Whole‐Body Muscle Magnetic Resonance Imaging in 81 Patients with Spinal and Bulbar Muscular Atrophy: A Prospective Study

Author:

Kim Hyunjin1ORCID,Seo Incheol2,Kang Minsung3,Park Jin‐Mo4,Seok Hung Youl5ORCID,Kim Sohyeon5,Park Jin‐Sung678ORCID

Affiliation:

1. Department of Neurology, Asan Medical Center University of Ulsan College of Medicine Seoul Republic of Korea

2. Department of Immunology, School of Medicine Kyungpook National University Daegu Republic of Korea

3. Department of Neurology Kyungpook National University Chilgok Hospital Daegu Republic of Korea

4. Department of Neurology Dongguk University College of Medicine, Dongguk University Gyeongju Hospital Gyeongju Republic of Korea

5. Department of Neurology, Dongsan Hospital Keimyung University School of Medicine Daegu Republic of Korea

6. Department of Neurology, School of Medicine Kyungpook National University, Kyungpook National University Chilgok Hospital Daegu Republic of Korea

7. Korea Brain Research Institute Dementia Research Group Daegu Republic of Korea

8. Brain Science & Engineering Institute Kyungpook National University Daegu Republic of Korea

Abstract

ObjectiveSpinal and bulbar muscular atrophy (SBMA) is characterized by slow, progressive bulbar and limb muscle weakness; however, the pattern of progression of muscle fat infiltration remains unclear. We assessed the progression of muscle involvement in 81 patients with SBMA using whole‐body muscle magnetic resonance imaging (MRI), alongside clinical and laboratory findings.MethodsThis prospective study included patients with genetically confirmed SBMA who underwent whole‐body muscle MRI. We analyzed muscle fat infiltration and the pattern of involved muscles using cluster analysis, visualizing the sequential progression of fat infiltration. Muscle clusters demonstrated correlation with clinical scales and laboratory findings. Additionally, linear regression analysis was performed to identify the MRI section most strongly associated with 6‐minute walk test (6MWT).ResultsWe included 81 patients with SBMA (age = 54.3 years). After categorizing the patients into 6 clusters based on the pattern of muscle fat infiltration, we observed that muscle involvement began in the posterior calf and progressed to the posterior thigh, pelvis, trunk, anterior thigh, medial thigh, anterior calf, and upper extremity muscles. These muscle clusters correlated significantly with disease duration (τ = 0.47, p < 0.001), 6MWT (τ = −0.49, p < 0.001), and serum creatinine level (τ = −0.46, p < 0.001). The whole‐body MRI indicated the thigh as the section most significantly correlated with 6MWT.InterpretationWe used whole‐body muscle MRI to determine the sequential progression of the fat infiltration in SBMA. Our findings may enable the identification of objective and reliable imaging outcome measures in the study of the natural history or future clinical trials of SBMA. ANN NEUROL 2023

Funder

Asan Institute for Life Sciences, Asan Medical Center

Ministry of Science and ICT, South Korea

Publisher

Wiley

Subject

Neurology (clinical),Neurology

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