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
Improvements in motor tasks through the use of smartphone technology for individuals with Duchenne muscular dystrophy
Capelini CM, da Silva TD, Tonks J, Watson S, Alvarez MPB, de Menezes LDC, Favero FM, Caromano FA, Massetti T, de Mello Monteiro CB Neuropsychiatr Dis Treat. 2017. 18(13):2209-2217. The main aim of the study was to verify whether individuals with Duchenne muscular...
Long-term follow-up of MRI changes in thigh muscles of patients with Facioscapulohumeral dystrophy: A quantitative study
Fatehi F, Salort-Campana E, Le Troter A, Lareau-Trudel E, Bydder M, Fouré A, Guye M, Bendahan D, Attarian S PLoS One. 2017 Aug 25;12(8):e0183825. The main aim of the study was to investigate longitudinally the time-dependent changes occurring in thigh muscles of...
Patients with Duchenne and Becker muscular dystrophies are not more asymmetrical than healthy controls on timed performance of upper limb tasks
Artilheiro MC, Sá CSC, Fávero FM, Caromano FA, Voos MC. Braz J Med Biol Res. 2017. 50(8): e6031. This study aimed to investigate possible asymmetries and relationships between performance of dominant and non-dominant upper limbs in patients with Duchenne and Becker...
Reliability and validity analyses of the North Star Ambulatory Assessment in Brazilian Portuguese
Okama LO, Zampieri LM, Ramos CL, Toledo FO, Alves CRJ, Mattiello-Sverzut AC, Mayhew A, Sobreira CFR Neuromuscul Disord. 2017. 27(8):723-729. Validation study of the cross-cultural adaptation to the Portuguese of the North Star Ambulatory Assessment.A positive...
Hyperleptinemia in children with autosomal recessive spinal muscular atrophy type I-III
Kölbel H, Hauffa BP, Wudy SA, Bouikidis A, Della Marina A, Schara U PLoS One. 2017. 9;12(3):e0173144. Study of prevalence of hyperleptinemia in a spinal muscular atrophy (SMA) population.Hyperleptinemia is highly prevalent in underweight children with SMA and is...
Electrical impedance myography (EIM) in individuals with COL6 and LAMA2 congenital muscular dystrophy: a cross-sectional and two-year analysis
Nichols C, Jain MS, Meilleur KG, Wu T, Collins J, Waite MR, Dastgir J, Salman A, Donkervoort S, Duong T, Keller K, Leach ME, Lott DJ, McGuire MN, Nelson L, Rutkowski A, Vuillerot C, Bönnemann CG, Lehky TJ. Muscle Nerve. 2018. 57(1):54-60. The main objective of the...