Objective 

Lance-Adams syndrome is a rare but devastating disorder characterized by rest, action, and stimulus-sensitive myoclonus after cardiorespiratory arrest. We aimed to present a case of multidrug-resistant Lance-Adams syndrome that was successfully treated with oral phenobarbital therapy.

Method and Results 

We report a previously healthy 11-year, 6-month-old boy was referred to our pediatric intensive care unit because of severe hypoxic ischemic brain injury due to sudden cardiorespiratory arrest. On the 15th day of hospitalization, he developed myoclonic jerks involving his limbs, trunk, and eyes. Despite many antiseizure medications in different combinations, myoclonic jerks persisted. Then, phenobarbital was started, and myoclonic jerks disappeared the next day. At the final evaluation, additional phenobarbital treatment was continued for 6 months and the patient remained myoclonus-free during this time.

Conclusions 

To the best of our knowledge, this case is the first report describing significant improvement with phenobarbital in a patient with multidrug-resistant Lance-Adams syndrome. We suggest that phenobarbital is an effective option and should be kept in mind in patients with multidrug-resistant Lance-Adams syndrome.

中文翻译：

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苯巴比妥成功治疗耐多药 Lance-Adams 综合征一例

客观的 

Lance-Adams 综合征是一种罕见但具有破坏性的疾病，其特征是心肺骤停后的休息、动作和刺激敏感肌阵挛。我们旨在介绍一例经口服苯巴比妥治疗成功治疗的多重耐药 Lance-Adams 综合征病例。

方法和结果 

我们报告一名既往健康的 11 岁 6 个月大男孩因心肺骤停导致严重缺氧缺血性脑损伤被转诊至我们的儿科重症监护病房。住院第 15 天，他的四肢、躯干和眼睛出现肌阵挛性抽搐。尽管有许多不同组合的抗癫痫药物，肌阵挛性抽搐仍然存在。然后，开始使用苯巴比妥，第二天肌阵挛抽搐消失。在最终评估时，额外的苯巴比妥治疗持续了 6 个月，在此期间患者没有出现肌阵挛。

结论 

据我们所知，该病例是第一份描述多药耐药 Lance-Adams 综合征患者使用苯巴比妥显着改善的报告。我们建议苯巴比妥是一种有效的选择，对于多重耐药的 Lance-Adams 综合征患者应牢记这一点。