难治性癫痫46例患者大脑半球或多脑叶切除标本的临床病理学分析  

A clinicopathologic analysis of specimens of hemispherectomy or multilobar resection from 46 cases of refractory epilepsy

作　　者：王跃峰[1] 朴月善[1] 卢德宏[1] 王玮[1] 陈莉[1] 隗立峰[1] 杨虹[1] 

机构地区：[1]首都医科大学宣武医院病理科,北京100053

出　　处：《中华神经科杂志》2011年第1期24-29,共6页Chinese Journal of Neurology

基　　金：基金项目：北京市科技新星计划资助项目（2006863）;首都医学发展科研基金资助项目（2007-2070）

摘　　要：目的研究难治性癫痫患者大脑半球或多脑叶切除标本的临床病理学特点。方法对2005--2009年在首都医科大学宣武医院接受大脑半球（行功能性大脑半球切除术）或多脑叶切除的46例难治性癫痫患者临床病理学资料进行回顾性分析。结果46例患者的平均发病年龄3．9岁，平均病程为10．2年。其中行大脑半球切除33例，多脑叶切除13例，且颞叶全部受累。组织学分型：继发性瘢痕脑回31例，皮质发育畸形7例，中枢神经系统感染8例。31例瘢痕脑回标本镜下可见皮质结构消失、神经元减少、反应性胶质细胞增生及淀粉样小体出现；此外还可观察到含铁血黄素（13例）、钙化（9例）以及岛状分布的神经元（5例）。瘢痕脑回均伴不同程度的皮质发育不良，并有7例伴海马硬化。7例脑回发育畸形中，局灶性皮质发育不良（FCD）5例（其中FCDIB型3例，FCDIA型1例，FCDⅡA型1例），多小脑回畸形及穿通脑畸形各1例。8例中枢神经系统感染中Rasmussen脑炎5例、巨细胞病毒性脑炎1例、结核性脑膜炎1例、囊虫感染1例。结论大脑半球或多脑叶切除的脑组织病理学主要是多种原因（比如外伤、缺氧等）引起的继发性瘢痕脑回，颞叶病变明显。中枢神经系统感染以Rasmussen脑炎为主，皮质发育畸形主要以FCDIB型为主。Objective To investigate the clinicopathologic features of the brain tissue from multilobar resection or hemispherectomy for refractory epilepsy. Methods The clinical and pathologic findings of 46 cases seen at Xuanwu hospital from 2005 to 2009 were reviewed retrospectively. Results The mean age of seizure onset and disease duration were 3.9 years and 10. 2 years, respectively. There were 33 cases of hemispherectomy and 13 cases of muhilobar resection. Temporal lobe abnormality was seen in all cases. The pathologic subgroups were as follows： ulegyria （31/46）, malformation of cortical development （MCD, 7/46） and infection （8/46）. Microscopic examination of ulegyria showed cortical architectural disturbances, neuronal loss, reactive gliosis and appearance of corpora amylacea. We also noted deposition of hemosiderin （ 13 cases）, calcification （9 cases ） and island-like neurons （5 cases）. All ulegyria cases were accompanied by varying degree of cortical dysplasia, and hippocampus sclerosis were identified in 7 cases. MCD comprised of 5 cases of focal cortical dysplasia （FCD） , including 3 cases of FCD I B, 1 case of FCD Ⅱ A and 1 case of FCD I A, 1 case of polymicrogyria and 1 case of porencephaly. Among 8 infection cases, there were 5 cases of Rasmussen encephalitis （ RE）, 1 case of cysticercosis, 1 case of tuberculous meningitis and I case of Cytomegalovirus encephalitis. Conclusions The most common pathological category of specimens from hemispherectomy or muhilobar resection is ulegyria with obvious temporal lobe abnormality. This is followed by MCD （with FCD I B as the main type） and central nervous system infection （RE as the most frequent abnormality）.

关 键 词：癫痫 大脑 瘢痕 皮质发育畸形 脑炎 

分 类 号：R742.1[医药卫生—神经病学与精神病学]