Lu Wen-Yu, Weng Wen-Chin, Wong Lee-Chin, Lee Wang-Tso
Department of Pediatrics, Min-Sheng General Hospital, No. 168, ChingKuo Rd., Taoyuan Dist., Taoyuan City 330, Taiwan; Department of Pediatrics, National Taiwan University Hospital, No. 8, Zhongshan S. Rd., Zhongzheng Dist., Taipei City 100, Taiwan.
Department of Pediatrics, National Taiwan University Hospital, No. 8, Zhongshan S. Rd., Zhongzheng Dist., Taipei City 100, Taiwan; Clinical Center for Neuroscience and Behavior, National Taiwan University Hospital, No. 7, Zhongshan S. Rd., Zhongzheng Dist., Taipei City 100, Taiwan.
Epilepsy Behav. 2018 Sep;86:66-71. doi: 10.1016/j.yebeh.2018.06.029. Epub 2018 Jul 10.
Both refractory convulsive status epilepticus (SE) and super-refractory SE are medical emergencies. However, there are limited data on super-refractory SE in children. Thus, this study focuses on characterizing the demographics, outcomes, and prognostic factors for super-refractory SE in children.
This study was a retrospective analysis of super-refractory SE treated in a tertiary referral center in Taiwan. The functional outcome was evaluated by modified Rankin scale (mRS). Significant functional decline was defined as an mRS difference (before hospital admission and at discharge) of more than 2. The variates and the follow-up mRS values were then analyzed statistically.
We enrolled 134 patients with 191 episodes of convulsive SE and identified 30 patients with 38 episodes of convulsive super-refractory SE. The incidence of convulsive super-refractory SE in the group with SE was 19.9%, and the age ranged from 2.5 months to 17 years. In-hospital mortality was 13.3%, which was much lower than that of adult cohorts. Newly acquired epilepsy and cognitive deficit occurred in 100% and 88.5%, respectively. Newly acquired epilepsy, as a sequel of super-refractory SE, was observed in all 18 patients (100%) who survived and had no history of epilepsy. Significant functional decline (mRS difference of more than 2) at discharge occurred in 76.7%. Poor functional outcome was associated with acute symptomatic etiology (P < 0.001) and the number of anesthetic agents (P = 0.002). The functional outcome improved after 1 year of follow-up in our population.
Super-refractory SE is associated with significant morbidity and mortality in children. However, the in-hospital mortality rate is much lower compared with adults. The functional outcome in children is associated with acute symptomatic etiology and the number of anesthetic agents and may improve after long-term follow-up.
难治性惊厥性癫痫持续状态（SE）和超难治性SE均为医疗急症。然而，关于儿童超难治性SE的数据有限。因此，本研究着重于描述儿童超难治性SE的人口统计学特征、结局及预后因素。
本研究是对台湾一家三级转诊中心治疗的超难治性SE进行的回顾性分析。功能结局通过改良Rankin量表（mRS）进行评估。显著功能下降定义为mRS差异（入院前和出院时）大于2。然后对变量和随访mRS值进行统计学分析。
我们纳入了134例有191次惊厥性SE发作的患者，并确定了30例有38次惊厥性超难治性SE发作的患者。SE组中惊厥性超难治性SE的发生率为19.9%，年龄范围为2.5个月至17岁。住院死亡率为13.3%，远低于成人队列。新发癫痫和认知缺陷的发生率分别为100%和88.5%。在所有存活且无癫痫病史的18例患者（100%）中均观察到新发癫痫，这是超难治性SE的后遗症。出院时显著功能下降（mRS差异大于2）的发生率为76.7%。功能结局不良与急性症状性病因（P<0.001）和麻醉药物数量（P=0.002）有关。在我们的研究人群中，随访1年后功能结局有所改善。
超难治性SE在儿童中与显著的发病率和死亡率相关。然而，与成人相比，住院死亡率要低得多。儿童的功能结局与急性症状性病因和麻醉药物数量有关，且长期随访后可能会改善。
