Kowalczyk Edyta E, Koszewicz Magdalena A, Budrewicz Sławomir P, Podemski Ryszard, Słotwiński Krzysztof
Z Kliniki Neurologii Akademii Medycznej im. Piastów Slaskich, Wrocławiu.
Wiad Lek. 2006;59(7-8):560-2.
Lance-Adams syndrome, described in 1963, is caused by anoxia of central nervous system, generally in the course of primary respiratory failure. It is characterized mainly by action myoclonus, associated cerebellar ataxia and very mild intellectual deficit. Occurrence of Lance-Adams syndrome is rare; about 100 cases have been described yet. The authors present the case of Lance-Adams syndrome in 36-year-old woman with many years' bronchial asthma. Three times acute cardiopulmonary arrest appeared during status asthmaticus. After successful cardiopulmonary resuscitation action myoclonus developed with cerebellar syndrome, aphonia, dysphagia and generalized convulsive seizures of tonic-clonic type. Electroencephalography showed polyspikes and complex of polyspikes-slow wave, synchronized with myoclonus. CT of the brain was normal. Action myoclonus responded appropriately to sodium valproate. The authors indicate the importance of the correct diagnosis and proper treatment.
兰斯-亚当斯综合征于1963年被描述,由中枢神经系统缺氧引起,通常发生在原发性呼吸衰竭过程中。其主要特征为动作性肌阵挛、伴发的小脑共济失调和非常轻微的智力缺陷。兰斯-亚当斯综合征的发病率很低;迄今约有100例相关病例被报道。作者报告了一名36岁患有多年支气管哮喘的女性患兰斯-亚当斯综合征的病例。在哮喘持续状态期间出现了三次急性心肺骤停。成功进行心肺复苏后,出现了动作性肌阵挛,并伴有小脑综合征、失音、吞咽困难和全身性强直阵挛性惊厥发作。脑电图显示多棘波以及多棘波-慢波综合波,与肌阵挛同步。脑部CT检查正常。动作性肌阵挛对丙戊酸钠反应良好。作者指出了正确诊断和恰当治疗的重要性。