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

Author:

Milev Evelin12ORCID,Selby Victoria12,Wolfe Amy12,Rohwer Annemarie12,Tillmann Ricarda3,Ramsey Danielle4,Iodice Mario2,Hogrel Jean‐Yves5,Baranello Giovanni12,Scoto Mariacristina12,Muntoni Francesco12

Affiliation:

1. Department of Developmental Neuroscience Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health London UK

2. Department of Neurology Great Ormond Street Hospital for Children NHS Foundation Trust London UK

3. Barts Health NHS Trust London UK

4. School of Health and Sports Sciences University of Suffolk Ipswich UK

5. Neuromuscular Physiology and Evaluation Laboratory Neuromuscular Investigation Centre, Institute of Myology Paris France

Abstract

AbstractIntroduction/AimsCurrent 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.MethodsThe 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.ResultsA 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 < .001), RULM and pinch strength (p < .001), RULM and revised Brooke (p < .001), grip strength and pinch strength (p < .001).DiscussionThe combined use of the RULM, dynamometry, and hand mobility provide insight about correlations between function and strength in children with SMA. The RULM and grip strength assessments captured a significant decline in upper limb function, whereas the pinch and finger/hand mobility showed an improvement over the course of 1 year and these results should be considered for future studies.

Publisher

Wiley

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