难治性癫痫273例的临床病理学分析  

Refractory epilepsy： a clinicopathologic study of 273 consecutive cases

作　　者：刘静[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》2009年第10期676-681,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的临床病理学特点。方法对2005年1月至2007年12月在首都医科大学宣武医院接受致疴灶外科手术切除治疗的连续273例患者的临床及病理学资料进行回顾性分析。结果273例患者的平均发病年龄为11．0岁，平均病程为11．2年。组织学具体分型：皮质发育畸形（MCD）158例，瘢痕性脑回63例，脑肿瘤26例，中枢神经系统炎症性病变13例，血管畸形3例，囊肿3例，胆脂瘤1例，其他分类未明6例。其中MCD又包括局灶性皮质发育不良（FCD）Ⅰ型104例（平均发病年龄11．1岁，平均病程11．2年），FCDⅡ型30例（平均发病年龄7．9岁，平均病程12．7年），轻微皮质发育不良（mildMCD）6例，结节性硬化6例，多小脑回3例，其余皮质发育不良9例。158例MCD中有31例伴有海马硬化的病理学表现。134例FCD中FCDIB型占86例（64．2％），多位于颞叶（45例）。脑肿瘤26例（平均发病年龄14．5岁，平均病程6．3年），其中20例为混合性神经元．胶质肿瘤（76．9％），常见于颞叶，多数周边伴有皮质发育不良的病理学改变。结论难治性癫疴中最常见的病理学类型依次分别为MCD、瘢痕性脑回和肿瘤。FCD最常见的病理学类型为FCDIB型，多位于颞叶，常伴有海马硬化。难治性癫痫相关的脑肿瘤多为位于颞叶且生长缓慢的混合性神经元．胶质肿瘤。Objective To investigate the clinicopathologic features of refractory epilepsy. Methods The clinical and pathologic features of refractory epilepsy of 273 cases undergoing surgical treatment in Xuanwu Hospital from January 2005 to December 2007 were reviewed retrospectively. Results The mean age of seizure onset and disease duration were 11.0 years and 11.2 years respectively. The following pathologic subgroups were identified： malformation of cortical development （MCD, 158/273） including focal cortical dysplasia （FCD） type Ⅰ （104, the mean age of seizure onset and disease duration were 11.1 years and 11.2 years respectively） , FCD type Ⅱ （30, the mean age of seizure onset and disease duration were 7.9 years and 12.7 years respectively） , mild MCD （6） , tuberous sclerosis complex （6） and other types （9） , brain tumours （26/273, the mean age of seizure onset and disease duration were 14. 5 years and 6. 3 years respectively）, ulegyria （63/273）, brain infections （13/273）, vascular malformation （3/273）, cyst （3/273）, eholesteatoma （1/273） and other unknown types （6/273）. Dual pathology was identified in 31 of 158 MCD cases. Eighty-six of 134 （64. 2% ） FCD cases were type Ⅰ B predominantly seen in temporal lobe. Twenty of 26 （76. 9% ）brain tumours were mixed neuronal-glial tumours predominantly located in temporal lobe. Cortical dysplasia was often seen in these cases. Conclusions The 3 most common causes of refractory epilepsy are MCD, ulegyria and brain turnouts. The predominant subtype of FCD is type Ⅰ B often located in temporal lobe, in which associated hippocampal sclerosis is often seen. Brain tumors in patients with refractory epilepsy are almost benign neoplasms located in temporal lobe, in which the most frequent type is mixed neuronal-glial tumour.

关 键 词：癫痫 皮质发育畸形 神经胶质瘤 

分 类 号：R686[医药卫生—骨科学]