局灶皮层发育不良51例的病理与临床

Pathology and clinic of 51 focal cortical dysplasia

目的探讨局灶性皮层发育不良(Focal cortical dysplasia,FCD)的病理与临床特征。方法按照2011年国际抗癫痫联盟(ILAE)的分类标准,对2015年1月—2018年9月中国人民解放军联勤保障部队第988医院收治的51例FCD患者病脑组织进行病理分型,分析不同分型的癫痫发作特征。结果 FCDⅠ型23例(45.1%)、Ⅱ型11例(21.6%)、Ⅲ型17例(33.3%)。Ⅰa型最多(23.5%,12/51),镜下特征为神经元呈微柱状结构排列,免疫组化NF呈紧贴着神经元的线样、瀑布样表达;其次是Ⅲa型(15.7%,8/51),镜下以海马CA4区锥体细胞减少或缺失为主。各类型在双侧大脑半球的发生率无统计学差异,主要是单脑叶发生,颞叶显著多余额叶,超过50%的病例18岁以前出现癫痫症状,主要表现为部分性发作、继发全身强直阵挛,Ⅲ型患者的癫痫首次发作年龄早于Ⅰ、Ⅱ型,癫痫病史也长于其他两型。影像上Ⅰ型阳性率78.3%,其余两型均为100%。结论 FCD是癫痫患者常见的病理特征,仔细的病理检查是各亚型准确分型的前提,Ⅲ型的癫痫发作不同于Ⅰ、Ⅱ型。

Objective To explore the microscopic character and clinical pathological feature of focal cortical dysplasia(FCD). Methods 51 cases were collected from January 2015 to September 2018 in the 988 th hospital of the joint logistics support force of the People’s Libereation Army. Pathology with FCD of their diseased brain tissue was classified according to the classification standard by the International Anti-Epilepsy Union(ILAE) in 2011. Epileptic seizure characteristics were analysed in different types. Results FCD I was 23 cases(45.1%). FCD II was 11(21.6%). FCD III was 17(33.3%). Ia was the most common type(23.5%,12/51). Neurons were arranged into microcolumnar structures in Ia. NF expression in immunohistochemistry was characteristic. It was close to the neuron like line or waterfall. The second type was Ⅲa(15.7%, 8/51). Hippocampal sclerosis was given priority to CA4 area pyramidal cells to reduce or disappear.Three types all happened in bilateral cerebral hemisphere. There was no statistical difference. Temporal lobe was significantly more than frontal lobe. More than 50% of the cases occurredepilepsy before the age of 18. The main manifestation was partial onset seizures and secondary body stiffness clonus. The onset age and history of epilepsy in patients with FCD Ⅲ were earlier than those in the other two types. On image the positive rate of I type was 78.3% and that of Ⅱ and Ⅲ was both 100%. Conclusion FCD is a common pathological feature of epilepsy patients. Carefully pathologic examination is the premise of accurate classification of each subtype. Ⅲ type is different from Ⅰ and Ⅱ type in epileptic seizures.