Assessment of the upper limb function, strength, and mobility in treatment-naive children with spinal muscular atrophy Types 2 and 3

Abstract

Introduction/Aims: Current upper limb assessments in pediatric spinal muscular atrophy (SMA) may not adequately capture change with disease progression. Our aim was to examine the relationship between motor function, strength, and hand/finger mobility of the upper limb in treatment-naïve children with SMA Types 2 and 3 to assess new methods to supplement current outcomes. Methods: The Revised Upper Limb Module (RULM), grip and pinch strength, and hand/finger mobility data were collected from 19 children with SMA Types 2 and 3 aged 5.2–16.9 years over a year. Results: A median loss between 0.5 and 2.5 points in the RULM was seen across all SMA subgroups with the biggest median loss recorded between 10 and 14 years of age. The grip strength loss was −0.06 kg (−4.69 to 3.49; IQR, 1.21); pinch improvement of 0.05 (−0.65 to 1.27; IQR, 0.48); hand/finger mobility test improvement of 4 points (−24 to 14; IQR, 6.75) for the whole cohort. Significant correlations were found between the RULM and grip strength (p