兰斯-亚当斯综合症:当前的理解和管理。

Aleksandar Sič
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摘要

兰斯-亚当斯综合征(LAS)是一种罕见的缺氧脑损伤的神经系统并发症,其特征是恢复意识的患者出现动作和刺激敏感性肌阵挛。虽然经常被误解为预后不良的标志,但LAS可导致严重的长期残疾,但也显示出通过适当管理功能恢复的潜力。方法:使用PubMed和ScienceDirect数据库中预定义的搜索策略进行叙述性综述,以确定2000年1月至2025年4月期间发表的关于LAS的同行评议研究。最终综合纳入了47项研究,包括34项病例报告、7项叙述性或范围评价、2项系统评价和4项原始临床研究。结果LAS的病理生理机制是多因素的,包括皮层高兴奋性、皮层下去抑制和神经递质失衡。脑电图和影像学研究提供了重要的诊断线索。根据经验证据,管理在很大程度上仍然是症状性的。氯硝西泮和丙戊酸钠仍然是一线治疗药物,而perampanel、氧化钠、大麻二酚和鞘内巴氯芬等药物已经在难治性病例中进行了试验。多学科康复在长期预后中起着至关重要的作用。结论提高对LAS的认识和早期识别可提高诊断准确性和治疗效果。尽管罕见,临床医生应保持警惕,LAS作为一个潜在的可逆的疾病,当适当的诊断和管理。标准化的治疗指南仍然是未来的优先事项。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Lance-Adams Syndrome: Current Understanding and Management.

BackgroundLance-Adams Syndrome (LAS) is a rare neurological complication of hypoxic brain injury, characterized by action- and stimulus-sensitive myoclonus in patients who have regained consciousness. Although often misinterpreted as a sign of poor prognosis, LAS can lead to significant long-term disability but also shows potential for functional recovery with appropriate management.MethodologyA narrative review was conducted using a predefined search strategy across PubMed and ScienceDirect databases to identify peer-reviewed studies on LAS published between January 2000 and April 2025. A total of 47 studies were included in the final synthesis, comprising 34 case reports, 7 narrative or scoping reviews, 2 systematic reviews and 4 original clinical studies.ResultsThe pathophysiology of LAS remains multifactorial, involving cortical hyperexcitability, subcortical disinhibition, and neurotransmitter imbalances. Electroencephalographic and imaging studies provide important diagnostic clues. Management remains largely symptomatic, based on empirical evidence. Clonazepam and valproate remain first-line therapies, while agents such as perampanel, sodium oxybate, cannabidiol, and intrathecal baclofen have been trialed in refractory cases. Multidisciplinary rehabilitation plays a crucial role in long-term outcomes.ConclusionGreater awareness and earlier recognition of LAS can improve diagnostic accuracy and therapeutic outcomes. Despite its rarity, clinicians should remain alert to LAS as a potentially reversible disorder when appropriately diagnosed and managed. Standardized treatment guidelines remain a future priority.

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