TY - JOUR
T1 - Assessment of the upper limb muscles in patients with Fukuyama muscular dystrophy
T2 - Noninvasive assessment using visual ultrasound muscle analysis and shear wave elastography
AU - Harada, Risa
AU - Taniguchi-Ikeda, Mariko
AU - Nagasaka, Miwako
AU - Nishii, Tatsuya
AU - Inui, Atsuyuki
AU - Yamamoto, Tetsushi
AU - Morioka, Ichiro
AU - Kuroda, Ryosuke
AU - Iijima, Kazumoto
AU - Nozu, Kandai
AU - Sakai, Yoshitada
AU - Toda, Tatsushi
N1 - Publisher Copyright:
© 2022 The Author(s)
PY - 2022/9
Y1 - 2022/9
N2 - Fukuyama-type congenital muscular dystrophy (FCMD) is severe, childhood-onset muscular dystrophy. Recently, our group has discovered a potential treatment using antisense oligonucleotides. Therefore, an effective, reliable, and objective method of assessing muscle is needed. Ultrasound is a minimally invasive tool that can be applied without radiation exposure or pain. Evaluating tissue stiffness by shear wave elastography (SWE) has especially recently attracted attention. Here, we aimed to evaluate SWE value of the upper limb muscles: biceps brachii, triceps brachii, brachioradialis, abductor pollicis brevis, and abductor finger muscle in patients with FCMD. Upper extremity function was evaluated by visual muscle ultrasound analysis (VMUA) and SWE in 13 patients with FCMD and 20 healthy controls. The motor function evaluation tool was used to evaluate motor function, and the correlation with the dynamics of the SWE was determined. VMUA scaled using the Heckmatt scale was higher in patients with FCMD. SWE was also significantly higher and stiffer in the biceps brachii and brachioradialis in patients with FCMD. Furthermore, the severity of FCMD symptoms was correlated with muscle stiffness. We conclude that VMUA and SWE can be useful tools for monitoring muscle atrophy and upper limb function in patients with FCMD.
AB - Fukuyama-type congenital muscular dystrophy (FCMD) is severe, childhood-onset muscular dystrophy. Recently, our group has discovered a potential treatment using antisense oligonucleotides. Therefore, an effective, reliable, and objective method of assessing muscle is needed. Ultrasound is a minimally invasive tool that can be applied without radiation exposure or pain. Evaluating tissue stiffness by shear wave elastography (SWE) has especially recently attracted attention. Here, we aimed to evaluate SWE value of the upper limb muscles: biceps brachii, triceps brachii, brachioradialis, abductor pollicis brevis, and abductor finger muscle in patients with FCMD. Upper extremity function was evaluated by visual muscle ultrasound analysis (VMUA) and SWE in 13 patients with FCMD and 20 healthy controls. The motor function evaluation tool was used to evaluate motor function, and the correlation with the dynamics of the SWE was determined. VMUA scaled using the Heckmatt scale was higher in patients with FCMD. SWE was also significantly higher and stiffer in the biceps brachii and brachioradialis in patients with FCMD. Furthermore, the severity of FCMD symptoms was correlated with muscle stiffness. We conclude that VMUA and SWE can be useful tools for monitoring muscle atrophy and upper limb function in patients with FCMD.
KW - Elastography
KW - Fukuyama congenital muscular dystrophy
KW - Motor function
KW - Muscle ultrasound
KW - Visual muscle ultrasound analysis
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U2 - 10.1016/j.nmd.2022.05.004
DO - 10.1016/j.nmd.2022.05.004
M3 - Article
C2 - 35902324
AN - SCOPUS:85134810957
SN - 0960-8966
VL - 32
SP - 754
EP - 762
JO - Neuromuscular Disorders
JF - Neuromuscular Disorders
IS - 9
ER -