Propagation disturbance of motor unit action potentials during transient paresis in generalized myotonia: a high-density surface EMG study

G Drost, J H Blok, D F Stegeman, J P van Dijk, B G van Engelen, M.J. Zwarts

Research output: Contribution to journalArticleAcademicpeer-review

52 Citations (Scopus)

Abstract

Patients with autosomal recessive generalized myotonia, or Becker's disease, often suffer from a peculiar transient paresis. As yet, the relationship between this transient paresis and the defect in the gene encoding for a voltage gated Cl- channel protein in the muscle membrane of these patients is unclear. In order to gain a better understanding of the electrophysiological properties of the muscle fibre membrane in these generalized myotonia patients, we have studied transient paresis with a novel high-density surface EMG (sEMG) technique. We conclude that the transient paresis is explained by a deteriorating muscle membrane function, ending in conduction block and paresis. Multi-channel sEMG during the period of force decline in transient paresis shows a decrease in peak-peak amplitude of the motor unit action potentials from endplate towards tendon. This disturbance increases with time and place, indicating a deteriorating membrane function, and ends in a complete blocking of propagation within seconds. Spatiotemporally, this leads to a V-shaped sEMG pattern. In a more general sense, this contribution shows how spatiotemporal information, available through non-invasive high-density sEMG, may provide novel insights into electrophysiological aspects of membrane dysfunction.

Original languageEnglish
Pages (from-to)352-360
Number of pages9
JournalBrain
Volume124
Issue numberPt 2
DOIs
Publication statusPublished - Feb 2001
Externally publishedYes

Keywords

  • Action Potentials
  • Adult
  • Electromyography/instrumentation
  • Female
  • Genes, Recessive
  • Humans
  • Male
  • Middle Aged
  • Motor Endplate/physiopathology
  • Muscle Contraction
  • Muscle Fibers, Skeletal
  • Myotonia Congenita/complications
  • Paresis/etiology
  • Stress, Mechanical

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