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. 2020 Apr;61(4):530-534.
doi: 10.1002/mus.26823. Epub 2020 Feb 7.

Evaluation of body composition as a potential biomarker in spinal muscular atrophy

Giovanni Baranello  1   2 , Ramona De Amicis  3 , Maria Teresa Arnoldi  1 , Riccardo Zanin  1 , Chiara Mastella  4 , Riccardo Masson  1 , Alessandro Leone  3 , Katia Alberti  4 , Andrea Foppiani  3 , Alberto Battezzati  3 , Simona Bertoli  3   5
Affiliations
Free article

Evaluation of body composition as a potential biomarker in spinal muscular atrophy

Giovanni Baranello et al. Muscle Nerve. 2020 Apr.
Free article

Abstract

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.

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References

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