What is known about muscle weakness, balance impairments and indoor mobility limitations in oculopharyngeal muscular dystrophy? A scoping review.

OBJECTIVE

The objective of this scoping review was to map the body of existing literature regarding muscle strength, balance and mobility limitations in oculopharyngeal muscular dystrophy (OPMD).

METHODS

The Joanna Briggs Institute's methodology was followed. All original research including participants with OPMD 18 years or older and describing muscle strength, balance and/or mobility limitations using clinical outcome assessments retrieved from the PubMed, MEDLINE, PEDro, Cochrane Database of Systematic Reviews, and CINHAL Plus databases (searched before January 30th, 2023) were included.

RESULTS

Titles and abstracts from 416 studies were screened, and 54 articles were fully reviewed. Proximal upper and lower limb muscle strength were assessed in majority (92.6% of studies). Lack of common methodology made conclusions on their prevalence and severity difficult. Regarding balance, no performance outcome measures was retrieved. 29.6 to 63.0% of studies addressed mobility limitations; only two prospective longitudinal studies were retrieved, and two studies used reference values to assess impairments/limitations severity.

CONCLUSION

This scoping review highlights the current knowledge on muscle strength, balance and mobility limitations in OPMD. A lack of balance and complete mobility assessments, and OPMD-specific questionnaire for motor impairments or mobility limitations are noted. Future studies should focus on using standardized clinical outcome assessments to better document these impairments/limitations.