Responsiveness of the Motor Function Measure in Patients With Spinal Muscular Atrophy.
Vuillerot C, Payan C, Iwaz J, Ecochard R, Bérard C and the MFM Spinal Muscular Atrophy Study Group.
Arch Phys Med Rehabil. 2013. 94(8) : 1555-61.
Assessment in 112 patients with Spinal Muscular Atrophy (SMA) aged 5.7 to 59 years of the ability of the MFM to detect changes in the progression of SMA. In patients with SMA type 2 and type 3, there was a moderate inverse relationship between age and the MFM total score. Patients with less than 6 months’ follow-up showed little score changes. Patients with a longer follow-up showed a slow deterioration (-0.9 points/y for type 2 and -0.6 points/y for type 3). Substantial responsiveness was obtained with the MFM D2 subscore (proximal and axial motricity) in patients with SMA type 2, and with the MFM D1 subscore (standing and transfers) in patients with SMA type 3.
Keywords: Clinical trial, Mobility limitation, Muscle weakness, Spinal Muscular Atrophy, Neuromuscular diseases, Rehabilitation
Articles dans les revues scientifiques
Predictive factors for masticatory performance in Duchenne Muscular Dystrophy
Van Bruggen HW, van de Engel-Hoek L, Steenks MH, Bronkhorst E, Creugers NHJ, de Groot IJM, Kalaykova SI. Neuromuscul Disord. 2014. 24(8):684-92. Patients with Duchenne Muscular Dystrophy (DMD) report masticatory and swallowing problems. Authors investigated whether...
What Can We Learn From Assisted Bicycle Training in a Girl With Dystrophinopathy? A Case Study
Huijben J, Jansen M, Ginjaar IN, Lammens M, van Putten M, van Alfen N, de Groot I. J Child Neurol. 2015. 30(5),659-63. Case study of a 9-year-old ambulatory girl with dystrophinopathy due to a mosaic translocation mutation participated in dynamic training. The girl...
Molecular therapeutic strategies for spinal muscular atrophies: current and future clinical trials
Zanetta C, Nizzardo M, Simone C, Monguzzi E, Bresolin N, Comi GP, Corti S. Clin Ther. 2014. 1;36(1):128-40. This article provides a general overview of the main aspects that need to be taken into account to design a more efficient clinical trial and to summarize the...
Relationship between muscle strength and motor function in Duchenne muscular dystrophy
Nunes MF, Hukuda ME, Favero FM, Oliveira AB, Voos MC, Caromano FA. Arq Neuropsiquiatr. 2016. 74(7):530-5. This study investigates the relationship between muscle strength as measured by Medical Research Council (MRC) scale and MFM and between these variables and age...
Influence of a two-year steroid treatment on body composition as measured by Dual X-Ray Absorptiometry in boys with Duchenne Muscular Dystrophy
Vuillerot C, Braillon P, Fontaine-Carbonnel S, Rippert P, André E, Iwaz J, Poirot I, Bérard C. Neuromuscul Disord. 2014. 24:467-473. Assessment of effects of long term steroid treatments on body composition, assessed by Dual X-Ray absorptiometry, in 21 patients with...
The applicability of four clinical methods to evaluate arm and hand function in all stages of spinal muscular atrophy type II.
Werlauff U, Fynbo Steffensen B. Disabil Rehabil. 2014. 36(25):2120-6. Assessment of ability of 4 clinical methods to reflect arm and hand function at impairment and activity level and to determine their ability to discriminate among SMA II patients of all ages and in...