Yum Mi-Sun, Lee Eun Hye, Ko Tae-Sung
Department of Pediatrics, Asan Medical Center, Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
J Child Neurol. 2013 Mar;28(3):308-13. doi: 10.1177/0883073812446485. Epub 2012 Jun 29.
Tuberous sclerosis complex is a genetic disorder resulting in epilepsy and mental retardation. Vigabatrin has shown efficacy in the treatment of infantile spasms caused by tuberous sclerosis complex, but its effects on focal seizures caused by tuberous sclerosis complex have not been determined. We compared the efficacy of vigabatrin in patients with tuberous sclerosis complex-induced focal seizures and infantile spasms and assessed the mental outcomes in both groups. We retrospectively evaluated 31 children with tuberous sclerosis complex and epilepsy, who were treated with vigabatrin in a single tertiary center in Seoul, Korea. Vigabatrin treatment resulted in spasms cessation in 16 of 18 (88.9%) patients with infantile spasms, whereas 6 of 13 (46.2%) patients with focal seizures became seizure free. Initial response to vigabatrin had no effect on intellectual disability. Vigabatrin was highly effective in eliminating infantile spasms caused by tuberous sclerosis complex but was less effective in patients with focal seizures.
结节性硬化症是一种导致癫痫和智力发育迟缓的遗传性疾病。氨己烯酸已显示出对结节性硬化症所致婴儿痉挛症的治疗效果,但它对结节性硬化症所致局灶性癫痫发作的影响尚未确定。我们比较了氨己烯酸对结节性硬化症所致局灶性癫痫发作和婴儿痉挛症患者的疗效,并评估了两组患者的智力发育结果。我们回顾性评估了在韩国首尔一家三级中心接受氨己烯酸治疗的31例结节性硬化症合并癫痫的儿童。氨己烯酸治疗使18例婴儿痉挛症患者中的16例(88.9%)痉挛停止,而13例局灶性癫痫发作患者中有6例(46.2%)癫痫发作停止。氨己烯酸的初始反应对智力残疾没有影响。氨己烯酸在消除结节性硬化症所致婴儿痉挛症方面非常有效,但对局灶性癫痫发作患者的效果较差。