Propagation disturbance of motor unit action potentials during transient paresis in generalized myotonia: a high-density surface EMG study.
Publication year
2001Source
Brain, 124, Pt 2, (2001), pp. 352--60ISSN
Publication type
Article / Letter to editor

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Organization
Neurology
Haematology
Journal title
Brain
Volume
vol. 124
Issue
iss. Pt 2
Page start
p. 352-
Page end
p. 60
Subject
Neuromuscular and neurometabolic disorders; Pathophysiology of Brain and Behaviour; Neuromusculaire en neurometabole aandoeningen; Pathofysiologie van Hersenen en GedragAbstract
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.
This item appears in the following Collection(s)
- Academic publications [205084]
- Faculty of Medical Sciences [81053]
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