Xeros Helena, Sakusic Amra, Fugate Jennifer E, Hawkes Maximiliano A, Wijdicks Eelco F M, Rabinstein Alejandro A, Braksick Sherri A
Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN, USA.
Neurocrit Care. 2025 Jun 23. doi: 10.1007/s12028-025-02307-z.
Lance-Adams syndrome (LAS) is a rare neurological complication following cardiopulmonary resuscitation. Patients present with action myoclonus within days to months after awakening. There is no established first-line treatment. The objectives of this study were to describe treatments, diagnostic approaches, and long-term clinical outcomes for LAS.
We performed a retrospective review of patients seen at three tertiary referral hospitals. An electronic database was used to identify all patients diagnosed with LAS from January 1, 2010, to June 30, 2022. Demographics, diagnostics, treatments, and long-term clinical outcomes were extracted. Descriptive statistics were completed to summarize treatments, diagnostics, and clinical outcomes, which were assessed by Cerebral Performance Category (CPC) at 6 months. Symptom control was defined as minimal or no residual myoclonus causing functional impairment on activities of daily living.
Thirty-nine patients met inclusion criteria. A total of 25 patients were diagnosed with LAS during the acute hospitalization after their cardiac arrest, and the rest were diagnosed in the outpatient setting. The most common initial treatment was levetiracetam (n = 26; 67%) followed by valproic acid (n = 4; 10.3%). Most patients initially treated with levetiracetam (n = 22; 85%) or valproic acid (n = 4; 100%) had symptom improvement. Most patients (n = 28; 72%) required a second medication. The most common second-line agent was a benzodiazepine (n = 13; 48%). Thirty-three patients (85%) had improvement of the myoclonus over time. Thirty (77%) were able to achieve symptom control. The median time to achieve symptom control for patients diagnosed in the inpatient setting was 70 days. At the 6-month follow-up, 8 patients (23%) achieved a CPC score of 1, whereas 11 patients (30%) had a CPC score of 2. Thirteen patients (33%) attempted to wean off their medications, of which six patients (46%) were successful.
Most patients with LAS experienced improvement of their myoclonus while being treated with levetiracetam, valproic acid, or benzodiazepines, although multiple medications and long-term treatment were often used. Half the patients attained favorable functional outcomes at 6 months.
兰斯-亚当斯综合征（LAS）是心肺复苏术后一种罕见的神经系统并发症。患者在苏醒后数天至数月内出现动作性肌阵挛。目前尚无既定的一线治疗方法。本研究的目的是描述LAS的治疗方法、诊断途径和长期临床结局。
我们对三家三级转诊医院诊治的患者进行了回顾性研究。使用电子数据库识别2010年1月1日至2022年6月30日期间所有诊断为LAS的患者。提取患者的人口统计学信息、诊断、治疗和长期临床结局。完成描述性统计以总结治疗方法、诊断和临床结局，6个月时通过脑功能分级（CPC）进行评估。症状控制定义为日常生活活动中导致功能障碍的残留肌阵挛极少或没有。
39例患者符合纳入标准。共有25例患者在心脏骤停后的急性住院期间被诊断为LAS，其余患者在门诊被诊断。最常见的初始治疗药物是左乙拉西坦（n = 26；67%），其次是丙戊酸（n = 4；10.3%）。大多数最初接受左乙拉西坦（n = 22；85%）或丙戊酸（n = 4；100%）治疗的患者症状有所改善。大多数患者（n = 28；72%）需要第二种药物。最常见的二线药物是苯二氮䓬类药物（n = 13；48%）。随着时间的推移，33例患者（85%）的肌阵挛有所改善。30例患者（77%）能够实现症状控制。住院患者实现症状控制的中位时间为70天。在6个月的随访中，8例患者（23%）的CPC评分为1，而11例患者（30%）的CPC评分为2。13例患者（33%）尝试减停药物，其中6例患者（46%）成功减停。
大多数LAS患者在接受左乙拉西坦、丙戊酸或苯二氮䓬类药物治疗时肌阵挛有所改善，尽管通常需要多种药物和长期治疗。一半的患者在6个月时获得了良好的功能结局。
