TY - JOUR
T1 - Multi-parametric quantitative magnetic resonance imaging of the upper arm muscles of patients with spinal muscular atrophy
AU - Hooijmans, Melissa T.
AU - Habets, Laura E.
AU - van den Berg-Faay, Sandra A. M.
AU - Froeling, Martijn
AU - Asselman, Fay-Lynn
AU - Strijkers, Gustav J.
AU - Jeneson, Jeroen A. L.
AU - Bartels, Bart
AU - Nederveen, Aart J.
AU - van der Pol, W. Ludo
N1 - Funding Information: Prinses Beatrix Spierfonds; Grant/Award Number: W.OR17–05. Stichting Spieren voor Spieren. Zwaluwen Jeugd Actie. Funding information Prinses Beatrix Spierfonds, Grant/Award Number: W.OR17–05; Stichting Spieren voor Spieren; Zwaluwen Jeugd Actie Publisher Copyright: © 2022 The Authors. NMR in Biomedicine published by John Wiley & Sons Ltd.
PY - 2022/7/1
Y1 - 2022/7/1
N2 - Quantitative magnetic resonance imaging (qMRI) is frequently used to map the disease state and disease progression in the lower extremity muscles of patients with spinal muscular atrophy (SMA). This is in stark contrast to the almost complete lack of data on the upper extremity muscles, which are essential for carrying out daily activities. The aim of this study was therefore to assess the disease state in the upper arm muscles of patients with SMA in comparison with healthy controls by quantitative assessment of fat fraction, diffusion indices, and water T2 relaxation times, and to relate these measures to muscle force. We evaluated 13 patients with SMA and 15 healthy controls with a 3-T MRI protocol consisting of DIXON, diffusion tensor imaging, and T2 sequences. qMRI measures were compared between groups and related to muscle force measured with quantitative myometry. Fat fraction was significantly increased in all upper arm muscles of the patients with SMA compared with healthy controls and correlated negatively with muscle force. Additionally, fat fraction was heterogeneously distributed within the triceps brachii (TB) and brachialis muscle, but not in the biceps brachii muscle. Diffusion indices and water T2 relaxation times were similar between patients with SMA and healthy controls, but we did find a slightly reduced mean diffusivity (MD), λ1, and λ3 in the TB of patients with SMA. Furthermore, MD was positively correlated with muscle force in the TB of patients with SMA. The variation in fat fraction further substantiates the selective vulnerability of muscles. The reduced diffusion tensor imaging indices, along with the positive correlation of MD with muscle force, point to myofiber atrophy. Our results show the feasibility of qMRI to map the disease state in the upper arm muscles of patients with SMA. Longitudinal data in a larger cohort are needed to further explore qMRI to map disease progression and to capture the possible effects of therapeutic interventions.
AB - Quantitative magnetic resonance imaging (qMRI) is frequently used to map the disease state and disease progression in the lower extremity muscles of patients with spinal muscular atrophy (SMA). This is in stark contrast to the almost complete lack of data on the upper extremity muscles, which are essential for carrying out daily activities. The aim of this study was therefore to assess the disease state in the upper arm muscles of patients with SMA in comparison with healthy controls by quantitative assessment of fat fraction, diffusion indices, and water T2 relaxation times, and to relate these measures to muscle force. We evaluated 13 patients with SMA and 15 healthy controls with a 3-T MRI protocol consisting of DIXON, diffusion tensor imaging, and T2 sequences. qMRI measures were compared between groups and related to muscle force measured with quantitative myometry. Fat fraction was significantly increased in all upper arm muscles of the patients with SMA compared with healthy controls and correlated negatively with muscle force. Additionally, fat fraction was heterogeneously distributed within the triceps brachii (TB) and brachialis muscle, but not in the biceps brachii muscle. Diffusion indices and water T2 relaxation times were similar between patients with SMA and healthy controls, but we did find a slightly reduced mean diffusivity (MD), λ1, and λ3 in the TB of patients with SMA. Furthermore, MD was positively correlated with muscle force in the TB of patients with SMA. The variation in fat fraction further substantiates the selective vulnerability of muscles. The reduced diffusion tensor imaging indices, along with the positive correlation of MD with muscle force, point to myofiber atrophy. Our results show the feasibility of qMRI to map the disease state in the upper arm muscles of patients with SMA. Longitudinal data in a larger cohort are needed to further explore qMRI to map disease progression and to capture the possible effects of therapeutic interventions.
KW - quantitative magnetic resonance imaging
KW - skeletal muscle
KW - spinal muscular atrophy
KW - upper arm muscles
UR - http://www.scopus.com/inward/record.url?scp=85124580081&partnerID=8YFLogxK
U2 - https://doi.org/10.1002/nbm.4696
DO - https://doi.org/10.1002/nbm.4696
M3 - Article
C2 - 35052014
SN - 0952-3480
VL - 35
JO - NMR in biomedicine
JF - NMR in biomedicine
IS - 7
M1 - e4696
ER -