局灶性皮质发育不良临床表现及其诊治（2例病例报告及文献回顾）

发布时间：2018-04-05 03:36

  本文选题：局灶性皮质发育不良　切入点：药物难治性癫痫　出处：《浙江大学》2014年博士论文

【摘要】：局灶性皮质发育不良(Focal cortical dysplasia, FCD)是皮层发育畸形(malformation of cortical development, MCD)的一种,是儿童难治性癫痫的最常见病因,是成人难治性癫痫的第二或者第三位病因,由于不少临床难治性癫痫被定义为隐源性癫痫,其实际的发病率可能高于目前的估计。自David Taylor et al.1971报道以来,FCD分类、诊断、治疗不断有新的改进。 目的： 局灶性皮质发育不良临床相对少见,但近年来国内报道亦有增多。本文报告两例我院收治并手术病例,术后恢复良好。在此基础上回顾分析了国内外报道,对其临床表现、治疗、预后做出分析总结,以期为局灶性皮质发育不良的临床诊断、治疗提供一定的线索。 临床资料和方法： 分析了近期我院收治的两名手术治疗的FCD患者,包括其临床表现、手术方式、避免功能区损害、术后随访等结果。 通过在线数据库MEDLINE/PubMed、Web of Science和Cochrane library等英文数据库及CNKI、维普、万方等中文数据库对以往国内外报道的进行搜索,关键词包括“Focal cortical dysplasia"、"FCD"、“局灶性皮质发育不良”、“局灶性大脑皮质发育不良”,对近期FCD临床研究予以分析总结,对FCD的流行病学、影像学表现、手术预后做归纳,以期有助于将来FCD及其相关癫痫的研究、诊断和治疗。 分类方法：1、Palmini分类：仅存在皮质发育异常,无正常分层及组织结构为FCD Ⅰ型,在此基础上存在异形神经元(Dysmorphic neuron)为FCD Ⅱa型,在此基础上有气球样细胞为FCD Ⅱb型(Palmini et al.,2004)。2、ILAE新的分类方法：在Palmini分类的基础上根据FCDⅠ型是否伴随其他病理改变提出FCDⅢ型,其中FCDⅢa伴发海马硬化,FCDⅢb伴发癫痫相关肿瘤,FCDⅢc伴发病灶附近血管畸形,FCDⅢd伴随生命早期致痫灶(如外伤、缺血性损伤、脑炎等)(Blumcke et al.,2011; Gaitanis and Donahue,2013)。本文如无特殊说明则默认采用Palmini分类。详细分类见后文讨论。 男女差异对照采用中华人民共和国国家统计局2012年统计数据,男女比例694：660。数据统计采用Fisher exact test双尾,P0.05认为有统计学差异。 结果和结论： 我们报道两例行手术治疗的药物难治性癫痫患者,术后病理证实皆为ILAE FCD Ⅱb型。两例病灶皆位于额叶,术前MRI均有阳性表现,1例靠近运动区,1例靠近Broca区,术后恢复良好,无功能损伤,随访癫痫无再发,Engel分级Ⅰ级。 我们回顾国内外FCD相关报道,予以分析总结：FCD常表现为难治性癫痫,亦是我国药物难治性癫痫的常见病因。药物难治性癫痫手术治疗的病人术后证实为FCD比例很高；表现为药物难治性癫痫的FCD患者男性多于女性；FCD Ⅰ型多位于颞叶,FCD Ⅱ型各脑叶均有分布；患者MRI多有阳性表现,不过影像表现缺乏特异性；手术治疗效果较好,预后与FCD分型、病灶位置、是否影像学阳性及临床表现密切相关,手术时间越早相对预后越好。
[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，

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