摘要目的 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.

abstractsObjective 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.