title: A motor function measure for neuromuscular diseases. Construction and validation study.
abstract: 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 (kappa=0.81-0.94) for nine items, good (kappa=0.61-0.80) for 20 items and moderate (kappa=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.
authors.affiliation: Department of Paediatric Rehabilitation, l'Escale, Centre Hospitalier Lyon-Sud, France. carole.berard@chu-lyon.fr
authors.forename: Carole
authors.initials: C
authors.lastname: Bérard
authors.forename: Christine
authors.initials: C
authors.lastname: Payan
authors.forename: Isabelle
authors.initials: I
authors.lastname: Hodgkinson
authors.forename: Jacques
authors.initials: J
authors.lastname: Fermanian
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004185:Disability Evaluation; D005121:Extremities / Q000503:physiopathology; D005163:Factor Analysis, Statistical; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology*; D009468:Neuromuscular Diseases / Q000145:classification / Q000503:physiopathology*; D010147:Pain Measurement; D011237:Predictive Value of Tests; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D013997:Time Factors