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

Abstract

Introduction This study aimed to investigate selective muscle involvement by shear wave elastography (SWE) in patients with spinal muscular atrophy (SMA) types 2-3 and to compare SWE values with magnetic resonance imaging (MRI) in demonstrating muscle involvement. Methods Seventeen patients with SMA types 2-3 were included in the study. SWE was used to evaluate stiffness of the upper, lower extremities and paraspinal muscles. Involvement of the paraspinal muscles was evaluated using 1.5-Tesla 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 type 2-3. 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 a alternative non-invasive 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.

Influence of the physical pressure of an ultrasound probe on shear-wave elastography measurements of the gastrocnemius muscle in a paediatric population: a non-interventional cohort study

“…While muscle applications of SWE are still developing, they may prove to be useful for both the diagnosis and monitoring of disease progression and treatment effects, particularly in diseases such as cerebral palsy, Duchenne disease, neonatal brachial plexus palsy, and spinal muscle atrophy [4,[13][14][15][16]. Currently, monitoring of the course and treatment of neuromuscular disease is often based on different clinical and functional scales.…”