Werhahn K J, Brown P, Thompson P D, Marsden C D
MRC Human Movement and Balance Unit, National Hospital for Neurology and Neurosurgery, London, England, UK.
Mov Disord. 1997 Mar;12(2):216-20. doi: 10.1002/mds.870120212.
The clinical and neurophysiologic features of 14 patients with chronic posthypoxic myoclonus are presented. Patients were first seen a mean of 2.5 years (range, 2 to 105 months) after the hypoxic event and followed up for 3.7 years (range, 7 to 84 months) thereafter. All patients had had a cardiorespiratory arrest, most caused by an acute asthmatic attack (11 cases). All patients had multifocal action myoclonus. Eleven patients had additional stimulus-sensitive myoclonus. There was late improvement in the myoclonic syndrome and the level of disability in all but one patient. Three patients were eventually able to discontinue antimyoclonic medication, and five patients were able to walk unaided. Cognitive deficits were found in seven patients and were usually mild. Other neurologic deficits were rare. Electrophysiologic investigation confirmed cortical action myoclonus in every case, although this could be combined with cortical reflex myoclonus, an exaggerated startle response, or brainstem reticular reflex myoclonus. We conclude that posthypoxic myoclonus typically consists of multifocal cortical action myoclonus that improves with time. It is only rarely associated with severe additional neurologic deficit.
本文介绍了14例慢性缺氧后肌阵挛患者的临床和神经生理学特征。患者在缺氧事件后首次就诊的平均时间为2.5年（范围为2至105个月），此后随访3.7年（范围为7至84个月）。所有患者均发生过心搏呼吸骤停，多数由急性哮喘发作引起（11例）。所有患者均有多灶性动作性肌阵挛。11例患者还伴有刺激敏感性肌阵挛。除1例患者外，所有患者的肌阵挛综合征和残疾程度均有后期改善。3例患者最终能够停用抗肌阵挛药物，5例患者能够独立行走。7例患者存在认知缺陷，通常较轻。其他神经功能缺损罕见。电生理检查证实每例患者均存在皮质动作性肌阵挛，尽管其可能与皮质反射性肌阵挛、夸张的惊吓反应或脑干网状反射性肌阵挛合并存在。我们得出结论，缺氧后肌阵挛通常由多灶性皮质动作性肌阵挛组成，且随时间推移会有所改善。它很少与严重的其他神经功能缺损相关。
