Witt Agnes, Bostock Hugh, Z'Graggen Werner J, Tan S Veronica, Kristensen Alexander Gramm, Kristensen Rikke Søgaard, Larsen Lotte Hardbo, Zeppelin Zennia, Tankisi Hatice
Department of Clinical Neurophysiology, Aarhus University Hospital.
UCL Queen Square Institute of Neurology, Queen Square House.
J Vis Exp. 2020 Feb 19(156). doi: 10.3791/60788.
Although conventional nerve conduction studies (NCS) and electromyography (EMG) are suitable for the diagnosis of neuromuscular disorders, they provide limited information about muscle fiber membrane properties and underlying disease mechanisms. Muscle velocity recovery cycles (MVRCs) illustrate how the velocity of a muscle action potential depends on the time after a preceding action potential. MVRCs are closely related to changes in membrane potential that follow an action potential, thereby providing information about muscle fiber membrane properties. MVRCs may be recorded quickly and easily by direct stimulation and recording from multi-fiber bundles in vivo. MVRCs have been helpful in understanding disease mechanisms in several neuromuscular disorders. Studies in patients with channelopathies have demonstrated the different effects of specific ion channel mutations on muscle excitability. MVRCs have been previously tested in patients with neurogenic muscles. In this prior study, muscle relative refraction period (MRRP) was prolonged, and early supernormality (ESN) and late supernormality (LSN) were reduced in patients compared to healthy controls. Thereby, MVRCs can provide in vivo evidence of membrane depolarization in intact human muscle fibers that underlie their reduced excitability. The protocol presented here describes how to record MVRCs and analyze the recordings. MVRCs can serve as a fast, simple, and useful method for revealing disease mechanisms across a broad range of neuromuscular disorders.
虽然传统的神经传导研究(NCS)和肌电图(EMG)适用于神经肌肉疾病的诊断,但它们提供的关于肌纤维膜特性和潜在疾病机制的信息有限。肌肉速度恢复周期(MVRCs)说明了肌肉动作电位的速度如何取决于前一个动作电位后的时间。MVRCs与动作电位后膜电位的变化密切相关,从而提供有关肌纤维膜特性的信息。通过在体内对多纤维束进行直接刺激和记录,可以快速轻松地记录MVRCs。MVRCs有助于理解几种神经肌肉疾病的发病机制。对通道病患者的研究表明,特定离子通道突变对肌肉兴奋性有不同影响。MVRCs此前已在神经源性肌肉患者中进行过测试。在这项先前的研究中,与健康对照相比,患者的肌肉相对不应期(MRRP)延长,早期超常期(ESN)和晚期超常期(LSN)缩短。因此,MVRCs可以为完整人类肌纤维中膜去极化提供体内证据,而这种膜去极化是其兴奋性降低的基础。本文介绍的方案描述了如何记录MVRCs并分析记录结果。MVRCs可作为一种快速、简单且有用的方法,用于揭示广泛的神经肌肉疾病的发病机制。