Univ Rennes, Inserm, EHESP, Irset (Research Institute for Environmental and Occupational Health), UMR_S 1085, Rennes, France.
Univ Rennes, CHU Rennes, INSERM, LTSI - UMR 1099, Rennes, France.
Epilepsia. 2018 Jul;59(7):e109-e113. doi: 10.1111/epi.14454. Epub 2018 Jun 14.
Childhood absence epilepsy (CAE) is one of the most frequent epilepsies in infancy. The first-line recommended therapy for CAE is based on the prescription of the narrow-spectrum ethosuximide and the broad-spectrum valproic acid, which have similar efficacy in the first 12 months. Nevertheless, some antiepileptic drugs (AEDs) may worsen seizure duration and type in this syndrome. In line with this, we have encountered a case of identical twins with CAE and early exposure to different antiseizure drugs leading to divergent outcomes. From this, we hypothesized that the first AED to treat CAE may determine the long-term prognosis, especially in the developing brain, and that some situations leading to drug resistance may be explained by use of an inappropriate first AED. Therefore, we investigated this hypothesis by using a genetic mouse model of absence epilepsy (BS/Orl). Mice received a first appropriate or inappropriate AED followed by the same appropriate AED. Our data demonstrate that an inappropriate first AED has a negative impact on the long-term efficacy of a second appropriate AED. This work supports the necessity to effectively diagnose epileptic syndromes prior to medication use, particularly in children, in order to prevent the deleterious effects of an inappropriate initial AED.
儿童失神癫痫(CAE)是婴儿期最常见的癫痫之一。CAE 的一线推荐治疗方法基于处方开窄谱乙琥胺和广谱丙戊酸,这两种药物在最初 12 个月的疗效相似。然而,一些抗癫痫药物(AEDs)在这种综合征中可能会使发作持续时间和类型恶化。基于这一点,我们遇到了一对 CAE 同卵双胞胎的病例,他们在早期接触了不同的抗癫痫药物,导致结果不同。由此,我们假设治疗 CAE 的第一种 AED 可能决定长期预后,特别是在发育中的大脑中,而一些导致耐药的情况可能可以用使用不合适的第一种 AED 来解释。因此,我们使用失神癫痫的遗传小鼠模型(BS/Orl)来研究这一假说。小鼠接受第一种合适或不合适的 AED,然后再接受相同的合适 AED。我们的数据表明,不合适的第一种 AED 对第二种合适 AED 的长期疗效有负面影响。这项工作支持在用药前有效诊断癫痫综合征的必要性,特别是在儿童中,以防止不合适的初始 AED 的有害影响。