Epilepsy Unit, Department of Neurology, Hospital Clínic, Barcelona, Spain.
Epileptic Disord. 2010 Jun;12(2):155-8. doi: 10.1684/epd.2010.0307. Epub 2010 May 18.
Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is a familial partial epilepsy syndrome characterized by seizures suggesting a frontal lobe origin occurring predominantly during sleep. Up to a third of patients may have refractory seizures, with repeated episodes of status epilepticus, intellectual disability of variable degree and psychiatric disturbances. We report a patient with ADNFLE, refractory seizures and repeated episodes of life-threatening convulsive status epilepticus who underwent prolonged video-EEG monitoring and was implanted with a vagal nerve stimulator. At 3.5 years of follow-up, a decrease of more than 80% in seizure frequency was achieved, episodes of status were completely controlled and he displayed improved mood and alertness. Vagal nerve stimulation may be considered as therapy for patients with refractory epilepsies of genetic cause, as well as repeated status epilepticus.
常染色体显性遗传性夜间额叶癫痫(ADNFLE)是一种家族性部分性癫痫综合征,其特征为主要在睡眠中发生、提示起源于额叶的癫痫发作。多达三分之一的患者可能有难治性癫痫发作,反复出现癫痫持续状态,不同程度的智力障碍和精神障碍。我们报告了一例 ADNFLE 患者,难治性癫痫发作和反复发生危及生命的惊厥性癫痫持续状态,患者接受了长时间的视频-脑电图监测,并植入了迷走神经刺激器。在 3.5 年的随访中,癫痫发作频率降低了 80%以上,癫痫持续状态完全得到控制,患者情绪和警觉性改善。迷走神经刺激器可被视为治疗难治性遗传性癫痫以及反复癫痫持续状态的一种方法。