Introduction: We aimed to investigate the correlation between body composition (BC) and spinal muscular atrophy (SMA)-specific motor function assessments.
Methods: Patients with SMA types I or II, aged 1 to 10 years, were recruited in this cross-sectional study. The protocol included anthropometric measurements, and dual-energy X-ray absoprtiometry to assess fat mass (FM), lean mass (LM), fat-free mass (FFM), FM and FFM indexes (FMI, FFMI), and motor function assessments (Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scale for SMAI, and Hammersmith Functional Motor Scale-Expanded for SMAII).
Results: Eighty-eight children were included. All had a higher FM percentage than reference values. Motor function was moderately correlated with body mass index (BMI), FFMI, and LMI in SMAI, and weakly correlated with FFMI, LMI, and LM:FM ratio in SMAII.
Discussion: BC shows promise as a potential biomarker for SMA, but further studies are needed.
Keywords: Hammersmith Functional Motor Scale―Expanded; The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders; body composition; fat-free mass; spinal muscular atrophy.
© 2020 The Authors. Muscle & Nerve published by Wiley Periodicals, Inc.