Fasciculations demonstrate daytime consistency in amyotrophic lateral sclerosis

James Bashford; Urooba Masood; Aidan Wickham; Raquel Iniesta; Emmanuel Drakakis; Martyn Boutelle; Kerry Mills; Christopher Shaw

doi:10.1002/mus.26864

Fasciculations demonstrate daytime consistency in amyotrophic lateral sclerosis

James Bashford, Urooba Masood, Aidan Wickham, Raquel Iniesta, Emmanuel Drakakis, Martyn Boutelle, Kerry Mills, Christopher Shaw

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Abstract

Introduction: Fasciculations represent early neuronal hyperexcitability in amyotrophic lateral sclerosis (ALS). To aid calibration as a disease biomarker, we set out to characterize the daytime variability of fasciculation firing. Methods: Fasciculation awareness scores were compiled from 19 ALS patients. In addition, 10 ALS patients prospectively underwent high-density surface electromyographic (HDSEMG) recordings from biceps and gastrocnemius at three time-points during a single day. Results: Daytime fasciculation awareness scores were low (mean: 0.28 muscle groups), demonstrating significant variability (coefficient of variation: 303%). Biceps HDSEMG recordings were highly consistent for fasciculation potential frequency (intraclass correlation coefficient [ICC] = 95%, n = 19) and the interquartile range of fasciculation potential amplitude (ICC = 95%, n = 19). These parameters exhibited robustness to observed fluctuations in data quality parameters. Gastrocnemius demonstrated more modest levels of consistency overall (44% to 62%, n = 20). Discussion: There was remarkable daytime consistency of fasciculation firing in the biceps of ALS patients, despite sparse and intermittent awareness among patients’ accounts.

Original language	English
Journal	Muscle & nerve
Early online date	24 Mar 2020
DOIs	https://doi.org/10.1002/mus.26864
Publication status	E-pub ahead of print - 24 Mar 2020

Keywords

amyotrophic lateral sclerosis
benign fasciculation syndrome
biomarker
fasciculation
high-density surface EMG
neuronal hyperexcitability

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Fasciculations demonstrate daytime consistency_BASHFORD_Accepted16March2020Publishedonline24March2020_GREEN AAMAccepted author manuscript, 1.93 MB

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title = "Fasciculations demonstrate daytime consistency in amyotrophic lateral sclerosis",

abstract = "Introduction: Fasciculations represent early neuronal hyperexcitability in amyotrophic lateral sclerosis (ALS). To aid calibration as a disease biomarker, we set out to characterize the daytime variability of fasciculation firing. Methods: Fasciculation awareness scores were compiled from 19 ALS patients. In addition, 10 ALS patients prospectively underwent high-density surface electromyographic (HDSEMG) recordings from biceps and gastrocnemius at three time-points during a single day. Results: Daytime fasciculation awareness scores were low (mean: 0.28 muscle groups), demonstrating significant variability (coefficient of variation: 303%). Biceps HDSEMG recordings were highly consistent for fasciculation potential frequency (intraclass correlation coefficient [ICC] = 95%, n = 19) and the interquartile range of fasciculation potential amplitude (ICC = 95%, n = 19). These parameters exhibited robustness to observed fluctuations in data quality parameters. Gastrocnemius demonstrated more modest levels of consistency overall (44% to 62%, n = 20). Discussion: There was remarkable daytime consistency of fasciculation firing in the biceps of ALS patients, despite sparse and intermittent awareness among patients{\textquoteright} accounts.",

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author = "James Bashford and Urooba Masood and Aidan Wickham and Raquel Iniesta and Emmanuel Drakakis and Martyn Boutelle and Kerry Mills and Christopher Shaw",

year = "2020",

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journal = "Muscle & nerve",

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AU - Bashford, James

AU - Masood, Urooba

AU - Wickham, Aidan

AU - Iniesta, Raquel

AU - Drakakis, Emmanuel

AU - Boutelle, Martyn

AU - Mills, Kerry

AU - Shaw, Christopher

PY - 2020/3/24

Y1 - 2020/3/24

N2 - Introduction: Fasciculations represent early neuronal hyperexcitability in amyotrophic lateral sclerosis (ALS). To aid calibration as a disease biomarker, we set out to characterize the daytime variability of fasciculation firing. Methods: Fasciculation awareness scores were compiled from 19 ALS patients. In addition, 10 ALS patients prospectively underwent high-density surface electromyographic (HDSEMG) recordings from biceps and gastrocnemius at three time-points during a single day. Results: Daytime fasciculation awareness scores were low (mean: 0.28 muscle groups), demonstrating significant variability (coefficient of variation: 303%). Biceps HDSEMG recordings were highly consistent for fasciculation potential frequency (intraclass correlation coefficient [ICC] = 95%, n = 19) and the interquartile range of fasciculation potential amplitude (ICC = 95%, n = 19). These parameters exhibited robustness to observed fluctuations in data quality parameters. Gastrocnemius demonstrated more modest levels of consistency overall (44% to 62%, n = 20). Discussion: There was remarkable daytime consistency of fasciculation firing in the biceps of ALS patients, despite sparse and intermittent awareness among patients’ accounts.

AB - Introduction: Fasciculations represent early neuronal hyperexcitability in amyotrophic lateral sclerosis (ALS). To aid calibration as a disease biomarker, we set out to characterize the daytime variability of fasciculation firing. Methods: Fasciculation awareness scores were compiled from 19 ALS patients. In addition, 10 ALS patients prospectively underwent high-density surface electromyographic (HDSEMG) recordings from biceps and gastrocnemius at three time-points during a single day. Results: Daytime fasciculation awareness scores were low (mean: 0.28 muscle groups), demonstrating significant variability (coefficient of variation: 303%). Biceps HDSEMG recordings were highly consistent for fasciculation potential frequency (intraclass correlation coefficient [ICC] = 95%, n = 19) and the interquartile range of fasciculation potential amplitude (ICC = 95%, n = 19). These parameters exhibited robustness to observed fluctuations in data quality parameters. Gastrocnemius demonstrated more modest levels of consistency overall (44% to 62%, n = 20). Discussion: There was remarkable daytime consistency of fasciculation firing in the biceps of ALS patients, despite sparse and intermittent awareness among patients’ accounts.

KW - amyotrophic lateral sclerosis

KW - benign fasciculation syndrome

KW - biomarker

KW - fasciculation

KW - high-density surface EMG

KW - neuronal hyperexcitability

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U2 - 10.1002/mus.26864

DO - 10.1002/mus.26864

M3 - Article

SN - 0148-639X

JO - Muscle & nerve

JF - Muscle & nerve

ER -

Fasciculations demonstrate daytime consistency in amyotrophic lateral sclerosis

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Keywords

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