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. 2023 Nov;270(11):5398-5407.
doi: 10.1007/s00415-023-11867-z. Epub 2023 Jul 17.

Evaluation of myotonometry for myotonia, muscle stiffness and elasticity in neuromuscular disorders

Katharina Lukas  1 , Kristina Gutschmidt  1 , Benedikt Schoser  2 , Stephan Wenninger  1
Affiliations

Evaluation of myotonometry for myotonia, muscle stiffness and elasticity in neuromuscular disorders

Katharina Lukas et al. J Neurol. 2023 Nov.

Abstract

Neuromuscular disorders show extremely varied expressions of different symptoms and the involvement of muscles. Non-invasively, myotonia and muscle stiffness are challenging to measure objectively. Our study aims to test myotonia, elasticity, and stiffness in various neuromuscular diseases and to provide reference values for different neuromuscular disease groups using a novel handheld non-invasive myometer device MyotonPRO®. We conducted a monocentric blinded cross-sectional study in patients with a set of distinct neuromuscular diseases (NCT04411732, date of registration June 2, 2020). Fifty-two patients in five groups and 21 healthy subjects were enrolled. We evaluated motor function (6-min walk test, handheld dynamometry, Medical Research Council (MRC) Scale) and used ultrasound imaging to assess muscle tissue (Heckmatt scale). We measured muscle stiffness, frequency, decrement, creep, or relaxation using myotonometry with the device MyotonPRO®. Statistically, all values were calculated using the t test and Mann-Whitney U test. No differences were found in comparing the results of myotonometry between healthy and diseased probands. Furthermore, we did not find significant results in all five disease groups regarding myotonometry correlating with muscle strength or ultrasound imaging results. In summary, the myometer MyotonPRO® could not identify significant differences between healthy individuals and neuromuscular patients in our patient collective. Additionally, this device could not distinguish between the five different groups of disorders displaying increased stiffness or decreased muscle tone due to muscle atrophy. In contrast, classic standard muscle tests could clearly decipher healthy controls and neuromuscular patients.

Keywords: Dynamometry; Motoneuron disease; Myotonic dystrophies; Myotonometry; Non-myotonic dystrophies; Ultrasound imaging.

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Conflict of interest statement

All authors report no disclosures regarding this study. Outside of this context, SW and KG have received a research grant from the DGM—Deutsche Gesellschaft für Muskelkranke e.V. KG received travel or speaker Honoraria from Sanofi-Aventis Germany GmbH, Amicus Therapeutics and Kedrion. SW has served on advisory boards for Alexion Pharma, CSL Behring and Sanofi Genzyme GmbH. In addition, he received funding for travel or speaker Honoraria from Sanofi-Aventis Germany GmbH; Amicus Therapeutics; SH Glykogenose Gesellschaft; AbbVie Germany GmbH; Recordati Pharma GmbH; CSL Behring GmbH; Alexion Pharma GmbH; Desitin Germany; Akcea GmbH. Outside of this context, BS has served on advisory boards for Amicus, Argenex, Astellas, and Sanofi; he has undertaken contracted available research for Amicus, Astellas, Roche, DFG, Horizon 2022, AMDA USA, Marigold foundation CA; and has received speaker honoraria from Alexion and Kedrion.

Figures

Fig. 1
Fig. 1
Correlation between MyotonPro® values1 and strength of the biceps brachii muscle on the dominant side. 1Stiffness is not depicted due to clarity reasons
Fig. 1
Fig. 1
Correlation between MyotonPro® values1 and strength of the biceps brachii muscle on the dominant side. 1Stiffness is not depicted due to clarity reasons
See this image and copyright information in PMC

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