难治性癫痫相关Sturge-Weber综合征7例临床及病理分析

发布时间：2018-02-11 18:18

  本文关键词： Sturge-Weber综合征 癫痫 神经外科手术 皮质发育畸形 软脑膜血管瘤　出处：《山东大学学报(医学版)》2017年04期 　论文类型：期刊论文

【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,EngelⅢ级1例,EngelⅣ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。
[Abstract]:Objective to analyze the clinical and pathological features of patients with intractable epilepsy associated with facial hemangiomatosis (SWS). Methods the clinical and pathological features of 7 patients with SWS undergoing resection of brain lesions and epileptiform lesions were retrospectively analyzed. Data of 7 cases: male 4 cases, The operative age was 20 years old and the age of onset was from 1 to 12 months. 6 cases were treated with multilobar resection and 1 case with functional hemispheric resection. Results pial hemangiomatosis was found in brain specimens, and the calcification of gray matter in brain tissue was diffuse and significant. Most of the small vascular walls in the cerebral parenchyma had perivascular wall calcification, accompanied by focal cortical dysplasia (FCDIII) type 鈪，

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