Fan Pi-Chuan, Peng Steven Shinn-Forng, Yen Ruoh-Fang, Shieh Jeng-Yi, Kuo Meng-Fai
Department of Pediatrics, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.
Department of Medical Imaging, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.
J Formos Med Assoc. 2014 Apr;113(4):258-63. doi: 10.1016/j.jfma.2013.02.008. Epub 2013 May 3.
Myoclonic astatic epilepsy (MAE) is characterized by multiple seizure types, which are often refractory. Although vagus nerve stimulation (VNS) is an alternative treatment for medically intractable seizures, its exact mechanism of action remains unclear. Herein, we report the case of a 4-year-old boy with intractable MAE who has been in a seizure-free status for 2 years and 3 months since 6 months after the implantation of a vagus nerve stimulator (Model 103, Cyberonics, Inc., Houston, TX). Various test results 6 months after VNS were compared with those before VNS. Results of an electroencephalograph revealed disappearance of epileptiform discharges and an increased beta-gamma spectrum rhythm. The brain diffusion-tensor imaging showed an increased ratio of fraction anisotropy in the right fimbria-fornix, indicating improved diffusion of the white matter tract, and (18)F-fluorodeoxyglucose positron emission tomography revealed globally improved cerebral glucose metabolism. His cognitive and social-emotional performances also improved at 2 years after VNS. To the best of our knowledge, this is the first report to describe the effects of VNS on fimbria-fornix and glucose metabolism in MAE.
肌阵挛失张力性癫痫（MAE）的特点是发作类型多样，且常常难以治疗。尽管迷走神经刺激（VNS）是药物难治性癫痫的一种替代治疗方法，但其确切作用机制仍不清楚。在此，我们报告一例4岁患有难治性MAE的男孩，自植入迷走神经刺激器（型号103，赛博onics公司，得克萨斯州休斯顿）6个月后，已处于无癫痫发作状态达2年3个月。将VNS治疗6个月后的各项检查结果与VNS治疗前的结果进行了比较。脑电图结果显示癫痫样放电消失，β-γ频谱节律增加。脑弥散张量成像显示右侧穹窿柱-穹窿的各向异性分数比值增加，表明白质束扩散改善，且（18）F-氟脱氧葡萄糖正电子发射断层扫描显示全脑葡萄糖代谢改善。在VNS治疗2年后，他的认知和社会情感表现也有所改善。据我们所知，这是第一份描述VNS对MAE患者穹窿柱-穹窿和葡萄糖代谢影响的报告。
