Assessment of respiratory muscles and motor function in children with SMA treated by nusinersen
Respiratory muscles performance and lung function were evaluated in children with SMA 1 and 2 after six injections of nusinersen (M14). Treated subjects were assessed with MFM and HINE-2 at baseline and M14.
A significant improvement in MFM and HINE-2 was observed at M14 as compared with baseline.
Keywords: lung function; motor function; nusinersen; respiratory muscles; spinal muscular atrophy.
Articles in scientific journal
Responsiveness of the Motor Function Measure in Neuromuscular Diseases
Vuillerot C, Payan C, Girardot F, Fermanian J, Iwaz J, Bérard C, Ecochard R, and the MFM Study Group.Arch Phys Med Rehabil 2012. 93(12):2551-2556. Study of the responsiveness of the MFM in 152 neuromuscular disease patients.MFM showed a good responsiveness, especially...
The role of physical therapy in the preservation of motor skills in Becker’s muscular dystrophy–a case study
Codrea TA, Pop NH. Palestrica of the Third Millennium - Civilization and Sport. 13(3):272-275. A physical therapy protocol was applied to a 19 years old Becker’s muscular dystrophy patients. Motor skills were evaluated using the MFM. The long term objectives of the...
Motor assessment in patients with Duchenne muscular dystrophy
Diniz GP, Lasmar LM, Giannetti JG. Arq Neuropsiquiatr. 2012. 70(6): 416-21. This study of 20 DMD patients shows the sensitivity of the MFM with six months between two applications. PubMed link Keywords: Duchenne muscular dystrophy, MFM, Medical research concil ...
Evaluation of muscle oxygenation by near-infrared spectroscopy in patients with Becker muscular dystrophy
Allart E, Olivier N, Hovart H, Thevenon A, Tiffreau V. Neuromuscul Disord. 2012. 22(8): 720-7. The primary objective of the study was to describe and compare muscle oxygenation patterns during constant-load exercise in patients with Becker Muscular Dystrophy (BMD) and...
Nitric oxide donor and non steroidal anti inflammatory drugs as a therapy for muscular dystrophies: evidence from a safety study with pilot efficacy measures in adult dystrophic patients.
D'Angelo MG, Gandossini S, Martinelli Boneschi F, Sciorati C, Bonato S, Brighina E, Comi GP, Turconi AC, Magri F, Stefanoni G, Brunelli S, Bresolin N, Cattaneo D, Clementi E. Pharmacol Res. 2012. 65(4): 472-9. The main of this monocentric pilot study was to evaluate...
Gait Assessment in Children With Duchenne Muscular Dystrophy During Long-Distance Walking
Ganea R, Jeannet PY, Paraschiv-Ionescu A, Goemans N, Piot C, Van den Hauwe M, Aminian K. J Child Neurol. 2012. 27: 1 30-38. Gait patterns of 25 children with Duchenne muscular dystrophy were compared to that of healthy children. Authors showed that Duchenne muscular...