周围神经过度兴奋综合征

Peripheral nerve hyperexcitability syndromes

周围神经过度兴奋综合征(peripheral nerve hyperexcitability syndromes,PNHS)是一组异质性疾病,临床和神经电生理均有不同程度的神经过度兴奋表现。主要包括Isaacs综合征、Morvan综合征和痉挛-束颤综合征,其中Isaacs综合征最常见。常以肌肉抽搐、痉挛、僵硬和神经性疼痛为主要表现。针电极肌电图对PNHS的诊断具有重要作用,可发现肌颤搐、神经性肌强直、痉挛电位和束颤电位等。文中就PNHS的临床表型、病理生理学、神经电生理特点及治疗进展进行综述。

Peripheral nerve hyperexcitability syndromes(PNHS)encompass a spectrum of a heterogeneous condition with clinical as well as electrophysiological manifestations of peripheral nerve hyperexcitability.The PNHS consist of Isaacs syndrome,Morvan syndrome and Cramp-fasciculation syndrome,which cause widespread symptoms and signs while without evident peripheral nerve disease.Probably the most well-known condition of PNHS is Isaacs syndrome,often called acquired neuromyotonia.Clinical symptoms of PNHS are characterized by muscle twitching,cramps,stiffness,and neuropathic pain.The electrophysiological findings that are very useful in the diagnosis of PNHS are spontaneous myokymic,neuromyotonic,and cramp discharges.An overview of the history,clinical manifestations,pathophysiology,electrophysiological findings and management of PNHS is presented.