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Published on: February 12, 2018
Instruments to assess upper-limb function in children and adolescents with neuromuscular diseases: a systematic
Gabriela Barroso De Queiroz Davoli1, Juliana Cardoso1, Giovanna Constantin Silva1
1Department of Health Science, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil.
Insights
The Performance of Upper Limb (PUL) and Revised Upper Limb Module (RULM) are best for assessing upper-limb function in common pediatric neuromuscular diseases. More research is needed for other NMDs due to instrument limitations.
Area of Science:
- Pediatric Neuromuscular Diseases
- Clinical Assessment Tools
- Upper Limb Function
Background:
- Neuromuscular diseases (NMDs) significantly impact pediatric upper-limb function.
- Standardized assessment tools are crucial for monitoring disease progression and treatment efficacy.
- Existing instruments require rigorous evaluation for their suitability in pediatric NMD populations.
Purpose of the Study:
- To systematically review and synthesize evidence on instruments for assessing upper-limb function in pediatric patients with NMD.
- To evaluate the methodological quality and measurement properties of available assessment tools.
- To identify gaps in current assessment strategies for pediatric NMDs.
Main Methods:
- Systematic review adhering to PRISMA and COSMIN guidelines.
- Searched PubMed/MEDLINE, LILACS, Embase, and Scopus databases.
- Assessed methodological quality using the COSMIN Risk of Bias checklist and measurement property criteria.
Main Results:
- Twelve instruments were identified across 34 articles, with Brooke Upper Extremity and PUL being most frequent.
- The PUL and Duchenne muscular dystrophy (DMD) Upper Limb patient-reported outcome measures (PROMs) showed good properties for DMD.
- The Revised Upper Limb Module (RULM) is suitable for spinal muscular atrophy; no instruments were found for Charcot-Marie-Tooth disease.
Conclusions:
- Performance of Upper Limb (PUL), DMD Upper Limb PROM, and RULM are recommended for prevalent pediatric NMDs.
- Significant gaps and methodological flaws necessitate the development of new, high-quality assessment instruments.
- Further research is crucial to develop and validate tools for underrepresented pediatric NMDs.
Aim:
To synthesize clinical and scientific evidence regarding the instruments available to assess upper-limb function in paediatric patients with neuromuscular disease (NMD).
Method:
This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) guidelines (Prospective Registry of Systematic Reviews no. CRD42020140343). Two independent reviewers searched the PubMed/MEDLINE, LILACS, Embase, and Scopus databases. Inclusion criteria were cross-sectional or longitudinal studies or randomized controlled trials that used scales or questionnaires to assess upper-limb function in paediatric patients with NMDs. The COSMIN Risk of Bias checklist and criteria for good measurement properties were applied to assess the methodological quality of the instruments.
Results:
In total, 34 articles and 12 instruments were included. The Brooke Upper Extremity (n=16) and Performance of Upper Limb (PUL) (n=12) instruments were the most used tools. The PUL and Duchenne muscular dystrophy (DMD) Upper Limb patient-reported outcome measures (PROMs) tested more measurement properties and provided higher methodological quality scores for patients with DMD. Likewise, the Revised Upper Limb Module (RULM) was the most suitable instrument for patients with spinal muscular atrophy. No instrument has been devised to assess upper-limb function in patients with Charcot-Marie-Tooth disease and no other disease-specific instruments were found.
Interpretation:
The PUL, DMD Upper Limb PROM, and RULM are the most suitable instruments to assess upper-limb function in the two most prevalent paediatric NMDs. The identified gaps and methodological flaws of the available instruments indicate a need to develop high-quality instruments to assess other types of paediatric NMDs. What this paper adds The most suitable observer-rater instrument to assess upper-limb function in Duchenne muscular dystrophy (DMD) is the Performance of Upper Limb. The most suitable observer-rater instrument to assess upper-limb function in spinal muscular atrophy is the Revised Upper Limb Module. The DMD Upper Limb patient-reported outcome measure is recommended to assess the upper-limb performance of patients with DMD. Literature gaps and methodological flaws indicate the need to develop high-quality instruments to assess other types of paediatric neuromuscular disease.
