A Case of Multidrug-Resistant Lance-Adams Syndrome Successfully Treated With Phenobarbital.

IF 0.8 4区医学 Q4 CLINICAL NEUROLOGY

Clinical Neuropharmacology Pub Date : 2023-01-01 DOI:10.1097/WNF.0000000000000532

Nurşah Yeniay Süt, Miraç Yıldırım, Ömer Bektaş, Tanıl Kendirli, Serap Teber

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引用次数: 1

Abstract

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.

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苯巴比妥治疗多药耐药兰斯-亚当斯综合征1例。

目的:兰斯-亚当斯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、动作和刺激敏感性肌阵挛。我们的目的是提出一个病例多药耐药兰斯-亚当斯综合征，是成功地治疗口服苯巴比妥治疗。方法和结果:我们报告了一名11岁6个月大的健康男孩，由于心肺骤停引起的严重缺氧缺血性脑损伤而被转介到我们的儿科重症监护室。住院第15天，患者出现四肢、躯干和眼睛肌阵挛性抽搐。尽管有许多不同组合的抗癫痫药物，肌阵挛痉挛仍然存在。然后开始使用苯巴比妥，第二天肌阵挛性抽搐消失。在最终评估时，额外的苯巴比妥治疗持续了6个月，在此期间患者没有肌阵挛。结论:据我们所知，该病例是第一例描述多药耐药兰斯-亚当斯综合征患者使用苯巴比妥显著改善的报告。我们建议苯巴比妥是一种有效的选择，对于多药耐药的兰斯-亚当斯综合征患者应该牢记这一点。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical Neuropharmacology 医学-临床神经学

CiteScore

1.20

自引率

10.00%

发文量

审稿时长

6-12 weeks

期刊介绍： Clinical Neuropharmacology is a peer-reviewed journal devoted to the pharmacology of the nervous system in its broadest sense. Coverage ranges from such basic aspects as mechanisms of action, structure-activity relationships, and drug metabolism and pharmacokinetics, to practical clinical problems such as drug interactions, drug toxicity, and therapy for specific syndromes and symptoms. The journal publishes original articles and brief reports, invited and submitted reviews, and letters to the editor. A regular feature is the Patient Management Series: in-depth case presentations with clinical questions and answers.