Enhanced low-threshold motor unit capacity during endurance tasks in patients with spinal muscular atrophy using pyridostigmine.

Autor: Habets LE; Child Development and Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, P.O. Box 85090, 3508 AB Utrecht, The Netherlands., Bartels B; Child Development and Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, P.O. Box 85090, 3508 AB Utrecht, The Netherlands. Electronic address: b.bartels-4@umcutrecht.nl., Jeneson JAL; Child Development and Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, P.O. Box 85090, 3508 AB Utrecht, The Netherlands., Asselman FL; UMC Utrecht Brain Center, Department of Neurology and Neurosurgery, University Medical Center Utrecht, Utrecht University, P.O. Box 85500, 3508 GA Utrecht, The Netherlands., Stam M; UMC Utrecht Brain Center, Department of Neurology and Neurosurgery, University Medical Center Utrecht, Utrecht University, P.O. Box 85500, 3508 GA Utrecht, The Netherlands., Wijngaarde CA; UMC Utrecht Brain Center, Department of Neurology and Neurosurgery, University Medical Center Utrecht, Utrecht University, P.O. Box 85500, 3508 GA Utrecht, The Netherlands., Wadman RI; UMC Utrecht Brain Center, Department of Neurology and Neurosurgery, University Medical Center Utrecht, Utrecht University, P.O. Box 85500, 3508 GA Utrecht, The Netherlands., van Eijk RPA; UMC Utrecht Brain Center, Department of Neurology and Neurosurgery, University Medical Center Utrecht, Utrecht University, P.O. Box 85500, 3508 GA Utrecht, The Netherlands; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, P.O. Box 85500 Utrecht, the Netherlands., Stegeman DF; Faculty of Behavioral and Movement Sciences, Vrije Universiteit Amsterdam, Van der, Boechorststraat 7, 1081 BT Amsterdam, The Netherlands., Ludo van der Pol W; UMC Utrecht Brain Center, Department of Neurology and Neurosurgery, University Medical Center Utrecht, Utrecht University, P.O. Box 85500, 3508 GA Utrecht, The Netherlands.
Jazyk: angličtina
Zdroj: Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology [Clin Neurophysiol] 2023 Oct; Vol. 154, pp. 100-106. Date of Electronic Publication: 2023 Jul 20.
DOI: 10.1016/j.clinph.2023.06.024
Abstrakt: Objective: To investigate the electrophysiological basis of pyridostigmine enhancement of endurance performance documented earlier in patients with spinal muscular atrophy (SMA).
Methods: We recorded surface electromyography (sEMG) in four upper extremity muscles of 31 patients with SMA types 2 and 3 performing endurance shuttle tests (EST) and maximal voluntary contraction (MVC) measurements during a randomized, double blind, cross-over, phase II trial. Linear mixed effect models (LMM) were used to assess the effect of pyridostigmine on (i) time courses of median frequencies and of root mean square (RMS) amplitudes of sEMG signals and (ii) maximal RMS amplitudes during MVC measurements. These sEMG changes over time indicate levels of peripheral muscle fatigue and recruitment of new motor units, respectively.
Results: In comparison to a placebo, patients with SMA using pyridostigmine had fourfold smaller decreases in frequency and twofold smaller increases in amplitudes of sEMG signals in some muscles, recorded during ESTs (p < 0.05). We found no effect of pyridostigmine on MVC RMS amplitudes.
Conclusions: sEMG parameters indicate enhanced low-threshold (LT) motor unit (MU) function in upper-extremity muscles of patients with SMA treated with pyridostigmine. This may underlie their improved endurance.
Significance: Our results suggest that enhancing LT MU function may constitute a therapeutic strategy to reduce fatigability in patients with SMA.
(Copyright © 2023 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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