Lee Sang Kun, Kim Dong-Wook
Department of Neurology, Seoul National University College of Medicine, Seoul;
Department of Neurology, Konkuk University College of Medicine, Seoul, Korea.
J Epilepsy Res. 2013 Dec 30;3(2):43-7. doi: 10.14581/jer.13009. eCollection 2013 Dec.
Focal cortical dysplasia (FCD) is the most commonly encountered developmental malformation that causes refractory epilepsy. With advances in neuroimaging techniques, in particular MRI, recent studies have revealed a higher prevalence of FCD than previously estimated and have improved the preoperative identification and classification of these abnormalities. However, MRI frequently does not show any abnormalities in patients with pathologically proven FCD. In this situation, functional neuroimaing such as FDG-PET and ictal SPECT can be helpful. FCD is thought to be intrinsically epileptogenic, because the dysplastic tissues contain aberrant neural networks that are highly susceptible to abnormal excitation. The response to the medical treatment of epilepsy has been documented as consistently poor. Therefore, surgical resection has been an important alternative treatment for patients with intractable epilepsy related to FCD. Incomplete resection of FCD has been consistently known to be a poor prognostic factor. However, the complete removal of FCD is often difficult because the demarcation of the lesion is frequently poor, and dysplastic tissues tend to be more extensive than is apparent on MRI. Evidence indicates that even patients with MRI abnormalities who have resective epilepsy surgery for FCD have worse surgical outcomes than those of patients who have surgery for other focal lesional epilepsy syndromes. Careful planning of evelauation using intracranial electrodes is necessary for successful epilepsy surgery.
局灶性皮质发育不良(FCD)是最常见的导致难治性癫痫的发育性畸形。随着神经影像学技术的进步,尤其是磁共振成像(MRI),最近的研究显示FCD的患病率高于先前的估计,并改善了这些异常的术前识别和分类。然而,在病理证实为FCD的患者中,MRI常常未显示任何异常。在这种情况下,功能神经影像学检查,如氟代脱氧葡萄糖正电子发射断层扫描(FDG-PET)和发作期单光子发射计算机断层扫描(ictal SPECT)可能会有所帮助。FCD被认为本质上具有致痫性,因为发育异常的组织包含高度易受异常兴奋影响的异常神经网络。癫痫药物治疗的反应一直被证明效果不佳。因此,手术切除一直是与FCD相关的难治性癫痫患者的重要替代治疗方法。一直以来,FCD切除不完全被认为是一个不良的预后因素。然而,FCD的完全切除往往很困难,因为病变的边界常常不清楚,而且发育异常的组织往往比MRI上显示的范围更广。有证据表明,即使是有MRI异常且因FCD接受切除性癫痫手术的患者,其手术效果也比因其他局灶性病变性癫痫综合征接受手术的患者差。对于成功的癫痫手术,使用颅内电极进行仔细的评估规划是必要的。