Saeki Keisuke, Saito Yoshiaki, Sugai Kenji, Nakagawa Eiji, Komaki Hirofumi, Sakuma Hiroshi, Sasaki Masayuki, Kaneko Yuu
Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, National Center of Neurology and Psychiatry (NCNP), Kodaira, Japan.
Epilepsia. 2009 May;50(5):1274-9. doi: 10.1111/j.1528-1167.2008.01890.x. Epub 2008 Dec 20.
We describe herein a girl who has had startle-induced seizures since she was 3 years old. These seizures were refractory to antiepileptic medications and worsened when the patient was 9 years old, following termination of phenytoin administration because of adverse effects. During this period she could walk only a few steps with support, and sudden drop attacks inevitably occurred. Interictal electroencephalography (EEG) revealed abundant spike-wave activity at the centroparietal midline areas, and ictal EEG of poststartle and gait-induced seizures revealed initial voltage attenuation followed by recruitment of vertex activity, which preceded a tonic or myoclonic-atonic phase. Magnetic resonance imaging (MRI) results were unremarkable, but magnetoencephalography (MEG) and positron emission tomography (PET) suggested the presence of an extensive epileptogenic zone in the bilateral pericentral gyri, and the bilateral paracentral and left precuneus lobules, including the primary motor, supplementary motor, and supplementary sensory areas. The pathophysiologic significance of these structures is discussed.
我们在此描述一名自3岁起就患有惊吓诱发癫痫发作的女孩。这些癫痫发作对抗癫痫药物难治,在患者9岁时因苯妥英钠出现不良反应而停药后病情恶化。在此期间,她仅在支撑下能走几步,不可避免地会发生突然跌倒发作。发作间期脑电图(EEG)显示中央顶叶中线区域有大量棘波活动,惊吓后发作和步态诱发发作的发作期EEG显示初始电压衰减,随后顶点活动募集,先于强直或肌阵挛-失张力期。磁共振成像(MRI)结果无异常,但脑磁图(MEG)和正电子发射断层扫描(PET)提示双侧中央旁回、双侧中央旁小叶和左侧楔前叶存在广泛的致痫区,包括初级运动区、辅助运动区和辅助感觉区。讨论了这些结构的病理生理意义。