Muscle velocity recovery cycles to examine muscle membrane properties

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DOI

  • Agnes Witt
  • Hugh Bostock, Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London
  • ,
  • Werner J. Z'graggen, University of Bern
  • ,
  • S. Veronica Tan, Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London
  • ,
  • Alexander Gramm Kristensen
  • Rikke Søgaard Kristensen
  • ,
  • Lotte Hardbo Larsen
  • ,
  • Zennia Zeppelin
  • ,
  • Hatice Tankisi

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.

OriginalsprogEngelsk
Artikelnummere60788
TidsskriftJournal of Visualized Experiments
Nummer156
Antal sider6
ISSN1940-087X
DOI
StatusUdgivet - 2020

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