Assessment of the reliability of the motor unit size index (MUSIX) in single subject “round-robin” and multi-centre settings

Alix, James J P; Neuwirth, Christoph; Gelder, Lucy C.; Burkhardt, Christian; Castro, Isabel; Carvalho, Mamede de; Gaweł, Malgorzata; Goedee, Stephan; Großkreutz, Julian; Lenglet, Timothée; Moglia, Cristina; Omer, Taha; Schrooten, Maarten; Nandedkar, Sanjeev D.; Stålberg, Erik; Barkhaus, Paul E.; Furtula, Jasna; Dijk, Hans van; Baldinger, Reto; Costa, João; Otto, Marit; Sandberg, Andreas; Weber, Markus

doi:10.1016/j.clinph.2019.01.020

Cited by 15 publications

(18 citation statements)

References 18 publications

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“…ICC results were similar to those reported for other EIM devices (e.g. (McIlduff et al , 2016)) and coefficient of variation results comparable to those reported for other neurophysiological techniques such as the motor unit number/size indices (Neuwirth et al , 2016, Alix et al , 2019.…”

Section: An Additional Limitationsupporting

confidence: 88%

Multi-dimensional electrical impedance myography of the tongue as a potential biomarker for amyotrophic lateral sclerosis

Alix

McDonough

Sonbas

et al. 2020

Clinical Neurophysiology

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In amyotrophic lateral sclerosis (ALS) bulbar disease biomarkers are lacking. We evaluated a novel tongue electrical impedance myography (EIM) system, utilising both 2D and 3D electrode configurations for detection of tongue pathology. MethodsLongitudinal multi-frequency phase angle spectra were recorded from 41 patients with ALS (baseline, 3 and 6 months) and 30 healthy volunteers (baseline and 6 months). . ALS functional rating scalerevised (ALSFRS-R) data and quantitative tongue strength measurements were collected. EIM data were analysed for reliability (intra-class correlation coefficient; ICC) and differences between patients and volunteers ascertained using both univariate (Mann-Whitney U test) and multivariate techniques (feature selection and L2 norm). ResultsThe device produced highly reliable data (pooled ICC: 0.836). Significant EIM differences were apparent between ALS patients and healthy volunteers (P<0.001). EIM data demonstrated a significant relationship to tongue strength and bulbar ALSFRS-R scores (P<0.015). The EIM recordings revealed a group level longitudinal change over 6 months and consistently identified patients in whom symptoms or tongue strength changed. ConclusionsThe novel EIM tongue system produces reliable data and can differentiate between healthy muscle and ALS-related disease. SignificanceTongue EIM utilising multiple frequencies and electrode configurations has potential as a bulbar disease biomarker in ALS. HighlightsNovel electrical impedance myography system uses electrodes on both surfaces of the tongue.The device produces reliable data, even in inexperienced hands.The results differentiate between healthy and ALS muscle and reveal change over time.

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Section: An Additional Limitationsupporting

confidence: 88%

Multi-dimensional electrical impedance myography of the tongue as a potential biomarker for amyotrophic lateral sclerosis

Alix

McDonough

Sonbas

et al. 2020

Clinical Neurophysiology

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“…Calculation of the MUNIX also generates another index, the motor unit size index (MUSIX), which may reflect the reinnervation process in neuromuscular disorders . Recently, a multicenter study showed that the MUSIX was a reliable neurophysiological biomarker of reinnervation …”

Section: Introductionmentioning

confidence: 99%

Reference values for the motor unit number index and the motor unit size index in five muscles

Cao

Zhang

et al. 2020

Muscle and Nerve

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Introduction: Few reference values have been established for the motor unit number index (MUNIX) and motor unit size index (MUSIX). In this study we aimed to investigate the features of MUNIX in healthy individuals for five muscles. Methods:We measured the MUNIX in the right abductor pollicis brevis (APB), abductor digiti minimi (ADM), biceps brachii (BB), tibialis anterior (TA), and trapezius (TR) muscles. Results:The study enrolled 76 male and 74 female participants. The mean MUNIX and mean MUSIX of the right APB, ADM, BB, TA, and TR muscles were 191.0

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“…Although the medians in TA increased slightly, they remained at the same level especially compared to the increase of the MUSIX medians in APB with increasing phases. This suggests, since MUSIX is considered a marker of reinnervation 10 , a lower reinnervation rate during increasing disease accumulation in TA compared with APB and ADM.…”

Section: Discussionmentioning

confidence: 98%

“…MUNIX measurements of abductor pollicis brevis (APB), abductor digiti minimi (ADM) and tibialis anterior (TA) muscles were conducted between December 2013 and October 2020 by trained and certified clinical neurophysiologists. Previous observations at our department of neurology and prior studies assume that APB, ADM and TA are the most technically feasible and reliable muscles for this method 8 – 10 . ALSFRS-R were collected in the years 2011 to 2020.…”

Section: Methodsmentioning

confidence: 96%

Motor unit number index (MUNIX) in the D50 disease progression model reflects disease accumulation independently of disease aggressiveness in ALS

Ebersbach

Roediger

Steinbach

et al. 2022

Sci Rep

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The neurophysiological technique motor unit number index (MUNIX) is increasingly used in clinical trials to measure loss of motor units. However, the heterogeneous disease course in amyotrophic lateral sclerosis (ALS) obfuscates robust correlations between clinical status and electrophysiological assessments. To address this heterogeneity, MUNIX was applied in the D50 disease progression model by analyzing disease aggressiveness (D50) and accumulation (rD50 phase) in ALS separately. 237 ALS patients, 45 controls and 22 ALS-Mimics received MUNIX of abductor pollicis brevis (APB), abductor digiti minimi (ADM) and tibialis anterior (TA) muscles. MUNIX significantly differed between controls and ALS patients and between ALS-Mimics and controls. Within the ALS cohort, significant differences between Phase I and II revealed in MUNIX, compound muscle action potential (CMAP) and motor unit size index (MUSIX) of APB as well as in MUNIX and CMAP of TA. For the ADM, significant differences occurred later in CMAP and MUNIX between Phase II and III/IV. In contrast, there was no significant association between disease aggressiveness and MUNIX. In application of the D50 disease progression model, MUNIX can demonstrate disease accumulation already in early Phase I and evaluate effects of therapeutic interventions in future therapeutic trials independent of individual disease aggressiveness.

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Assessment of the reliability of the motor unit size index (MUSIX) in single subject “round-robin” and multi-centre settings

Cited by 15 publications

References 18 publications

Multi-dimensional electrical impedance myography of the tongue as a potential biomarker for amyotrophic lateral sclerosis

Multi-dimensional electrical impedance myography of the tongue as a potential biomarker for amyotrophic lateral sclerosis

Reference values for the motor unit number index and the motor unit size index in five muscles

Motor unit number index (MUNIX) in the D50 disease progression model reflects disease accumulation independently of disease aggressiveness in ALS

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