CAJ | 학술논문

目的研究难治性癫(癎)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(癎)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,搬痕性脑回63例,肭肿瘤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(mild MCD)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FCD Ⅰ B型占86例(64.2%),多位于颢叶(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(癎)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FCD Ⅰ B型,多位于颞叶,常伴有海马硬化.难治性癫(癎)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.
목적 연구난치성전(간)적림상병이학특점.방법 대2005년1월지2007년12월재수도의과대학선무의원접수치(간)조외과수술절제치료적련속273례환자적림상급병이학자료진행회고성분석.결과 273례환자적평균발병년령위11.0세,평균병정위11.2년.조직학구체분형:피질발육기형(MCD)158례,반흔성뇌회63례,눌종류26례,중추신경계통염증성병변13례,혈관기형3례,낭종3례,담지류1례,기타분류미명6례.기중MCD우포괄국조성피질발육불량(FCD)Ⅰ형104례(평균발병년령11.1세,평균병정11.2년),FCDⅡ형30례(평균발병년령7.9세,평균병정12.7년),경미피질발육불량(mild MCD)6례,결절성경화6례,다소뇌회3례,기여피질발육불량9례.158례MCD중유31례반유해마경화적병이학표현.134례FCD중FCD Ⅰ B형점86례(64.2%),다위우호협(45례).뇌종류26례(평균발병년령14.5세,평균병정6.3년),기중20례위혼합성신경원-효질종류(76.9%),상견우섭협,다수주변반유피질발육불량적병이학개변.결론 난치성전(간)중최상견적병이학류형의차분별위MCD、반흔성뇌회화종류.FCD최상견적병이학류형위FCD Ⅰ B형,다위우섭협,상반유해마경화.난치성전(간)상관적뇌종류다위위우섭협차생장완만적혼합성신경원-효질종류.
Objective To investigate the elinieopathologie 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 turnouts (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), cholesteatoma (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 tumours. The predominant subtype of FCD is type Ⅰ B often located in temporal lobe, in which associated hippoeampal 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.