Shear Wave Elastography in Patients with Spinal Muscular Atrophy Types 2 and 3
Introduction This study aimed to investigate selective muscle involvement by shear wave elastography (SWE) in patients with spinal muscular atrophy (SMA) types 2 and 3 and to compare SWE values with magnetic resonance imaging (MRI) in demonstrating muscle involvement.
Methods Seventeen patients with SMA types 2 and3 were included in the study. SWE was used to evaluate stiffness of the upper and lower extremities and paraspinal muscles. Involvement of the paraspinal muscles was evaluated using 1.5-T MRI.
Results Among the upper extremity muscles, SWE values were the highest for the triceps brachii; however, no significant difference was noted (p = 0.23). In post hoc analysis, a significant difference was observed between triceps brachii and biceps brachii (p = 0.003). Patients with a longer disease duration have the highest SWE values for the triceps brachii (r = 0.67, p = 0.003). Among the lower extremity muscles, SWE values for the iliopsoas were significantly higher than the gluteus maximus (p < 0.001). A positive correlation was found between SWE values and MRI scores of paraspinal muscles (r = 0.49, p = 0.045; r = 0.67, p = 0.003).
Conclusion This is the first study to report muscle involvement assessed by SWE in patients with SMA types 2 and 3. Our findings are similar to the presence of selective muscle involvement demonstrated in previous studies, and also SWE and MRI values were similar. SWE is an alternative noninvasive practical method that can be used to demonstrate muscle involvement in patients with SMA, to understand the pathogenesis of segmental involvement, and to guide future treatments or to monitor the effectiveness of existing new treatment options.
Keywordsspinal muscular atrophy - shear wave elastography - magnetic resonance imaging - muscle stiffness
All authors have read and approved the manuscript.
Eingereicht: 05. November 2022
Angenommen: 22. Januar 2023
Accepted Manuscript online:
27. Januar 2023
Artikel online veröffentlicht:
27. Februar 2023
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- 1 Zerres K, Davies KE. 59th ENMC International Workshop: Spinal Muscular Atrophies: recent progress and revised diagnostic criteria 17-19 April 1998, Soestduinen, The Netherlands. Neuromuscul Disord 1999; 9 (04) 272-278
- 2 Schorling DC, Pechmann A, Kirschner J. Advances in treatment of spinal muscular atrophy - new phenotypes, new challenges, new implications for care. J Neuromuscul Dis 2020; 7 (01) 1-13
- 3 Deymeer F, Serdaroğlu P, Poda M, Gülşen-Parman Y, Ozçelik T, Ozdemir C. Segmental distribution of muscle weakness in SMA III: implications for deterioration in muscle strength with time. Neuromuscul Disord 1997; 7 (08) 521-528
- 4 Merlini L, Bertini E, Minetti C. et al. Motor function-muscle strength relationship in spinal muscular atrophy. Muscle Nerve 2004; 29 (04) 548-552
- 5 Wilbourn AJ, Aminoff MJ. AAEE minimonograph #32: the electrophysiologic examination in patients with radiculopathies. Muscle Nerve 1988; 11 (11) 1099-1114
- 6 Deymeer F, Serdaroglu P, Parman Y, Poda M. Natural history of SMA IIIb: muscle strength decreases in a predictable sequence and magnitude. Neurology 2008; 71 (09) 644-649
- 7 Caliskan E, Akkoc O, Bayramoglu Z. et al. Effects of static stretching duration on muscle stiffness and blood flow in the rectus femoris in adolescents. Med Ultrason 2019; 21 (02) 136-143
- 8 Akkoc O, Caliskan E, Bayramoglu Z. Effects of passive muscle stiffness measured by shear wave elastography, muscle thickness, and body mass index on athletic performance in adolescent female basketball players. Med Ultrason 2018; 20 (02) 170-176
- 9 Taljanovic MS, Gimber LH, Becker GW. et al. Shear-wave elastography: basic physics and musculoskeletal applications. Radiographics 2017; 37 (03) 855-870
- 10 Zaidman CM, Malkus EC, Connolly AM. Muscle ultrasound quantifies disease progression over time in infants and young boys with Duchenne muscular dystrophy. Muscle Nerve 2015; 52 (03) 334-338
- 11 Berko NS, Hay A, Sterba Y, Wahezi D, Levin TL. Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?. Pediatr Radiol 2015; 45 (10) 1522-1528
- 12 Jacobson JA. Musculoskeletal ultrasound: focused impact on MRI. AJR Am J Roentgenol 2009; 193 (03) 619-627
- 13 Munsat TL, Davies KE. International SMA consortium meeting. (26-28 June 1992, Bonn, Germany). Neuromuscul Disord 1992; 2 (5-6): 423-428
- 14 Finkel R, Bertini E, Muntoni F, Mercuri E. ENMC SMA Workshop Study Group. 209th ENMC International Workshop: Outcome Measures and Clinical Trial Readiness in Spinal Muscular Atrophy 7–9 November 2014, Heemskerk, The Netherlands. Neuromuscul Disord 2015; 25 (07) 593-602
- 15 Durmus H, Yilmaz R, Gulsen-Parman Y. et al. Muscle magnetic resonance imaging in spinal muscular atrophy type 3: selective and progressive involvement. Muscle Nerve 2017; 55 (05) 651-656
- 16 Janssen MMHP, Peeters LHC, de Groot IJM. Quantitative description of upper extremity function and activity of people with spinal muscular atrophy. J Neuroeng Rehabil 2020; 17: 1-13
- 17 Wadman RI, Wijngaarde CA, Stam M. et al. Muscle strength and motor function throughout life in a cross-sectional cohort of 180 patients with spinal muscular atrophy types 1c-4. Eur J Neurol 2018; 25 (03) 512-518
- 18 Mercuri E, Pichiecchio A, Allsop J, Messina S, Pane M, Muntoni F. Muscle MRI in inherited neuromuscular disorders: past, present, and future. J Magn Reson Imaging 2007; 25 (02) 433-440
- 19 Quijano-Roy S, Avila-Smirnow D, Carlier RY. WB-MRI muscle study group. Whole body muscle MRI protocol: pattern recognition in early onset NM disorders. Neuromuscul Disord 2012; 22 (Suppl. 02) S68-S84
- 20 Brogna C, Cristiano L, Verdolotti T. et al. MRI patterns of muscle involvement in type 2 and 3 spinal muscular atrophy patients. J Neurol 2020; 267 (04) 898-912
- 21 Koppenhaver SL, Scutella D, Sorrell BA. et al. Normative parameters and anthropometric variability of lumbar muscle stiffness using ultrasound shear-wave elastography. Clin Biomech (Bristol, Avon) 2019; 62: 113-120
- 22 Liu X, Yu HK, Sheng SY. et al. Quantitative evaluation of passive muscle stiffness by shear wave elastography in healthy individuals of different ages. Eur Radiol 2021; 31 (05) 3187-3194
- 23 Chen J, O'Dell M, He W, Du LJ, Li PC, Gao J. Ultrasound shear wave elastography in the assessment of passive biceps brachii muscle stiffness: influences of sex and elbow position. Clin Imaging 2017; 45: 26-29
- 24 Green MA, Geng G, Qin E, Sinkus R, Gandevia SC, Bilston LE. Measuring anisotropic muscle stiffness properties using elastography. NMR Biomed 2013; 26 (11) 1387-1394
- 25 Creze M, Bedretdinova D, Soubeyrand M. et al. Posture-related stiffness mapping of paraspinal muscles. J Anat 2019; 234 (06) 787-799
- 26 Pichiecchio A, Alessandrino F, Bortolotto C. et al. Muscle ultrasound elastography and MRI in preschool children with Duchenne muscular dystrophy. Neuromuscul Disord 2018; 28 (06) 476-483