Abstract INTRODUCTION: The aim of the study was to assess 12 month changes in upper limb function in patients affected by spinal muscular atrophy type 2 and 3. METHODS: Longitudinal 12 month data was collected in 114 patients, 60 type 2 and 54 type 3, using the Revised Upper Limb Module. RESULTS: The 12 month changes ranged between -7 and 9 (mean: -0.41; SD: 2.93). The mean changes were not significantly different between the three spinal muscular atrophy groups (-0.45 in type 2, -0.23 in non-ambulant type 3 and -0.34 in ambulant type 3, p = 0.96) and the relationship between 12 month change and age classes was not significantly different among the three types of SMA patients. DISCUSSION: Our results confirm that the Module explores a wide range of functional abilities and can be used in ambulant and non-ambulant patients of different ages in conjunction with other functional scales. Muscle Nerve 59:426-430, 2019. © 2019 Wiley Periodicals, Inc. KEYWORDS: Functional assessment; Longitudinal Data; Neuromuscular disorders; Outcome measures; Spinal Muscular Atrophy; Upper limb
Pera, M. C., Coratti, G., Mazzone, E., Montes, J., Scoto, M., De Sanctis, R., Main, M., Mayhew, A., Muni Lofra, R., Dunaway Young, S., Glanzman, A., Duong, T., Pasternak, A., Ramsey, D., Darras, B., Day, J., Finkel, R., De Vivo, D., Sormani, M., Bovis, F., Straub, V., Muntoni, F., Pane, M., Mercuri, E. M., Ismac Consortium, G., Revised upper limb module for spinal muscular atrophy: 12 month changes, <<MUSCLE & NERVE>>, 2019; (59): 426-430. [doi:10.1002/mus.26419] [http://hdl.handle.net/10807/131893]
Revised upper limb module for spinal muscular atrophy: 12 month changes
Pera, Maria Carmela;Coratti, Giorgia;De Sanctis, Roberto;Pane, Marika;Mercuri, Eugenio Maria;
2019
Abstract
Abstract INTRODUCTION: The aim of the study was to assess 12 month changes in upper limb function in patients affected by spinal muscular atrophy type 2 and 3. METHODS: Longitudinal 12 month data was collected in 114 patients, 60 type 2 and 54 type 3, using the Revised Upper Limb Module. RESULTS: The 12 month changes ranged between -7 and 9 (mean: -0.41; SD: 2.93). The mean changes were not significantly different between the three spinal muscular atrophy groups (-0.45 in type 2, -0.23 in non-ambulant type 3 and -0.34 in ambulant type 3, p = 0.96) and the relationship between 12 month change and age classes was not significantly different among the three types of SMA patients. DISCUSSION: Our results confirm that the Module explores a wide range of functional abilities and can be used in ambulant and non-ambulant patients of different ages in conjunction with other functional scales. Muscle Nerve 59:426-430, 2019. © 2019 Wiley Periodicals, Inc. KEYWORDS: Functional assessment; Longitudinal Data; Neuromuscular disorders; Outcome measures; Spinal Muscular Atrophy; Upper limb
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