Vellieux Geoffroy, Apartis Emmanuelle, Navarro Vincent
Paris Brain Institute, ICM, Inserm, CNRS, Sorbonne Université, Paris F-75013, France.
Assistance Publique-Hôpitaux de Paris, EEG Unit, Department of Neurology, Pitié-Salpêtrière Hospital, Paris F-75013, France.
Brain Commun. 2025 Sep 9;7(5):fcaf329. doi: 10.1093/braincomms/fcaf329. eCollection 2025.
Lance-Adams syndrome, or chronic post-hypoxic myoclonus, is a disabling chronic myoclonic disorder occurring in survivors of brain hypoxic events. Using a systematic methodology for literature search and data acquisition, we extensively reviewed all published cases of Lance-Adams syndrome since the first original patients were described by JW Lance and RD Adams in 1963. We analysed the available data of 272 patients extracted from the 153 included studies to summarize the natural history of Lance-Adams syndrome, outline the full spectrum of this disorder and deepen our understanding of its underlying mechanisms. Two-thirds of patients suffered from a respiratory hypoxic leading event. The main causes of anoxia were peri-surgery and anaesthetic accidents, asthma attacks/bronchospasm, cardiac disorders and intoxications/drug overdoses. Many patients exhibited a 'pure' Lance-Adams syndrome, characterized by multi-focal action-induced myoclonic jerks predominant to distal limbs. Morphologic brain imaging did not show any specific abnormalities. Neurophysiological evaluations, including EEG recordings, polymyography of myoclonus and jerk-locked back averaging of myoclonus, revealed features of cortical myoclonus in the majority of patients. Both EEG, showing epileptiform discharges on the frontal and central median regions, and brain metabolism imaging, showing hypometabolism on the pericentral regions, indicated that myoclonus in Lance-Adams syndrome originates within the motor cortex. Some anti-seizure medications have shown some effectiveness and certain neuromodulation techniques have promising effects.
兰斯-亚当斯综合征，即慢性缺氧后肌阵挛，是一种发生于脑缺氧事件幸存者中的致残性慢性肌阵挛障碍。我们采用系统的文献检索和数据采集方法，广泛回顾了自1963年JW·兰斯和RD·亚当斯首次描述首例原发病例以来所有已发表的兰斯-亚当斯综合征病例。我们分析了从153项纳入研究中提取的272例患者的可用数据，以总结兰斯-亚当斯综合征的自然病史，勾勒出该疾病的全貌，并加深我们对其潜在机制的理解。三分之二的患者经历过呼吸性缺氧引发事件。缺氧的主要原因是围手术期和麻醉意外、哮喘发作/支气管痉挛、心脏疾病以及中毒/药物过量。许多患者表现为“单纯性”兰斯-亚当斯综合征，其特征为多灶性动作诱发性肌阵挛抽搐，以远端肢体为主。脑部形态学成像未显示任何特定异常。神经生理学评估，包括脑电图记录、肌阵挛的多导肌电图以及肌阵挛的抽搐锁定反向平均法，在大多数患者中揭示了皮质肌阵挛的特征。脑电图显示额叶和中央中线区域有癫痫样放电，脑部代谢成像显示中央周围区域代谢减低，这两者均表明兰斯-亚当斯综合征中的肌阵挛起源于运动皮层。一些抗癫痫药物已显示出一定疗效，某些神经调节技术也有显著效果。
