吡仑帕奈改善兰斯-亚当斯综合征的“弹性步态”
Improvement of "Bouncy Gait" in Lance-Adams Syndrome with Perampanel.
作者信息
Lim Shen-Yang, Jasti Dushyanth Babu, Tan Ai Huey
机构信息
Division of Neurology and the Mah Pooi Soo & Tan Chin Nam Centre for Parkinson's & Related Disorders, Faculty of Medicine, University of Malaya, Kuala Lumpur, MYS.
Division of Neurology, Kasturba Medical College, Manipal, IND.
出版信息
Cureus. 2020 Jan 25;12(1):e6773. doi: 10.7759/cureus.6773.
Lance-Adams syndrome (LAS) is chronic post-hypoxic myoclonus that is often associated with sudden lapses in muscle tone (negative myoclonus) in the legs, causing a disabling "bouncy gait." Given its relative rarity, there are no controlled treatment studies of LAS. The majority of cases require polypharmacy management, with an incomplete response. "Bouncy gait," in particular, is notoriously medication-refractory. Here, we report a patient with long-standing LAS who improved markedly when low-dose perampanel was added to his existing treatment regime consisting of clonazepam, levetiracetam, sodium valproate, and acetazolamide.
兰斯-亚当斯综合征（LAS）是一种慢性缺氧后肌阵挛，常伴有腿部肌张力突然下降（阴性肌阵挛），导致致残性的“跳跃步态”。鉴于其相对罕见，目前尚无关于LAS的对照治疗研究。大多数病例需要联合用药治疗，但反应不完全。特别是“跳跃步态”，对药物治疗 notoriously 难治。在此，我们报告一例长期患有LAS的患者，当在其现有的由氯硝西泮、左乙拉西坦、丙戊酸钠和乙酰唑胺组成的治疗方案中添加低剂量的吡仑帕奈时，病情有显著改善。 （注：notoriously 直译为“臭名昭著地”，结合语境这里可意译为“极其、非常”等类似意思）
Zotero 插件