Motor unit reserve capacity in spinal muscular atrophy during fatiguing endurance performance

Objective: To investigate the availability of any motor unit reserve capacity during fatiguing endurance testing in patients with spinal muscular atrophy (SMA). Methods: We recorded surface electromyography (sEMG) of various muscles of upperand lower extremities of 70 patients with SMA types 2-4 and 19 healthy controls performing endurance shuttle tests (ESTs) of arm and legs. We quantitatively evaluated the development of fatigability and motor unit recruitment using time courses of median frequencies and amplitudes of sEMG signals. Linear mixed effect statistical models were used to evaluate group differences in median frequency and normalized amplitude at onset and its time course. Results: Normalized sEMG amplitudes at onset of upper body ESTs were significantly higher in patients compared to controls, yet submaximal when related to maximal voluntary contractions, and showed an inverse correlation to SMA phenotype. sEMG median frequencies decreased and amplitudes increased in various muscles during execution of ESTs in patients and controls. Conclusions: Decreasing median frequencies and increasing amplitudes reveal motor unit reserve capacity in individual SMA patients during ESTs at submaximal performance intensities. Significance: Preserving, if not expanding motor unit reserve capacity may present a potential therapeutic target in clinical care to reduce fatigability in individual patients with SMA. (C) 2021 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Automated summary

The study investigated motor unit reserve capacity in SMA patients during submaximal performance intensities in endurance shuttle tests. The findings showed decreased median frequencies and increased amplitudes in patients, indicating potential therapeutic targets for reducing fatigability in individual SMA patients. Differences in sEMG responses were observed between patients and healthy controls during endurance testing.