手术治疗不伴面部血管瘤的Sturge-Weber综合征1例

Surgical resection of epileptogenic focus in Sturge-Weber syndrome without facial hemangioma:case report

目的总结不伴面部血管瘤的致癎性Sturge-Weber综合征的诊治经验。方法报告1例女性病人,9岁,表现为药物难治性癫癎1年,发作形式为微笑-意识丧失-倒地抽搐,不伴面部和全身血管瘤。MRI显示:左侧顶枕交界区皮质病变,T1W呈等信号,增强后病变沿脑回强化;PET显示:病变及周围葡萄糖代谢降低,病变呈"电车轨道样"钙化。在神经导航和术中皮质脑电图(ECoG)监测下行左侧顶枕叶致癎灶切除术。结果病理报告为软脑膜血管瘤。随访11个月,病人无癫癎发作,无严重手术并发症发生。结论应加强对不伴面部血管痣性Sturge-Weber综合征的认识。手术切除致癎灶是治疗致癎性Sturge-Weber综合征的有效方法。

Objective To summarize the surgical experience with epileptogenic focus in Sturge-Weber syndrome without facial hemangioma stains. Methods A 9-year-old girl with Sturge-Weber syndrome without facial hemangioma was reported. She suffered from intractable epilepsy for one year, and clinical manifestations included epileptic seizure with smile, loss of consciousness and convulsions, without facial or whole-body hemangiomas. MRI demonstrated a lesion was located in the junction of left parietal and occipital lobe, isointense on T^W and enhanced along with the involved gyrus after gadolinium administration. Decreased glucose metabolism around the lesion and ＂tram-track＂ like calcification were observed by PET-CT scan. The epileptogenic focus was resected under intra-operative ECoG monitoring and neuronavigation. Results The diagnosis of leptomeningeal angioma was confirmed by pathological examination. During a follow-up of 11 months, the patient became seizure free and no severe complications occurred. Conclusion Recognition of Sturge-Weber syndrome without a facial hemangioma should be strengthened. Resection of the epileptogenic focus is a safe and effective treatment for refractory epilepsy in Sturge-Weber syndrome.