A motor function measure scale for neuromuscular diseases. Construction and validation study

• Published in: Neuromuscular disorders : NMD 2005-07, Vol.15 (7), p.463-470
• Main Authors: Bérard, Carole, Payan, Christine, Hodgkinson, Isabelle, Fermanian, Jacques, The MFM Collaborative Study Group
• Format: Article
• Language: English
• Subjects: Clinical trials; Metrology; Motor function; Neuromuscular disease; Scale; Validation
• ISSN: 0960-8966; 1873-2364
• DOI: 10.1016/j.nmd.2005.03.004

A new scale for motor function measurement has been developed for neuromuscular diseases. The validation study included 303 patients, aged 6–62 years. Seventy-two patients had Duchenne muscular dystrophy, 32 Becker muscular dystrophy, 30 limb-girdle muscular dystrophy, 39 facio-scapulo-humeral dystrophy, 29 myotonic dystrophy, 21 congenital myopathy, 10 congenital muscular dystrophy, 35 spinal muscular atrophy and 35 hereditary neuropathy. The scale comprised 32 items, in three dimensions: standing position and transfers, axial and proximal motor function, distal motor function. Agreement coefficients for inter-rater reliability were excellent ( κ=0.81–0.94) for nine items, good ( κ=0.61–0.80) for 20 items and moderate ( κ=0.51–0.60) for three items. High correlations were found between the total score and other scores: Vignos ( r=0.91) and Brooke ( r=0.85) grades, Functional Independence Measure ( r=0.91), the global severity of disability evaluated with visual analog scales by physicians ( r=0.88) and physiotherapists ( r=0.91). This scale is reliable, does not require any special equipment and is well-accepted by patients. Its sensitivity to change is being assessed to permit its use in clinical trials of neuromuscular diseases.