难治性癫痫伴局灶性大脑皮质发育障碍15例患者的临床特点及病理学分析

Clinical characteristics and pathology of 15 cases of intractable epilepsy with focal cortical dysplasia

目的：探讨难治性癫痫致痫灶切除术后发现有大脑皮质发育障碍患者的脑组织病理学分型及临床特点。方法：收集贵州医科大学附属医院、第三军医大学附属新桥医院、遵义医学院附属医院神经外科2012年12月～2017年12月共15例难治性癫痫伴大脑皮质发育障碍术后患者，采集手术后脑组织标本，行HE和免疫组化染色检查，探讨局灶性大脑皮质发育障碍、临床和病理学特点，并收集患者临床影像学和脑电图资料，以及临床主要症状。结果：15例患者术后标本中包含轻度局灶皮质发育不良2例，局灶性皮质发育不良（FCD）13例，其中FCDI型4例、FEDII型9例。临床主要特点为认知功能损害、反复痫性发作和小头畸形等。影像学头颅MRI提示患者中5例有脑发育不良，其余10例未见特殊。15例患者的24h长程脑电图均为异常。结论：局灶性大脑皮质发育障碍是难治性癫痫的重要病因，临床诊断有一定困难，需要临床医师密切观察。

Objective： To investigate the histopathological classification and clinical characteristics of intractable epilepsy with focal cerebral dysplasia. Methods： 15 cases of intractable epilepsy with cortical dysplasia were collected from the Department of Neurosurgery of the Affiliated Hospital of Guizhou Med ical University, the Affiliated Xinqiao Hospital of the Third Military Medical University and the Affiliated Hospital of Zunyi Medical College from December 2012 to December 2017. The brain tissue specimens were collected after the operation. The hematoxylin and eosin （HE） staining and immunohistochemical staining were used to investigate the cerebral cortex dysplasia, Clinical images, EEG data and clinical symptoms were analyzed. Results：Of the 15 patients, 2 cases had mild hypoplasia, 13 cases had focal cortical dysptasia （FCD）, including 4 cases of FCD I type and 9 cases of FCD It type. The main clinical features included cognitive impairment, recurrent seizures, and ：small head deformity. Cranial MRI images showed that 5 patients had brain dysplasia, and the remaining 10 cases had not special symp toms. 24 h？ ambulatory electroencephalogram showed that 15 patients had abnormal EEG. Conclusion： The development disorders of cerebral cortex is an important cause of intractable epilepsy, The clinicians should pay more attention to intractable epilepsy with focal cerebral dysplasia because it is rather difficult in elinieal diagnosis.