本文選題：局灶性皮質發(fā)育不良　切入點：藥物難治性癲癇　出處：《浙江大學》2014年博士論文

【摘要】：局灶性皮質發(fā)育不良(Focal cortical dysplasia, FCD)是皮層發(fā)育畸形(malformation of cortical development, MCD)的一種,是兒童難治性癲癇的最常見病因,是成人難治性癲癇的第二或者第三位病因,由于不少臨床難治性癲癇被定義為隱源性癲癇,其實際的發(fā)病率可能高于目前的估計。自David Taylor et al.1971報道以來,FCD分類、診斷、治療不斷有新的改進。 目的： 局灶性皮質發(fā)育不良臨床相對少見,但近年來國內報道亦有增多。本文報告兩例我院收治并手術病例,術后恢復良好。在此基礎上回顧分析了國內外報道,對其臨床表現(xiàn)、治療、預后做出分析總結,以期為局灶性皮質發(fā)育不良的臨床診斷、治療提供一定的線索。 臨床資料和方法： 分析了近期我院收治的兩名手術治療的FCD患者,包括其臨床表現(xiàn)、手術方式、避免功能區(qū)損害、術后隨訪等結果。 通過在線數(shù)據(jù)庫MEDLINE/PubMed、Web of Science和Cochrane library等英文數(shù)據(jù)庫及CNKI、維普、萬方等中文數(shù)據(jù)庫對以往國內外報道的進行搜索,關鍵詞包括“Focal cortical dysplasia"、"FCD"、“局灶性皮質發(fā)育不良”、“局灶性大腦皮質發(fā)育不良”,對近期FCD臨床研究予以分析總結,對FCD的流行病學、影像學表現(xiàn)、手術預后做歸納,以期有助于將來FCD及其相關癲癇的研究、診斷和治療。 分類方法：1、Palmini分類：僅存在皮質發(fā)育異常,無正常分層及組織結構為FCD Ⅰ型,在此基礎上存在異形神經元(Dysmorphic neuron)為FCD Ⅱa型,在此基礎上有氣球樣細胞為FCD Ⅱb型(Palmini et al.,2004)。2、ILAE新的分類方法：在Palmini分類的基礎上根據(jù)FCDⅠ型是否伴隨其他病理改變提出FCDⅢ型,其中FCDⅢa伴發(fā)海馬硬化,FCDⅢb伴發(fā)癲癇相關腫瘤,FCDⅢc伴發(fā)病灶附近血管畸形,FCDⅢd伴隨生命早期致癇灶(如外傷、缺血性損傷、腦炎等)(Blumcke et al.,2011; Gaitanis and Donahue,2013)。本文如無特殊說明則默認采用Palmini分類。詳細分類見后文討論。 男女差異對照采用中華人民共和國國家統(tǒng)計局2012年統(tǒng)計數(shù)據(jù),男女比例694：660。數(shù)據(jù)統(tǒng)計采用Fisher exact test雙尾,P0.05認為有統(tǒng)計學差異。 結果和結論： 我們報道兩例行手術治療的藥物難治性癲癇患者,術后病理證實皆為ILAE FCD Ⅱb型。兩例病灶皆位于額葉,術前MRI均有陽性表現(xiàn),1例靠近運動區(qū),1例靠近Broca區(qū),術后恢復良好,無功能損傷,隨訪癲癇無再發(fā),Engel分級Ⅰ級。 我們回顧國內外FCD相關報道,予以分析總結：FCD常表現(xiàn)為難治性癲癇,亦是我國藥物難治性癲癇的常見病因。藥物難治性癲癇手術治療的病人術后證實為FCD比例很高；表現(xiàn)為藥物難治性癲癇的FCD患者男性多于女性；FCD Ⅰ型多位于顳葉,FCD Ⅱ型各腦葉均有分布；患者MRI多有陽性表現(xiàn),不過影像表現(xiàn)缺乏特異性；手術治療效果較好,預后與FCD分型、病灶位置、是否影像學陽性及臨床表現(xiàn)密切相關,手術時間越早相對預后越好。
[Abstract]:Focal cortical dysplasia (FCDD) is a kind of cortical malformation of cortical development, which is the most common cause of intractable epilepsy in children and the second or third cause of intractable epilepsy in adults.Because many clinical refractory epilepsy are defined as cryptogenic epilepsy, the actual incidence may be higher than current estimates.Since the report of David Taylor et al.1971, new improvements have been made in the classification, diagnosis and treatment of FCD.Objective:Focal cortical dysplasia is relatively rare in clinical practice, but there are more reports in China in recent years.Two cases of surgical treatment in our hospital were reported.On this basis, the domestic and foreign reports were reviewed, and the clinical manifestations, treatment and prognosis were analyzed and summarized in order to provide some clues for the clinical diagnosis and treatment of focal cortical dysplasia.Clinical data and methods:Two patients with FCD treated by surgery in our hospital were analyzed, including their clinical manifestations, surgical methods, avoidance of damage to functional areas, postoperative follow-up, and so on.English and Chinese databases such as MEDLINE / PubMed Web of Science and Cochrane library, as well as Chinese databases such as CNKI, Weip and Wanfang, were used to search for previously reported reports at home and abroad, including "Focal cortical dysplasia", "FCD", "focal cortical dysplasia", etc."focal cerebral cortical dysplasia". The recent clinical studies of FCD were analyzed and summarized. The epidemiology, imaging manifestations and surgical prognosis of FCD were summarized in order to contribute to the study, diagnosis and treatment of FCD and its associated epilepsy in the future.Method: there were only cortical dysplasia, no normal stratification and no FCD type 鈪，

本文編號：1713042