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

Laura E Habets; Bart Bartels; Janke F de Groot; W Ludo van der Pol; Jeroen A L Jeneson; Fay-Lynn Asselman; Ruben P A van Eijk; Dick F Stegeman

doi:10.1016/j.clinph.2020.11.044

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

Laura E Habets, Bart Bartels, Janke F de Groot, W Ludo van der Pol, Jeroen A L Jeneson, Fay-Lynn Asselman, Ruben P A van Eijk, Dick F Stegeman

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

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 upper- and 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.

Original language	English
Pages (from-to)	800-807
Number of pages	8
Journal	Clinical Neurophysiology
Volume	132
Issue number	3
Early online date	28 Jan 2021
DOIs	https://doi.org/10.1016/j.clinph.2020.11.044
Publication status	Published - Mar 2021

Keywords

Exercise
Fatigability
Muscle electrical activation
Spinal muscular atrophy
Surface-EMG

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@article{9c9e0fe9382a410ea32cfeba6e7dc308,

title = "Motor unit reserve capacity in spinal muscular atrophy during fatiguing endurance performance",

abstract = "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 upper- and 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.",

keywords = "Exercise, Fatigability, Muscle electrical activation, Spinal muscular atrophy, Surface-EMG",

author = "Habets, {Laura E} and Bart Bartels and {de Groot}, {Janke F} and {van der Pol}, {W Ludo} and Jeneson, {Jeroen A L} and Fay-Lynn Asselman and {van Eijk}, {Ruben P A} and Stegeman, {Dick F}",

note = "Funding Information: Bart Bartels obtained research grants from Prinses Beatrix Spierfonds and Stichting Spieren voor Spieren, both non-profit foundations. His employer receives fees for SMA-related consultancy activities. W. Ludo van der Pol obtained grants from Prinses Beatrix Spierfonds, Stichting Spieren voor Spieren and Vriendenloterij. All other authors reported no conflict of interest. Funding Information: The study was funded by Prinses Beatrix Spierfonds, Stichting Spieren voor Spieren and Vriendenloterij. These funding sources had no involvement in data collection, data analysis and interpretation, writing of the report nor in the decision to submit the article for publication. Publisher Copyright: {\textcopyright} 2021 International Federation of Clinical Neurophysiology",

year = "2021",

month = mar,

doi = "10.1016/j.clinph.2020.11.044",

language = "English",

volume = "132",

pages = "800--807",

journal = "Clinical Neurophysiology",

issn = "1388-2457",

publisher = "Elsevier Ireland Ltd",

number = "3",

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TY - JOUR

T1 - Motor unit reserve capacity in spinal muscular atrophy during fatiguing endurance performance

AU - Habets, Laura E

AU - Bartels, Bart

AU - de Groot, Janke F

AU - van der Pol, W Ludo

AU - Jeneson, Jeroen A L

AU - Asselman, Fay-Lynn

AU - van Eijk, Ruben P A

AU - Stegeman, Dick F

N1 - Funding Information: Bart Bartels obtained research grants from Prinses Beatrix Spierfonds and Stichting Spieren voor Spieren, both non-profit foundations. His employer receives fees for SMA-related consultancy activities. W. Ludo van der Pol obtained grants from Prinses Beatrix Spierfonds, Stichting Spieren voor Spieren and Vriendenloterij. All other authors reported no conflict of interest. Funding Information: The study was funded by Prinses Beatrix Spierfonds, Stichting Spieren voor Spieren and Vriendenloterij. These funding sources had no involvement in data collection, data analysis and interpretation, writing of the report nor in the decision to submit the article for publication. Publisher Copyright: © 2021 International Federation of Clinical Neurophysiology

PY - 2021/3

Y1 - 2021/3

N2 - 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 upper- and 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.

AB - 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 upper- and 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.

KW - Exercise

KW - Fatigability

KW - Muscle electrical activation

KW - Spinal muscular atrophy

KW - Surface-EMG

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U2 - 10.1016/j.clinph.2020.11.044

DO - 10.1016/j.clinph.2020.11.044

M3 - Article

C2 - 33581592

SN - 1388-2457

VL - 132

SP - 800

EP - 807

JO - Clinical Neurophysiology

JF - Clinical Neurophysiology

IS - 3

ER -

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

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