Neuropediatrics
DOI: 10.1055/a-2021-0403
Original Article

Shear Wave Elastography in Patients with Spinal Muscular Atrophy Types 2 and 3

Burçin Nazlı Karacabey
1   Department of Pediatric Neurology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey
,
Zuhal Bayramoğlu
2   Department of Radiology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey
,
Orhan Coşkun
1   Department of Pediatric Neurology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey
,
Zeynep Nur Akyol Sarı
2   Department of Radiology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey
,
Melis Ulak Özkan
3   Institute of Child Health, Istanbul University, Fatih, Istanbul, Turkey
,
Edibe Pembegül Yıldız
1   Department of Pediatric Neurology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey
2   Department of Radiology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey
3   Institute of Child Health, Istanbul University, Fatih, Istanbul, Turkey
,
Nur Aydınlı
1   Department of Pediatric Neurology, Istanbul Faculty of Medicine, Istanbul University, Fatih, Istanbul, Turkey
,
Mine Çalışkan
3   Institute of Child Health, Istanbul University, Fatih, Istanbul, Turkey
› Author Affiliations

Abstract

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.

Note

All authors have read and approved the manuscript.


Supplementary Material



Publication History

Received: 05 November 2022

Accepted: 22 January 2023

Accepted Manuscript online:
27 January 2023

Article published online:
27 February 2023

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