多脑叶皮层切除治疗Sturge—Weber综合征继发癫痫一例及文献复习

Surgical treatment for epilepsy due to Sturge - Weber syndrome： a case report and review of the literature

目的探讨Sturge—Weber综合征继发癫痫和发育迟滞的手术治疗的方法和疗效。方法回顾性分析2007年11月手术治疗的Sturge—Weber综合征继发癫痫和智力、体格等多方面发育迟滞的1例患儿的临床资料。进行术前评估后，在全麻下行“右侧半球开颅，右侧额、顶叶和部分颞、枕叶脑组织皮层切除术”。结果术后当天患儿左侧肢体肌力为Ⅰ级，但3d后左侧肢体肌力开始逐渐恢复，至术后21d，左侧肢体肌力恢复至术前水平。随访18个月，患儿癫痫发作完全消失，并于术后12个月时自行停用抗癫痫药物。左侧肢体肌力达到Ⅴ^-级，功能好于术前，左手手指活动好转，能握住钢笔等物体。语言功能显著好转。攻击行为消失，性格较温顺，认知和行为能力均有明显好转。发育状况评估DQ值从术前的48改善为75。结论Sturge—Weber综合征继发癫痫和发育迟滞可行病变多脑叶切除性生手术治疗，不仅可以控制癫痫，还可能改善发育。

Objective To explore the strategy and its effect of surgical treatment of epilepsy and development retardation due to Sturge - Weber syndrome. Methods The clinical data of an infant surgically treated for epilepsy, mental and physical development retardation due to Sturge - Weber syndrome were retrospectively reviewed. After sufficient preoperative evaluation, the operation was performed through the hemisphere craniectomy, and the right total parietal lobe, partial frontal temporal and occipital lobes were removed. Results The muscle force of left limbs was grade I post operation, but they recovered gradually after 3 days, and recovered to preoperative level after 3 weeks. During the 18 months interval of follow - up the patient was seizure free, her muscle force of right upper limb improved to grade Ⅴ^- , left fingers movement improved significantly, aggressive behaviour disappeared, and DQ improved from preoperative 48 to 75. Conclusion Epilepsy and development retardation due to Sturge - Weber syndrome can be treated by resection the cortex of involved multiple brain lobes.