中华病理学杂志
中華病理學雜誌
중화병이학잡지
Chinese Journal of Pathology
2012年
10期
671-675
,共5页
王丹丹%桂秋萍%林久銮%郑重%杨姝杰%胡京霞%王世伦%石岩芳%罗阳%张光明%周文静
王丹丹%桂鞦萍%林久鑾%鄭重%楊姝傑%鬍京霞%王世倫%石巖芳%囉暘%張光明%週文靜
왕단단%계추평%림구란%정중%양주걸%호경하%왕세륜%석암방%라양%장광명%주문정
癫痫%精神外科手术%皮质发育畸形
癲癇%精神外科手術%皮質髮育畸形
전간%정신외과수술%피질발육기형
Epilepsy%Psychosurgery%Malformations of cortical development
目的 探讨大脑半球瘢痕性脑回的临床病理学特点.方法 对2007至2011年在清华大学玉泉医院癫痫中心接受改良式解剖性大脑半球切除术术后病理诊断为瘢痕性脑回的39例难治性癫痫患者临床病理学资料进行回顾性分析.结果 39例患者中男30例,女9例,癫痫平均发病年龄4.0岁,平均病程7年.既往史包括:高热惊厥(14例,35.9%),脑出血(8例,20.5%),难产窒息缺氧(6例,15.4%),外伤(6例,15.4%),血管畸形出血(1例,2.6%),不明确(4例,10.2%).镜下观察可见部分皮质结构消失,神经元减少,星形胶质细胞反应性增生.所有患者病变周围均伴不同程度的局灶皮质发育不良Ⅲd,并有2例伴海马硬化.术后患者随访,Engel Ⅰ级37例(94.9%),EngelⅡ级2例(5.1%).结论 儿童期尤其是婴儿期高热惊厥、脑出血、难产、外伤等可导致难治性癫痫,病理改变为瘢痕性脑回伴局灶皮质发育不良Ⅲd,如果病变较为广泛且局限于一侧半球,采用改良式解剖性大脑半球切除术效果较好.
目的 探討大腦半毬瘢痕性腦迴的臨床病理學特點.方法 對2007至2011年在清華大學玉泉醫院癲癇中心接受改良式解剖性大腦半毬切除術術後病理診斷為瘢痕性腦迴的39例難治性癲癇患者臨床病理學資料進行迴顧性分析.結果 39例患者中男30例,女9例,癲癇平均髮病年齡4.0歲,平均病程7年.既往史包括:高熱驚厥(14例,35.9%),腦齣血(8例,20.5%),難產窒息缺氧(6例,15.4%),外傷(6例,15.4%),血管畸形齣血(1例,2.6%),不明確(4例,10.2%).鏡下觀察可見部分皮質結構消失,神經元減少,星形膠質細胞反應性增生.所有患者病變週圍均伴不同程度的跼竈皮質髮育不良Ⅲd,併有2例伴海馬硬化.術後患者隨訪,Engel Ⅰ級37例(94.9%),EngelⅡ級2例(5.1%).結論 兒童期尤其是嬰兒期高熱驚厥、腦齣血、難產、外傷等可導緻難治性癲癇,病理改變為瘢痕性腦迴伴跼竈皮質髮育不良Ⅲd,如果病變較為廣汎且跼限于一側半毬,採用改良式解剖性大腦半毬切除術效果較好.
목적 탐토대뇌반구반흔성뇌회적림상병이학특점.방법 대2007지2011년재청화대학옥천의원전간중심접수개량식해부성대뇌반구절제술술후병리진단위반흔성뇌회적39례난치성전간환자림상병이학자료진행회고성분석.결과 39례환자중남30례,녀9례,전간평균발병년령4.0세,평균병정7년.기왕사포괄:고열량궐(14례,35.9%),뇌출혈(8례,20.5%),난산질식결양(6례,15.4%),외상(6례,15.4%),혈관기형출혈(1례,2.6%),불명학(4례,10.2%).경하관찰가견부분피질결구소실,신경원감소,성형효질세포반응성증생.소유환자병변주위균반불동정도적국조피질발육불량Ⅲd,병유2례반해마경화.술후환자수방,Engel Ⅰ급37례(94.9%),EngelⅡ급2례(5.1%).결론 인동기우기시영인기고열량궐、뇌출혈、난산、외상등가도치난치성전간,병리개변위반흔성뇌회반국조피질발육불량Ⅲd,여과병변교위엄범차국한우일측반구,채용개량식해부성대뇌반구절제술효과교호.
Objective To investigate the clinicopathologic features of the brain tissue diagnosed as ulegyria from modified anatomic hemispherectomy for refractory epilepsy.Methods The clinical and pathologic findings were reviewed in 39 patients who underwent modified anatomic hemispherectomy and diagnosed as ulegyria in the Epilepsy Center of Tsinghua University Yuquan Hospital from 2007 to 2011.Results All patients including 30 males and 9 females had medically intractable seizures,and the mean age of seizure onset and disease duration were 4.0 years and 7.3 years respectively.Significant history included febrile seizure in 14 patients (35.9%),cerebral hemorrhage in 8 patients (20.5%),fetal distress and surgical trauma each in 6 patients (15.4%),vascular malformation and cerebral hemorrhage in 1 patient (2.6%),and unclear history in 4 patients (10.2%).Histologically,all cases were characterized by cortical destruction,with neuronal loss and gliosis.All cases were accompanied by varying degree of cortical dysplasia,which were diagnosed as focal cortical dysplasia Ⅲ d.Hippocampus sclerosis was identified in 2 cases.Seizure outcome after surgery revealed 37 patients (94.9%) had an Engel grade Ⅰ,two patients (5.1%) had an Engel grade Ⅱ.Conclusions Febrile seizure,cerebral hemorrhage,fetal distress and surgical trauma in childhood can lead to refractory epilepsy.Histopathological change in the brain is ulegyria accompanied by focal cortical dysplasia Ⅲ d.Modified anatomic hemispherectomy is an effective therapy to treat those patients with extensive changes of one hemisphere.