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Updated: Feb 18, 2026
Assessment of Neuromuscular Function Using Percutaneous Electrical Nerve Stimulation
Published on: September 13, 2015
Development and preliminary evidence of the psychometric properties of the GNE myopathy functional activity scale
Jill Mayhew1, Nicola Bonner2, Rob Arbuckle2
1Ultragenyx Pharmaceutical, 60 Leveroni Court, Novato, CA 94949, USA.
Aim:
GNE myopathy, a rare, severe, progressive myopathy, presents with lower extremity distal muscle weakness. The GNE myopathy functional activity scale (GNEM-FAS) evaluates the impact of GNE myopathy on functioning in adults. This paper presents the psychometric validation of the GNEM-FAS.
Patients & Methods:
Validation of the GNEM-FAS was performed using data from a randomized, double-blind, placebo-controlled Phase-II study (n = 46).
Results:
Domain score distributions were acceptable. Moderate inter-item correlations (typical range, 0.40-0.70), strong item convergent and discriminant validity and high internal consistency reliability (α = 0.88-0.92) supported the instrument structure. Test-retest reliability was strong (ICC range: 0.87-0.95). Scale scores distinguished among subjects with differing disease severity (p < 0.05).
Conclusion:
This study provides preliminary evidence of the GNEM-FAS as a valid, reliable assessment.
