Brain, Vol. 124, No. 2, 352-360,
February 2001
© 2001 Oxford University Press
Propagation disturbance of motor unit action potentials during transient paresis in generalized myotonia
A high-density surface EMG study
1 Department of Clinical Neurophysiology and 2 Institute of Neurology, University Medical Centre, Nijmegen, The Netherlands, and 3 Motor Research Group, Institute of Pathological Physiology, Friedrich-Schiller University Jena, Germany
Correspondence to:
Professor M. J. Zwarts, MD, Department of Clinical Neurophysiology, University Medical Centre Nijmegen, PO Box 9101, 6500 HB Nijmegen, The Netherlands E-mail: M.Zwarts{at}czzoknf.azn.nl
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 electrophysio- logical aspects of membrane dysfunction.
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