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

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 (kZ0.81–0.94) for nine items, good (kZ0.61–0.80) for 20 items and moderate (kZ0.51–0.60) for three items. High correlations were found between the total score and other scores: Vignos (rZ0.91) and Brooke (rZ0.85) grades, Functional Independence Measure (rZ0.91), the global severity of disability evaluated with visual analog scales by physicians (rZ0.88) and physiotherapists (rZ0.91). 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. q 2005 Elsevier B.V. All rights reserved.