Upper limb function in adults with Duchenne muscular dystrophy
Bartels G, Pangalila RF, Bergen MP, Cobben NAM, Stam HJ, Roebroeck ME.
J Rehabil Med 2011. 43(9) : 770-5.
This study illustrates a large variability in upper limb function in adult patients with Duchenne muscular dystrophy, and identifies muscle strength and range of motion as factors strongly associated with upper limb function. The results suggest that preserving muscle strength and range of motion in Duchenne patients might be relevant for a better outcome of distal motor function of the upper limb when adult.
Keywords: Duchenne muscular dystrophy, Adult, Upper limb function, Measurement of motor function, Muscle strength, Range of motion
Articles dans les revues scientifiques
Muscular involvement assessed by MRI correlates to motor function measurement values in oculopharyngeal muscular dystrophy
Fischmann A, Gloor M, Fasler S, Haas T, Rodoni Wetzel R, Bieri O, Wetzel S, Heinimann K, Scheffler K, Fischer D. J Neurol 2011. 258: 1333–1340. Oculopharyngeal muscular dystrophy (OPMD) is a progressive skeletal muscle dystrophy characterized by ptosis, dysphagia, and...
Impaired Mandibular Function in Spinal Muscular Atrophy Type II: Need for Early Recognition
van Bruggen HW, van den Engel-Hoek L, van der Pol WL, de Wijer A, de Groot IJ, Steenks MH. J Child Neurol. Nov 2011. 26: 1392 - 1396. The aim of the study is to assess mandibular function in young patients with spinal muscular atrophy type II. The mandibular function...
Idebenone for the treatment of Duchenne muscular dystrophy (Protocol)
Geng J, Dong J, Jiang K, Shen L, Wu T, Ni H, Shi LL, Wang G, Wu H. The Cochrane Library 2011. Description of a protocol aiming at assessing efficacy of ibedenone on muscle strength, muscle function, lung function, cardiac function and quality of life for patients with...
High-frequency, low-intensity vibrations increase bone mass and muscle strength in upper limbs, improving autonomy in disabled children
Reyes ML, Hernandez M, Holmgren LJ, Sanhueza E, Escobar RG. J Bone Miner Res, 2011. 26(8): 1759-66. The main objective of this study was to evaluate the efficacy and safety of high-frequency, low-magnitude vibration, in patients with disabilities. 55 children were a...
Motor Function Measure Scale (MFM): New Instrument for Follow-Up Brazilian Patients with Neuromuscular Disease
Iwabe C, Nucci A, Pfeilsticker BHM, Magna LA. Muscular Dystrophy ed.Croacia : Intech. 2012. 303-320. Book chapter presenting the validation and applicability of the MFM Portuguese version.Link Articles dans les revues scientifiques
Recombinant human acid alpha-glucosidase (rhGAA) in adult patients with severe respiratory failure due to Pompe disease
Orlikowski D, Pellegrini N, Prigent H, Laforêt P, Carlier R, Carlier P, Eymard B, Lofaso F, Annane D. Neuromuscul Disord. 2011. 21(7):477-82. This open-label prospective exploratory study investigated the effect of 12 months of recombinant enzyme replacement therapy...