难治性癫痫相关Sturge-Weber综合征7例临床及病理分析

doi:10.6040/j.issn.1671-7554.0.2016.666

摘要/Abstract

摘要： 目的分析难治性癫痫相关脑面血管瘤病(SWS)患者的临床特征及手术后标本的病理学特点。方法回顾性分析接受脑病灶及致痫灶切除手术的7例SWS患者的临床及病理资料。7例患者中男4例,女3例,手术年龄2~20岁,发病年龄1~12个月。6例为多脑叶切除,1例为功能性大脑半球切除。结果脑标本病理可见软脑膜血管瘤病,脑组织中灰质钙化较弥漫、显著,脑实质内绝大多数小血管壁出现管壁全周的钙化,并伴发局灶性皮质发育不良(FCD)Ⅲc型。术后随访1~7年,EngelⅠ级3例,EngelⅡ级2例,Engel Ⅲ级1例,Engel Ⅳ级1例。结论 SWS患者发病早、病程长、病理改变有特征性,病变典型的患儿早期接受癫痫外科手术后效果良好,病变不典型的患者在成年后接受相同的手术也能达到良好的效果。

关键词: Sturge-Weber综合征, 神经外科手术, 皮质发育畸形, 软脑膜血管瘤, 癫痫

Abstract: Objective To investigate the clinicopathologic features of the brain tissues from the patients with refractory epilepsy related Sturge-Weber syndrome(SWS). Methods The clinical and pathologic data were reviewed in 7 SWS patients who underwent epilepsy operation. Seven patients including 4 males and 3 females had been diagnosed SWS. The age of patients for surgery was 2-20 years old. The age of patients when seizure onset was 1-12 months. Six cases accepted multilobar resettion, and 1 case accepted functional hemispherectomy. Results The pathological diagnoses were meningioangiomatosis, remarkable calcification in the brains gray matter and small blood vessels, focal cortical dysplasia(FCD)Ⅲc. Seizure outcome after the epilepsy operation revealed 3 patients had an Engel grade I, 2 patients had an Engel grade Ⅱ, 1 patient had an Engel grade Ⅲ, and 1 patient had an Engel grade Ⅳ. Conclusion SWS patients had earlier onset, longer course and characteristic pathological change. Children with typical symptom should accept operation as soon as possible. Atypical patients also had good prognosis after operation.

Key words: Sturge-Weber syndrome, Neurosurgery, Malformations of cortical development, Leptomeningeal angiomatosis, Epilepsy

中图分类号:

R741.02

李岩,郭珺,王荣祺,刘永玲. 难治性癫痫相关Sturge-Weber综合征7例临床及病理分析[J]. 山东大学学报（医学版）, 2017, 55(4): 91-95.

LI Yan, GUO Jun, WANG Rongqi, LIU Yongling. Sturge-Weber syndrome in patients with intractable epilepsy: a clinicopathologic study of 7 cases[J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2017, 55(4): 91-95.

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