本文關(guān)鍵詞： Sturge-Weber綜合征 癲癇 神經(jīng)外科手術(shù) 皮質(zhì)發(fā)育畸形 軟腦膜血管瘤　出處：《山東大學(xué)學(xué)報(bào)(醫(yī)學(xué)版)》2017年04期 　論文類型：期刊論文

【摘要】：目的分析難治性癲癇相關(guān)腦面血管瘤病(SWS)患者的臨床特征及手術(shù)后標(biāo)本的病理學(xué)特點(diǎn)。方法回顧性分析接受腦病灶及致癇灶切除手術(shù)的7例SWS患者的臨床及病理資料。7例患者中男4例,女3例,手術(shù)年齡2~20歲,發(fā)病年齡1~12個(gè)月。6例為多腦葉切除,1例為功能性大腦半球切除。結(jié)果腦標(biāo)本病理可見軟腦膜血管瘤病,腦組織中灰質(zhì)鈣化較彌漫、顯著,腦實(shí)質(zhì)內(nèi)絕大多數(shù)小血管壁出現(xiàn)管壁全周的鈣化,并伴發(fā)局灶性皮質(zhì)發(fā)育不良(FCD)Ⅲc型。術(shù)后隨訪1~7年,EngelⅠ級(jí)3例,EngelⅡ級(jí)2例,EngelⅢ級(jí)1例,EngelⅣ級(jí)1例。結(jié)論 SWS患者發(fā)病早、病程長、病理改變有特征性,病變典型的患兒早期接受癲癇外科手術(shù)后效果良好,病變不典型的患者在成年后接受相同的手術(shù)也能達(dá)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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