Piao Yue-shan, Chen Li, Fu Yong-juan, Wang Wei, Li Yong-jie, Lu De-hong
Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China.
Zhonghua Bing Li Xue Za Zhi. 2007 Mar;36(3):150-4.
To investigate the clinicopathologic features of focal cortical dysplasia (FCD) in patients with refractory epilepsy.
The clinical, radiologic and pathologic features of 38 cases of FCD receiving surgical treatment in 2005 were reviewed retrospectively.
The mean age of disease onset was 9.2 years. The disease lasted for 11.9 years on average and often presented as complex partial seizure. Radiologic examination revealed hippocampal sclerosis, or abnormal signals in the grey matter in 21 cases. According to Palmini's classification system, the following pathologic subgroups were identified: FCD type IA (3/38), FCD type IB (20/38), FCD type IIA (5/38) and FCD type IIB (5/38). The remaining 5 cases were classified as mild cortical dysplasia. Topographically, FCD type II was often seen in the extratemporal region (8/10), predominantly in the frontal lobe (5/8). Dual pathology was identified only in cases with FCD type IB. Immunohistochemical study showed that the giant neurons, immature neurons and dysmorphic neurons were strongly positive for NeuN. A small number of balloon cells expressed nestin.
FCD is a common cause of refractory epilepsy. FCD type IB is the predominant pathologic subtype. Associated hippocampal sclerosis is sometimes seen. Clinicopathologic differences between FCD type I and FCD type II are observed.
探讨难治性癫痫患者局灶性皮质发育不良(FCD)的临床病理特征。
回顾性分析2005年接受手术治疗的38例FCD患者的临床、影像学和病理特征。
发病平均年龄为9.2岁。病程平均11.9年,常表现为复杂部分性发作。影像学检查发现21例有海马硬化或灰质异常信号。根据帕尔米尼分类系统,确定了以下病理亚组:IA型FCD(3/38)、IB型FCD(20/38)、IIA型FCD(5/38)和IIB型FCD(5/38)。其余5例归为轻度皮质发育不良。从部位上看,II型FCD多见于颞叶外区域(8/10),主要在额叶(5/8)。仅在IB型FCD病例中发现双重病理改变。免疫组织化学研究显示,巨型神经元、未成熟神经元和异形神经元NeuN呈强阳性。少数气球样细胞表达巢蛋白。
FCD是难治性癫痫的常见病因。IB型FCD是主要的病理亚型。有时可见相关的海马硬化。观察到I型和II型FCD之间的临床病理差异。