{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:23:26Z","timestamp":1760145806372,"version":"build-2065373602"},"reference-count":50,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2024,9,8]],"date-time":"2024-09-08T00:00:00Z","timestamp":1725753600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"PATE program of the medical faculty of the University of T\u00fcbingen","award":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"],"award-info":[{"award-number":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"]}]},{"name":"clinician-scientist program of the medical faculty of the University of T\u00fcbingen","award":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"],"award-info":[{"award-number":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"]}]},{"name":"Open Access Publishing Fund of the University of T\u00fcbingen","award":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"],"award-info":[{"award-number":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"]}]},{"name":"Deutsche Forschungsgemeinschaft (DFG)\u2014German Research Foundation","award":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"],"award-info":[{"award-number":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"]}]},{"name":"Bundesministerium f\u00fcr Bildung und Forschung (BMBF, Federal Ministry of Education and Research) through the project \u201d3DFoot\u201d","award":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"],"award-info":[{"award-number":["3037-0-0","45800","GRK 2198\u2014277536708","01EC1907B"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>There is no general consensus on evaluating disease progression in facioscapulohumeral muscular dystrophy (FSHD). Recently, shear wave elastography (SWE) has been proposed as a noninvasive diagnostic tool to assess muscle stiffness in vivo. Therefore, this study aimed to characterize biceps brachii (BB) muscle mechanics in mild-FSHD patients using SWE. Eight patients with mild FSHD, the BB were assessed using SWE, surface electromyography (sEMG), elbow moment measurements during rest, maximum voluntary contraction (MVC), and isometric ramp contractions at 25%, 50%, and 75% MVC across five elbow positions (60\u00b0, 90\u00b0, 120\u00b0, 150\u00b0, and 180\u00b0 flexion). The mean absolute percentage deviation (MAPD) was analyzed as a measure of force control during ramp contractions. The shear elastic modulus of the BB in FSHD patients increased from flexed to extended elbow positions (e.g., p &lt; 0.001 at 25% MVC) and with increasing contraction intensity (e.g., p &lt; 0.001 at 60\u00b0). MAPD was highly variable, indicating significant deviation from target values during ramp contractions. SWE in mild FSHD is influenced by contraction level and joint angle, similar to findings of previous studies in healthy subjects. Moreover, altered force control could relate to the subjective muscle weakness reported by patients with dystrophies.<\/jats:p>","DOI":"10.3390\/diagnostics14171985","type":"journal-article","created":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T03:04:18Z","timestamp":1725851058000},"page":"1985","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Characterizing Mechanical Changes in the Biceps Brachii Muscle in Mild Facioscapulohumeral Muscular Dystrophy Using Shear Wave Elastography"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7575-0020","authenticated-orcid":false,"given":"Benedict","family":"Kleiser","sequence":"first","affiliation":[{"name":"Department of Epileptology, Hertie-Institute for Clinical Brain Research, University of T\u00fcbingen, Hoppe-Seyler-Str. 3, 72076 T\u00fcbingen, Germany"},{"name":"Department of Neural Dynamics and Magnetoencephalography, Hertie-Institute for Clinical Brain Research, University of T\u00fcbingen, Otfried-M\u00fcller-Str. 25, 72076 T\u00fcbingen, Germany"},{"name":"MEG-Center, University of T\u00fcbingen, Otfried-M\u00fcller-Str. 47, 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2405-863X","authenticated-orcid":false,"given":"Manuela","family":"Zimmer","sequence":"additional","affiliation":[{"name":"Institute of Structural Mechanics and Dynamics in Aerospace Engineering, University of Stuttgart, Pfaffenwaldring 27, 70569 Stuttgart, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5634-3913","authenticated-orcid":false,"given":"Filiz","family":"Ate\u015f","sequence":"additional","affiliation":[{"name":"Institute of Structural Mechanics and Dynamics in Aerospace Engineering, University of Stuttgart, Pfaffenwaldring 27, 70569 Stuttgart, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2039-5498","authenticated-orcid":false,"given":"Justus","family":"Marquetand","sequence":"additional","affiliation":[{"name":"Department of Epileptology, Hertie-Institute for Clinical Brain Research, University of T\u00fcbingen, Hoppe-Seyler-Str. 3, 72076 T\u00fcbingen, Germany"},{"name":"Department of Neural Dynamics and Magnetoencephalography, Hertie-Institute for Clinical Brain Research, University of T\u00fcbingen, Otfried-M\u00fcller-Str. 25, 72076 T\u00fcbingen, Germany"},{"name":"MEG-Center, University of T\u00fcbingen, Otfried-M\u00fcller-Str. 47, 72076 T\u00fcbingen, Germany"},{"name":"Institute for Modelling and Simulation of Biomechanical Systems, University of Stuttgart, Pfaffenwaldring 5a, 70569 Stuttgart, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1002\/mus.27951","article-title":"The Facioscapulohumeral Muscular Dystrophy-Health Index: Development and evaluation of a disease-specific outcome measure","volume":"68","author":"Varma","year":"2023","journal-title":"Muscle Nerve"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1617","DOI":"10.1007\/s00421-021-04650-3","article-title":"Increased resistance towards fatigability in patients with facioscapulohumeral muscular dystrophy","volume":"121","author":"Calanni","year":"2021","journal-title":"Eur. J. Appl. Physiol."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Ghasemi, M., Emerson, C.P., and Hayward, L.J. (2022). Outcome Measures in Facioscapulohumeral Muscular Dystrophy Clinical Trials. Cells, 11.","DOI":"10.3390\/cells11040687"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2646","DOI":"10.1007\/s00415-018-9037-y","article-title":"Quantitative muscle MRI and ultrasound for facioscapulohumeral muscular dystrophy: Complementary imaging biomarkers","volume":"265","author":"Mul","year":"2018","journal-title":"J. Neurol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e1488","DOI":"10.1212\/WNL.0000000000009211","article-title":"Muscle ultrasound is a responsive biomarker in facioscapulohumeral dystrophy","volume":"94","author":"Goselink","year":"2020","journal-title":"Neurology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1002\/mus.21015","article-title":"Muscle ultrasound in neuromuscular disorders","volume":"37","author":"Pillen","year":"2008","journal-title":"Muscle Nerve"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1002\/mus.27081","article-title":"Muscle ultrasound: Present state and future opportunities","volume":"63","author":"Wijntjes","year":"2021","journal-title":"Muscle Nerve"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1002\/mus.24651","article-title":"Reachable workspace reflects dynamometer-measured upper extremity strength in facioscapulohumeral muscular dystrophy","volume":"52","author":"Han","year":"2015","journal-title":"Muscle Nerve"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1249\/JES.0000000000000049","article-title":"Elastography for Muscle Biomechanics: Toward the Estimation of Individual Muscle Force","volume":"43","author":"Hug","year":"2015","journal-title":"Exerc. Sport Sci. Rev."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1002\/mus.24445","article-title":"Non-invasive assessment of muscle stiffness in patients with Duchenne muscular dystrophy","volume":"51","author":"Lacourpaille","year":"2015","journal-title":"Muscle Nerve"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Zimmer, M., Kleiser, B., Marquetand, J., and Ate\u015f, F. (2022). Shear wave elastography characterizes passive and active mechanical properties of biceps brachii muscle in vivo. J. Mech. Behav. Biomed. Mater., 137.","DOI":"10.2139\/ssrn.4101072"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1007\/s00421-018-3798-y","article-title":"Passive stiffness of monoarticular lower leg muscles is influenced by knee joint angle","volume":"118","author":"Andrade","year":"2018","journal-title":"Eur. J. Appl. Physiol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1016\/j.jelekin.2015.02.005","article-title":"Muscle shear elastic modulus is linearly related to muscle torque over the entire range of isometric contraction intensity","volume":"25","author":"Hug","year":"2015","journal-title":"J. Electromyogr. Kinesiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1016\/j.ultrasmedbio.2018.03.026","article-title":"Muscle Shear Wave Elastography in Inclusion Body Myositis: Feasibility, Reliability and Relationships with Muscle Impairments","volume":"44","author":"Bachasson","year":"2018","journal-title":"Ultrasound Med. Biol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1016\/j.nmd.2018.02.007","article-title":"Muscle ultrasound elastography and MRI in preschool children with Duchenne muscular dystrophy","volume":"28","author":"Pichiecchio","year":"2018","journal-title":"Neuromuscul. Disord."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"2880","DOI":"10.1016\/j.ultrasmedbio.2021.06.008","article-title":"Quantifying Lower Limb Muscle Stiffness as Ambulation Function Declines in Duchenne Muscular Dystrophy with Acoustic Radiation Force Impulse Shear Wave Elastography","volume":"47","author":"Lin","year":"2021","journal-title":"Ultrasound Med. Biol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"106661","DOI":"10.1016\/j.ultsonch.2023.106661","article-title":"Acoustic radiation force impulse shear wave elastography quantifies upper limb muscle in patients with Duchenne muscular dystrophy","volume":"101","author":"Lin","year":"2023","journal-title":"Ultrason. Sonochem."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1016\/j.nmd.2022.05.004","article-title":"Assessment of the upper limb muscles in patients with Fukuyama muscular dystrophy: Noninvasive assessment using visual ultrasound muscle analysis and shear wave elastography","volume":"32","author":"Harada","year":"2022","journal-title":"Neuromuscul. Disord"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1445","DOI":"10.1152\/jappl.1999.86.5.1445","article-title":"Length dependence of active force production in skeletal muscle","volume":"86","author":"Rassier","year":"1999","journal-title":"J. Appl. Physiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.jbiomech.2019.06.018","article-title":"Influence of low muscle activation levels on the ankle torque and muscle shear modulus during plantar flexor stretching","volume":"93","author":"Gross","year":"2019","journal-title":"J. Biomech."},{"key":"ref_21","doi-asserted-by":"crossref","unstructured":"Zimmer, M., Kleiser, B., Marquetand, J., and Ates, F. (2023). Characterization of Muscle Weakness Due to Myasthenia Gravis Using Shear Wave Elastography. Diagnostics, 13.","DOI":"10.3390\/diagnostics13061108"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.clinph.2022.04.013","article-title":"A scoping review of methods used in musculoskeletal soft tissue and nerve shear wave elastography studies","volume":"140","author":"Cipriano","year":"2022","journal-title":"Clin. Neurophysiol."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Romano, A., Staber, D., Grimm, A., Kronlage, C., and Marquetand, J. (2021). Limitations of Muscle Ultrasound Shear Wave Elastography for Clinical Routine-Positioning and Muscle Selection. Sensors, 21.","DOI":"10.3390\/s21248490"},{"key":"ref_24","unstructured":"MRC (1976). Aids to the Examination of the Peripheral Nervous System, HMSO."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"2838","DOI":"10.1093\/brain\/awq270","article-title":"Aids to the Investigation of Peripheral Nerve Injuries. Medical Research Council: Nerve Injuries Research Committee. His Majesty\u2019s Stationery Office: 1942; pp. 48 (iii) and 74 figures and 7 diagrams; with Aids to the Examination of the Peripheral Nervous System. By Michael O\u2019Brien for the Guarantors of Brain. Saunders Elsevier: 2010; pp. [8] 64 and 94 Figures","volume":"133","author":"Compston","year":"2010","journal-title":"Brain"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"102440","DOI":"10.1016\/j.jelekin.2020.102440","article-title":"Tutorial. Surface EMG detection, conditioning and pre-processing: Best practices","volume":"54","author":"Merletti","year":"2020","journal-title":"J. Electromyogr. Kinesiol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/S1050-6411(00)00027-4","article-title":"Development of recommendations for SEMG sensors and sensor placement procedures","volume":"10","author":"Hermens","year":"2000","journal-title":"J. Electromyogr. Kinesiol."},{"key":"ref_28","first-page":"100071","article-title":"Reliability and Validity of the Modified Heckmatt Scale in Evaluating Muscle Changes with Ultrasound in Spasticity","volume":"2","author":"Moreta","year":"2020","journal-title":"Arch. Rehabil. Res. Clin. Transl."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1016\/j.ultrasmedbio.2021.09.003","article-title":"Performance of Passive Muscle Stiffness in Diagnosis and Assessment of Disease Progression in Duchenne Muscular Dystrophy","volume":"48","author":"Yu","year":"2022","journal-title":"Ultrasound Med. Biol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1353","DOI":"10.1152\/japplphysiol.00858.2012","article-title":"Shear elastic modulus can be used to estimate an index of individual muscle force during a submaximal isometric fatiguing contraction","volume":"113","author":"Bouillard","year":"2012","journal-title":"J. Appl. Physiol. (1985)"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1152\/japplphysiol.01058.2013","article-title":"Length-force characteristics of in vivo human muscle reflected by supersonic shear imaging","volume":"117","author":"Sasaki","year":"2014","journal-title":"J. Appl. Physiol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1088\/0967-3334\/34\/6\/713","article-title":"Length and activation dependent variations in muscle shear wave speed","volume":"34","author":"Chernak","year":"2013","journal-title":"Physiol. Meas."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1152\/japplphysiol.00112.2019","article-title":"Shear wave velocity is sensitive to changes in muscle stiffness that occur independently from changes in force","volume":"128","author":"Bernabei","year":"2020","journal-title":"J. Appl. Physiol. (1985)"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"2403","DOI":"10.1121\/10.0010110","article-title":"The combined importance of finite dimensions, anisotropy, and pre-stress in acoustoelastography","volume":"151","author":"Crutison","year":"2022","journal-title":"J. Acoust. Soc. Am."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"4775","DOI":"10.1093\/rheumatology\/keac196","article-title":"Muscle involvement in systemic lupus erythematosus: Multimodal ultrasound assessment and relationship with physical performance","volume":"61","author":"Smerilli","year":"2022","journal-title":"Rheumatology"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1007\/s00256-019-03175-3","article-title":"Muscle shear wave elastography in idiopathic inflammatory myopathies: A case-control study with MRI correlation","volume":"48","author":"Alfuraih","year":"2019","journal-title":"Skeletal. Radiol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1007\/s11910-016-0667-0","article-title":"Facioscapulohumeral Dystrophy","volume":"16","author":"Wang","year":"2016","journal-title":"Curr. Neurol. Neurosci. Rep."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"3187","DOI":"10.1007\/s00330-020-07367-7","article-title":"Quantitative evaluation of passive muscle stiffness by shear wave elastography in healthy individuals of different ages","volume":"31","author":"Liu","year":"2021","journal-title":"Eur. Radiol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.clinbiomech.2014.11.011","article-title":"Shear wave elastography of passive skeletal muscle stiffness: Influences of sex and age throughout adulthood","volume":"30","author":"Eby","year":"2015","journal-title":"Clin. Biomech."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1755","DOI":"10.1007\/s40520-019-01139-0","article-title":"The effect of ageing on shear wave elastography muscle stiffness in adults","volume":"31","author":"Alfuraih","year":"2019","journal-title":"Aging Clin. Exp. Res."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"109038","DOI":"10.1016\/j.ejrad.2020.109038","article-title":"Evaluation of effects of aging on skeletal muscle elasticity using shear wave elastography","volume":"128","author":"Cindil","year":"2020","journal-title":"Eur. J. Radiol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"2906","DOI":"10.1016\/j.ultrasmedbio.2015.07.011","article-title":"Age-Related Differences in Muscle Shear Moduli in the Lower Extremity","volume":"41","author":"Akagi","year":"2015","journal-title":"Ultrasound Med. Biol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/0022-510X(68)90003-8","article-title":"The pathology of the muscle spindle. A study of biopsy material in various muscular and neuromuscular diseases","volume":"7","author":"Cazzato","year":"1968","journal-title":"J. Neurol. Sci."},{"key":"ref_44","first-page":"148","article-title":"Morphologic and morphometrical study of the muscle spindle in muscular dystrophy","volume":"29","author":"Kararizou","year":"2007","journal-title":"Anal. Quant. Cytol. Histol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/s13395-020-00258-x","article-title":"Muscle spindle function in healthy and diseased muscle","volume":"11","author":"Watkins","year":"2021","journal-title":"Skelet. Muscle"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1002\/(SICI)1097-4598(199805)21:5<662::AID-MUS18>3.0.CO;2-X","article-title":"Functional involvement of cerebral cortex in Duchenne muscular dystrophy","volume":"21","author":"Restuccia","year":"1998","journal-title":"Muscle Nerve"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"2196","DOI":"10.3174\/ajnr.A2718","article-title":"Decreased gray matter concentration and local synchronization of spontaneous activity in the motor cortex in Duchenne muscular dystrophy","volume":"32","author":"Lv","year":"2011","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1237","DOI":"10.1016\/j.neuroimage.2006.04.226","article-title":"Modifications of brain tissue volumes in facioscapulohumeral dystrophy","volume":"32","author":"Quarantelli","year":"2006","journal-title":"Neuroimage"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.nmd.2003.08.002","article-title":"Changes in motor cortex excitability in facioscapulohumeral muscular dystrophy","volume":"14","author":"Oliviero","year":"2004","journal-title":"Neuromuscul. Disord."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1016\/j.jelekin.2008.08.003","article-title":"A bi-dimensional index for the selective assessment of myoelectric manifestations of peripheral and central muscle fatigue","volume":"19","author":"Mesin","year":"2009","journal-title":"J. Electromyogr. Kinesiol."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/14\/17\/1985\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T15:51:28Z","timestamp":1760111488000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/14\/17\/1985"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,8]]},"references-count":50,"journal-issue":{"issue":"17","published-online":{"date-parts":[[2024,9]]}},"alternative-id":["diagnostics14171985"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics14171985","relation":{},"ISSN":["2075-4418"],"issn-type":[{"type":"electronic","value":"2075-4418"}],"subject":[],"published":{"date-parts":[[2024,9,8]]}}}