu;Shan Yongzhi;Zhao Guoguang(Department of Neurosurgery,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)
出处
《中国脑血管病杂志》
CAS
CSCD
北大核心
2020年第1期44-48,共5页
Chinese Journal of Cerebrovascular Diseases
基金
国家重点研发计划数字诊疗装备研发重点专项(2106YFC0103909)
2016年市科委“首都临床特色应用研究”专项课题(Z161100000516008)
国家自然科学基金资助项目(81871009)
