CAJ | 학술논문

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.
목적 연구난치성전간환자대뇌반구혹다뇌협절제표본적림상병이학특점.방법 대2005-2009년재수도의과대학선무의원접수대뇌반구(행공능성대뇌반구절제술)혹다뇌협절제적46례난치성전간환자림상병이학자료진행회고성분석.결과 46례환자적평균발병년령3.9세,평균병정위10.2년.기중행대뇌반구절제33례,다뇌협절제13례,차섭협전부수루.조직학분형:계발성반흔뇌회31례,피질발육기형7례,중추신경계통감염8례.31례반흔뇌회표본경하가견피질결구소실、신경원감소、반응성효질세포증생급정분양소체출현;차외환가관찰도함철혈황소(13례)、개화(9례)이급도상분포적신경원(5례).반흔뇌회균반불동정도적피질발육불량,병유7례반해마경화.7례뇌회발육기형중,국조성피질발육불량(FCD)5례(기중FCDⅠB형3례,FCDⅠA형1례,FCDⅡA형1례),다소뇌회기형급천통뇌기형각1례.8례중추신경계통감염중Rasmussen뇌염5례、거세포병독성뇌염1례、결핵성뇌막염1례、낭충감염1례.결론 대뇌반구혹다뇌협절제적뇌조직병이학주요시다충원인(비여외상、결양등)인기적계발성반흔뇌회,섭협병변명현.중추신경계통감염이Rasmussen뇌염위주,피질발육기형주요이FCDⅠB형위주.
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 multilobar 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 FCDⅠB, 1 case of FCDⅡA and 1 case of FCDⅠA, 1 case of polymicrogyria and 1 case of porencephaly. Among 8 infection eases, there were 5 cases of Rasmussen encephalitis ( RE), l case of cysticercosis, 1 case of tuberculous meningitis and l case of Cytomegalovirus encephalitis. Conclusions The most common pathological category of specimens from hemispherectomy or multilobar resection is ulegyria with obvious temporal lobe abnormality. This is followed by MCD ( with FCDⅠB as the main type) and central nervous system infection (RE as the most frequent abnormality).