Hammersmith Functional Motor Scale and Motor Function Measure-20 in non ambulant SMA patients
E. Mazzone, R. De Sanctis, L. Fanelli, F. Bianco, M. Main, M. van den Hauwe, M. Ash, R. de Vries, J. Fagoaga Mata, K. Schaefer, A. D’Amico, G. Colia, C. Palermo, M. Scoto, A. Mayhew, M. Eagle, L. Servais, M. Vigo, A. Febrer, R. Korinthenberg, M. Jeukens M. de Viesser, A. Totoescu, T. Voit, K. Bushby, F. Muntoni, N. Goemans, E. Bertini, M. Pane, E. Mercuri.
Neuromuscul Disord. 2014. 24:347-352.
Evaluation of the correlation between the Hammersmith Functional Motor Scale and the MFM-20 in non-ambulant SMA children and adults at baseline, and over a 12 months period. Seventy-four patients performed both measures at baseline and 49 also had an assessment 12 months later. The correlation between the two scales was 0.733. The 12 month changes ranged between [1]11 and 4 for the Hammersmith and between [1]11 and 7 for the MFM-20. The results suggest that both scales provide useful information although they appeared to work differently at the two extremes of the spectrum of abilities. Hammersmith appeared to be more suitable in strong non ambulant patients, while the MFM-20 appeared to be more sensitive in the very weak patients.
Keywords: Outcome measures, Spinal muscular atrophy
Articles dans les revues scientifiques
Health-related quality of life, self-reported impairments and activities of daily living in relation to muscle function in post-polio syndrome
Gocheva V, Hafner P, Orsini AL, Schmidt S, Schaedelin S, Rueedi N, Rubino-Nacht D, Weber P, Fischer D.J Patient Rep Outcomes. 2020. 16;4(1):59. Investigation of health-related quality of life (HRQOL), self-reported impairments and activities of daily living and...
Effects of nusinersen after one year of treatment in 123 children with SMA type 1 or 2: a French real-life observational study
Audic F, de la Banda MGG, Bernoux D, Ramirez-Garcia P, Durigneux J, Barnerias C, Isapof A, Cuisset JM, Cances C, Richelme C, Vuillerot C, Laugel V, Ropars J, Altuzarra C, Espil-Taris C, Walther-Louvier U, Sabouraud P, Chouchane M, Vanhulle C, Trommsdorff V, Pervillé...
Long-term benefit of enzyme replacement therapy with alglucosidase alfa in adults with Pompe disease: prospective analysis from the French Pompe Registry
Semplicini C, De Antonio M, Taouagh N, Béhin A, Bouhour F, Echaniz-Laguna A, Magot A, Nadaj-Pakleza A, Orlikowski D, Sacconi S, Salort-Campana E, Solé G, Tard C, Zagnoli F, Jean-Yves H, Hamroun D, Laforêt P; French Pompe Study Group J Inherit Metab Dis. 2020. doi:...
Muscle MRI: A biomarker of disease severity in Duchenne muscular dystrophy? A systematic review
Ropars J, Gravot F, Ben Salem D, Rousseau F, Brochard S, Pons C. Neurology. 2020. 94(3):117-133. Review to assess the evidence of a relationship between muscle MRI and disease severity in Duchenne muscular dystrophy (DMD). Correlations between motor function tests,...
Evaluation of Speed-Accuracy Trade-Off in a Computer Task to Identify Motor Difficulties in Individuals With Duchenne Muscular Dystrophy – A Cross-Sectional Study
da Silva TD, Ribeiro-Papa DC, Coe S, Malheiros SRP, Massetti T, Meira Junior CM, Nicolai Ré AH, Collett J, Monteiro CBM, Dawes H. Res Dev Disabil. 2020. 96:103541. The aim of the study was to evaluate movement time during a task at various levels of difficulty and to...
Timed immersion expiration measures in patients with muscular dystrophies
Voos MC, Goya PSA, de Freitas BL, Pires AMT, Favero FM, Caromano FA. Arch Physiother. 2020 18;10:4. doi: 10.1186/s40945-020-0074-3. eCollection 2020. The aim of the study was to describe the evolution of timed immersion expiration in 57 patients with muscular...