Guerrini R, Belmonte A, Parmeggiani L, Perucca E
Institute of Child Neurology and Psychiatry, University of Pisa, Italy.
Brain Dev. 1999 Sep;21(6):420-4. doi: 10.1016/s0387-7604(99)00048-0.
An 8-year-old girl with Lennox-Gastaut syndrome showed a partial reduction in seizure frequency when lamotrigine (LTG), 15 mg/kg per day, was added to clobazam (CLB) and vigabatrin (VGB). An increase in LTG dosage to 20 mg/kg per day produced no further improvement and was followed by myoclonic status epilepticus. The condition developed insidiously and ultimately became stable. Video-EEG polygraphy and jerk-locked back-averaged EEG demonstrated continuous myoclonus of cortical origin. Discontinuation of LTG resulted in rapid disappearance of clinical and electrophysiological manifestations of myoclonic status epilepticus. No episodes of myoclonus occurred in the subsequent 2 years, during which CLB and VGB were kept unchanged. The striking response to drug discontinuation suggests that LTG may have played a role in the precipitation of status, possibly within the context of paradoxical intoxication.
一名患有 Lennox-Gastaut 综合征的 8 岁女孩，在氯巴占（CLB）和氨己烯酸（VGB）基础上加用拉莫三嗪（LTG），剂量为每日 15 mg/kg 时，癫痫发作频率有所部分降低。将 LTG 剂量增加至每日 20 mg/kg 未带来进一步改善，随后出现了肌阵挛性癫痫持续状态。病情隐匿发展，最终趋于稳定。视频脑电图多导记录及抽搐锁定反向平均脑电图显示为皮质起源的持续性肌阵挛。停用 LTG 后，肌阵挛性癫痫持续状态的临床及电生理表现迅速消失。在随后 2 年中，CLB 和 VGB 剂量不变，未再出现肌阵挛发作。对停药的显著反应提示，LTG 可能在癫痫持续状态的诱发中起了作用，可能是在反常中毒的情况下。
