Division of Neurology, Saitama Children's Medical Center, Saitama, Japan.
Division of Neurology, Saitama Children's Medical Center, Saitama, Japan.
Brain Dev. 2022 May;44(5):319-328. doi: 10.1016/j.braindev.2022.01.004. Epub 2022 Feb 2.
We aimed to evaluate choice and efficacy of intravenous antiepileptic drugs (AEDs) for status epilepticus (SE) in Dravet syndrome and to find predictable clinical features demonstrating the effectiveness of benzodiazepine (BZD) for SE.
We retrospectively investigated the medical records in patients with Dravet syndrome and evaluated the effectiveness rate of intravenous AEDs and the rate of adverse effects. To find the clinical features of BZD-effective SE, we divided the SE episodes into the following two groups: BZD effective group and BZD non-effective group. The choice of treatment was dependent on physicians' discretion according to the protocol for SE in our institution.
Sixty-eight SE episodes in 10 patients were assessed. The median age at SE was 31 months. Of 68 episodes, 42 episodes (61.8%) were in the BZD effective group and 26 (38.2%) in the BZD non-effective group. There were no significant differences in clinical features. In the BZD non-effective group, the effective rates of continuous midazolam, phenobarbital, phenytoin/fosphenytoin were 9/9 episodes (100%), 14/17 (82.4%), and 2/5 (40.0%), respectively. Adverse effects were identified in 19/68 episodes (27.9%), including 11/42 episodes in the BZD effective group and 8/26 in the BZD non-effective group, which was no statistical difference between the two groups. Respiratory suppression was found in all 19 episodes and the incidence of endotracheal intubation in the BZD non-effective group (15.4%) was higher than that in the BZD effective group (2.4%) (p = 0.046).
BZD may be used as first choice, and phenobarbital prior to continuous midazolam as second choice for SE with Dravet syndrome. There might be no predictable clinical features showing that BZD will be effective.
我们旨在评估苯二氮䓬（BZD）类药物治疗 Dravet 综合征癫痫持续状态（SE）的选择和疗效，并寻找预测 BZD 治疗 SE 有效性的临床特征。
我们回顾性调查了 Dravet 综合征患者的病历，并评估了静脉用抗癫痫药物（AED）的有效率和不良反应发生率。为了找到 BZD 有效 SE 的临床特征，我们将 SE 发作分为 BZD 有效组和 BZD 无效组。根据我们机构的 SE 方案，治疗选择取决于医生的判断。
评估了 10 例患者的 68 例 SE 发作。SE 的中位年龄为 31 个月。68 例 SE 发作中，42 例（61.8%）为 BZD 有效组，26 例（38.2%）为 BZD 无效组。两组的临床特征无显著差异。在 BZD 无效组中，连续咪达唑仑、苯巴比妥、苯妥英/磷苯妥英的有效率分别为 9/9 例（100%）、14/17 例（82.4%）和 2/5 例（40.0%）。68 例 SE 发作中有 19 例（27.9%）出现不良反应，其中 BZD 有效组 11/42 例（26.2%），BZD 无效组 8/26 例（30.8%），两组间无统计学差异。所有 19 例均出现呼吸抑制，BZD 无效组气管插管发生率（15.4%）高于 BZD 有效组（2.4%）（p=0.046）。
BZD 类药物可作为首选，苯巴比妥可作为 Dravet 综合征 SE 患者连续咪达唑仑之前的二线选择。可能没有可预测的临床特征表明 BZD 类药物会有效。
