Lerner Jason T, Salamon Noriko, Hauptman Jason S, Velasco Tonicarlo R, Hemb Marta, Wu Joyce Y, Sankar Raman, Donald Shields W, Engel Jerome, Fried Itzhak, Cepeda Carlos, Andre Veronique M, Levine Michael S, Miyata Hajime, Yong William H, Vinters Harry V, Mathern Gary W
Department of Pediatric Neurology, University of California, Los Angeles, California, USA.
Epilepsia. 2009 Jun;50(6):1310-35. doi: 10.1111/j.1528-1167.2008.01998.x. Epub 2009 Jan 21.
Recent findings on the clinical, electroencephalography (EEG), neuroimaging, and surgical outcomes are reviewed comparing patients with Palmini type I (mild) and type II (severe) cortical dysplasia. Resources include peer-reviewed studies on surgically treated patients and a subanalysis of the 2004 International League Against Epilepsy (ILAE) Survey of Pediatric Epilepsy Surgery. These sources were supplemented with data from University of California, Los Angeles (UCLA). Cortical dysplasia is the most frequent histopathologic substrate in children, and the second most common etiology in adult epilepsy surgery patients. Cortical dysplasia patients present with seizures at an earlier age than other surgically treated etiologies, and 33-50% have nonlocalized scalp EEG and normal magnetic resonance imaging (MRI) scans. 2-((18)F)Fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) is positive in 75-90% of cases. After complete resection, 80% of patients are seizure free compared with 20% with incomplete resections. Compared with type I, patients with type II cortical dysplasia present at younger ages, have higher seizure frequencies, and are extratemporal. Type I dysplasia is found more often in adult patients in the temporal lobe and is often MRI negative. These findings identify characteristics of patients with mild and severe cortical dysplasia that define surgically treated epilepsy syndromes. The authors discuss future challenges to identifying and treating medically refractory epilepsy patients with cortical dysplasia.
对帕尔米尼I型(轻度)和II型(重度)皮质发育异常患者进行比较,综述了近期有关临床、脑电图(EEG)、神经影像学及手术结果的研究发现。资料来源包括对接受手术治疗患者的同行评议研究以及对2004年国际抗癫痫联盟(ILAE)小儿癫痫手术调查的一项子分析。这些资料还补充了来自加利福尼亚大学洛杉矶分校(UCLA)的数据。皮质发育异常是儿童最常见的组织病理学基础,也是成人癫痫手术患者的第二大常见病因。皮质发育异常患者比其他接受手术治疗的病因患者出现癫痫发作的年龄更早,33% - 50%的患者头皮脑电图无定位表现且磁共振成像(MRI)扫描正常。2 - (18)F氟 - 2 - 脱氧 - D - 葡萄糖正电子发射断层扫描(FDG - PET)在75% - 90%的病例中呈阳性。完全切除术后,80%的患者无癫痫发作,而不完全切除的患者中这一比例为20%。与I型相比,II型皮质发育异常患者就诊年龄更小,癫痫发作频率更高,且发作部位在颞叶以外。I型发育异常在成人颞叶患者中更常见,且MRI常为阴性。这些发现明确了轻度和重度皮质发育异常患者的特征,这些特征界定了接受手术治疗的癫痫综合征。作者讨论了识别和治疗患有皮质发育异常的药物难治性癫痫患者未来面临的挑战。