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Hammersmith Functional Motor Scale and Motor Function Measure-20 in non ambulant SMA patients

2014; Elsevier BV; Volume: 24; Issue: 4 Linguagem: Inglês

10.1016/j.nmd.2014.01.003

1873-2364

Elena Mazzone, Rita De Sanctis, Lavinia Fanelli, F. Bianco, Marion Main, M. van den Hauwe, M. Ash, R. de Vries, Joaquim Fagoaga Mata, Karen Moore Schaefer, Adele D’Amico, Giulia Colia, Concetta Palermo, Mariacristina Scoto, Anna Mayhew, Michelle Eagle, Laurent Servais, M. Vigo, A. Febrer, Rudolf Korinthenberg, M. Jeukens, M. de Viesser, A. Totoescu, Thomas Voït, Kate Bushby, Francesco Muntoni, Nathalie Goemans, Enrico Bertini, Marika Pane, Eugenio Mercuri,

Cardiomyopathy and Myosin Studies

The aim of this prospective longitudinal multi centric study was to evaluate the correlation between the Hammersmith Functional Motor Scale and the 20 item version of the Motor Function Measure in non ambulant SMA children and adults at baseline and over a 12 month period. Seventy-four non-ambulant patients performed both measures at baseline and 49 also had an assessment 12 month later. At baseline the scores ranged between 0 and 40 on the Hammersmith Motor function Scale and between 3 and 45 on the Motor Function Measure 20. The correlation between the two scales was 0.733. The 12 month changes ranged between −11 and 4 for the Hammersmith and between −11 and 7 for the Motor Function Measure 20. The correlation between changes was 0.48. Our results suggest that both scales provide useful information although they appeared to work differently at the two extremes of the spectrum of abilities. The Hammersmith Motor Function Scale appeared to be more suitable in strong non ambulant patients, while the Motor Function Measures appeared to be more sensitive to capture activities and possible changes in the very weak patients, including more items capturing axial and upper limb activities. The choice of these measures in clinical trials should therefore depend on inclusion criteria and magnitude of expected changes.