Divisions of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
J Neuropathol Exp Neurol. 2013 Sep;72(9):884-91. doi: 10.1097/NEN.0b013e3182a38d88.
To identify pathologic characteristics that are associated with outcome, we performed a retrospective analysis of the clinical, radiologic, and pathologic features of 44 children with isolated focal cortical dysplasia (FCD) after epilepsy surgery. Based on the International League Against Epilepsy Classification, 16 patients had FCD Type I and 28 subjects had FCD Type II. A significantly higher percentage of subjects with FCD Type IIb versus Types I and IIa were seizure-free after surgery. Akt (also known as protein kinase B) is the main downstream target of phosphatidylinositol 3'-kinase and has been implicated in epilepsy pathogenesis. Semiquantitative analysis of cortical gliosis and quantitation of Akt1-immunoreactive neurons indicated that individuals with FCD Type II were more likely to have diffuse astrogliosis and higher counts of Akt1-positive neurons versus those with FCD Type I. A logistic regression model, including Akt1-positive neurons, age at surgery, and the interaction of these factors, was significantly associated with seizure-free outcome. This study provides evidence that astrogliosis and overexpression of neuronal Akt1 protein may be important factors in the pathogenesis of FCD and suggests that the pathogenesis of FCD Type I may differ from that of FCD Type II in children.
为了确定与预后相关的病理特征,我们对 44 例接受癫痫手术后的孤立性局灶性皮质发育不良(FCD)患儿的临床、影像学和病理学特征进行了回顾性分析。根据国际抗癫痫联盟分类,16 例患者为 FCD I 型,28 例患者为 FCD II 型。与 FCD I 型和 FCD IIa 型相比,FCD IIb 型患者术后无癫痫发作的比例明显更高。Akt(也称为蛋白激酶 B)是磷脂酰肌醇 3'-激酶的主要下游靶标,与癫痫发病机制有关。皮质神经胶质增生的半定量分析和 Akt1 免疫反应性神经元的定量表明,与 FCD I 型相比,FCD II 型患者更有可能出现弥漫性星形胶质增生和更多的 Akt1 阳性神经元。包括 Akt1 阳性神经元、手术年龄以及这些因素的相互作用的逻辑回归模型与无癫痫发作的结果显著相关。这项研究提供了证据表明,星形胶质增生和神经元 Akt1 蛋白的过度表达可能是 FCD 发病机制中的重要因素,并表明 FCD I 型的发病机制可能与儿童的 FCD II 型不同。