'Reading the palm' - A pilot study of grip and finger flexion strength as an outcome measure in 5q spinal muscular atrophy

Research output: Contribution to journalResearch articleContributedpeer-review

Abstract

BACKGROUND: Innovative RNA modifying and gene replacement therapies are currently revolutionizing the therapeutic landscape in 5q-associated spinal muscular atrophy (SMA). In order to provide individual recommendations for choice of treatment and therapy (dis-) continuation, objective outcome measures are needed. The purpose of this study was to determine whether maximum isometric voluntary grip and finger flexion strength is a useful sensitive outcome measure in children and adult patients with SMA.

METHODS: In this non-interventional, longitudinal pilot study, we assessed grip and finger flexion strength on 39 patients with SMA II and III (n = 16 children, mean age = 10.0; n = 23 adults, mean age = 38.4) using the Weber hand and finger dynamometer HFD 200. Grip and finger flexion strength, clinical examinations and motor function scores (Revised Upper Limb Module, Hammersmith Functional Motor Scale Expanded) were assessed over a 12-month treatment period concurrent with the nusinersen treatment.

RESULTS: Grip and finger flexion strength was highly associated with motor function and disease severity, SMA type and SMN2 copy number. During nusinersen treatment, grip and finger flexion strength significantly increased in children and adults with SMA.

CONCLUSION: Grip and finger flexion strength measured with the HFD 200 is a promising sensitive outcome measure for SMA.

Details

Original languageEnglish
Pages (from-to)189-198
Number of pages10
Journal Brain & development : official journal of the Japanese Society of Child Neurology
Volume46
Issue number5
Early online date7 Feb 2024
Publication statusPublished - May 2024
Peer-reviewedYes

External IDs

Scopus 85184750682

Keywords

Keywords

  • Pilot Projects, Humans, Spinal Muscular Atrophies of Childhood, Adult, Upper Extremity, Muscular Atrophy, Spinal/drug therapy, Outcome Assessment, Health Care, Child, Hand Strength