822例手术治疗难治性癫痫相关的病理学特征回顾分析  

Neuropathologic findings in intractable epilepsy： a clinicopathologic analysis of 822 cases

作　　者：段泽君[1] 姚坤[1] 周健[2] 李霖 翟锋[2] 刘长青[2] 马忠 边宇 栾国明[2] 齐雪岭[1] Duan Zejun Yao Kun Zhou Jian Li Lin Zhai Feng Lia Changqing Ma Zhong Bian Yu Luan Guoming Qi Xueling(Department of Pathology, Belting Key Lab of Epilepsy Research, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China)

机构地区：[1]首都医科大学三博脑科医院病理科,北京100093 [2]首都医科大学三博脑科医院神经外科一病区癫痫病临床医学研究北京市重点实验室,北京100093

出　　处：《中华病理学杂志》2017年第10期673-678,共6页Chinese Journal of Pathology

摘　　要：目的探讨难治性癫痫病例相关的临床病理学特征。方法按照2011年International League Against Epilepsy（ILAE）分类标准对2008年6月至2012年12月在首都医科大学三博脑科医院功能神经外科接受致痫灶手术的822例患者标本进行病理学特征回顾分析。结果822例患者平均发病年龄9．9岁，平均病程11．9年。癫痫发作形式以复杂部分性发作为主；病理组织学发现轻微皮层发育不良33例（4．01％）、局灶性皮层发育不良（FCD）690例（83．94％）及其他99例（单纯海马硬化39例、囊肿20例、Sturge—Weber综合征19例、结节硬化8例、无显著病理变化6例、脑回畸形5例、错构瘤2例）。FCD中，Ⅰ型106例，Ⅱ型91例，Ⅲ型493例（111a型160例、11Ib型106例、Ⅲc型26例、Ⅲd型201例）。结论FCDIIId型是引起难治性癫痫最多的病理类型，其最主要病因是由于围产期缺氧／缺血导致局部脑组织瘢痕形成；其次是单纯FCD（I型和Ⅱ型）；FCDⅢa型和FCDⅢb型分别位居其后。将单纯FCD与FCDⅢ型分开，并将FCDⅢ型进一步细分，更好地定义了大脑皮层分层紊乱，更加清晰直观地反映出难治性癫痫的致病因素，为进一步诊疗提供参考。Objective To investigate the clinicopathologic characteristics of intractable epilepsy. Methods Based on the classification criteria proposed by the International League Against Epilepsy （ILAE）, a retrospective analysis of the pathological characteristics was done in 822 patients who underwent epilepsy surgery in Sanbo Brain Hospital, Capital Medical University, from June 2008 to December 2012. Results The mean age of epilepsy onset was 9. 9 years, mean duration of epilepsy was 11.9 years. Complex partial seizures were the main presenting features. Histopathological study showed 33 cases （4.01%） with mild forms of cortical malformations, 690 cases （83.94%） with focal cortical dysplasia （FCD） and 99 cases with others （including 39 pure hippocampal sclerosis, 20 cystosclerosis, 19 Sturge-Weber syndrome, 8 tuberous sclerosis complex, 6 without significant pathological changes, 5 gyral malformations and ：2 hamartoma）. Among the 690 FCD cases, 106 were FCD typeⅠ , 91 were FCD type Ⅱ and 493 were FCDm（ Ilia. 160, rob.106, Ⅲc：26 and Ⅲd： 201）. Conclusions FCDⅢd is the most common histopathological subtype causing intractable epilepsy, mainly due to focal hypoxia/ischemia in the perinatal period, which results in scarring of local brain tissue; this is followed by other isolated forms of FCD （ FCD Ⅰand FCD Ⅱ ）, and then FCD m a and FCD Ⅲ b. The reason to distinguish isolated forms of FCD （types I and 11 ） from FCD m and to snbclassify FCD m is to allow better definition of cortical dyslamination. Therefore, the pathogenic factors of intractable epilepsy can be grouped in greater details, and facilitate the diagnosis and potential curative treatment of intractable epilepsy.

关 键 词：癫痫 复杂部分性 皮质发育畸形 疾病特征 诊断 

分 类 号：R651.1[医药卫生—外科学]