Sič Aleksandar
Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
Clin EEG Neurosci. 2025 Jul 10:15500594251358581. doi: 10.1177/15500594251358581.
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.
背景
兰斯-亚当斯综合征(LAS)是缺氧性脑损伤的一种罕见神经并发症,其特征是意识恢复的患者出现动作和刺激敏感的肌阵挛。尽管LAS常被误解为预后不良的迹象,但它可导致严重的长期残疾,但通过适当管理也显示出功能恢复的潜力。
方法
采用预定义的检索策略,在PubMed和ScienceDirect数据库中进行叙述性综述,以识别2000年1月至2025年4月期间发表的关于LAS的同行评审研究。最终综合纳入了47项研究,包括34篇病例报告、7篇叙述性或范围综述、2篇系统综述和4篇原始临床研究。
结果
LAS的病理生理学仍然是多因素的,涉及皮质兴奋性过高、皮质下抑制解除和神经递质失衡。脑电图和影像学研究提供了重要的诊断线索。基于经验证据,管理在很大程度上仍然是对症治疗。氯硝西泮和丙戊酸盐仍然是一线治疗药物,而诸如吡仑帕奈、γ-羟基丁酸钠、大麻二酚和鞘内注射巴氯芬等药物已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关键作用。
结论
对LAS有更高的认识和更早的识别可以提高诊断准确性和治疗效果。尽管LAS罕见,但临床医生在适当诊断和管理时应警惕其作为一种潜在可逆转的疾病。标准化治疗指南仍然是未来的优先事项。