Clinicopathologic analysis of cerebral hemisphere ulegyria in 26 patients with refractory epilepsy

目的 探讨难治性癫痫患儿大脑半球瘢痕性脑回的临床病理学特点.方法 对2011年1月至2015年8月在北京市海淀医院暨北京大学第三医院海淀院区功能神经外科接受癫痫病灶及致痫灶切除手术治疗、病理诊断为瘢痕脑回的26例难治性癫痫患儿的临床资料及病理资料进行回顾性分析.结果 26例患儿中男19例,女7例,发病年龄出生10余天～12岁(平均3.93岁),病程1个月余～13年(平均5.42年).其中围生期获得性损伤后发病者8例,围生期后脑损伤后发病者12例,无诱因出现癫痫发作6例.手术年龄5 ～14岁(平均9.35岁).手术方式包括多脑叶切除+胼胝体全段切开术(14例)及改良式解剖性大脑半球切除术(12例).脑切除标本病理诊断为瘢痕脑回伴局灶性皮质发育不良(FCD)Ⅲd及双重病理(瘢痕脑回伴FCDⅢd及海马硬化).术后随访3个月～5年,Engel Ⅰ级19例(73.08％),EngelⅡ级3例(11.54％),EngelⅢ级2例(7.69％),失访2例(7.69％).结论 婴幼儿时期的获得性脑损伤可致大脑半球大范围病变,形成瘢痕脑回及皮质发育不良,导致难治性癫痫.早期、及时地经改良式解剖性大脑半球切除术或多脑叶切除手术治疗后效果显著.Objective To investigate the clinicopathologic features of cerebral hemisphere ulegyria in children with refractory epilepsy.Methods The clinical and pathologic findings were reviewed in 26 children who underwent resection of lesion and epileptogenic lobectomy operation in the Neurosurgery Department of Haidian Hospital,Haidian District of Peking University Third Hospital,from January 2011 to August 2015,and pathological diagnosis was cerebral hemisphere ulegyria.Results All children including 19 male and 7 female had medically intractable seizures.The mean ages of seizure onset and disease duration were 3.93 years old (from 10 days to 12 years old) and 5.42 years (from 1 month to 13 years),respectively.Eight cases had seizures because of perinatal period injury,and 12 cases developed seizures owing to infancy injury and others had no cause of disease.The mean operation age was 9.35 years old (5-14 years old).Fourteen cases underwent multilobar resection and the whole corpus callosum incision,and 12 cases were given modified anatomical cerebral hemisphere resection.The pathological diagnoses of brain tissues were cerebral hemisphere ulegyria with focal cortical dysplasia(FCD) Ⅲ d and dual pathology.Seizure outcome after the operation revealed that 19 cases (73.08％) had an Engel grade Ⅰ,3 cases (11.54％)had an Engel grade Ⅱ,2 cases (7.69％) had an Engel grade Ⅲ,and 2 cases (7.69％) missed the follow-up.Conclusions Acquired brain injury during the period of infant can cause ulegyria and cortical dysplasia,resulting in intractable epilepsy.The treatment of improved anatomical cerebral hemisphere resection and multilobar resection is significantly effective.Foundation of Capital Institute of Pediatrics (Fx201704)首都儿科研究所基金中国科学引文数据库(CSCD)10784-7873