Revised upper limb module for spinal muscular atrophy: 12 month changes

Maria Carmela Pera, Giorgia Coratti, Elena S Mazzone, Jacqueline Montes, Mariacristina Scoto, Roberto De Sanctis, Marion Main, Anna Mayhew, Robert Muni Lofra, Sally Dunaway Young, Allan M Glanzman, Tina Duong, Amy Pasternak, Danielle Ramsey, Basil Darras, John W Day, Richard S Finkel, Maria Pia Sormani, Francesca Bovis, Darryl C De VivoVolker Straub, Francesco Muntoni, Marika Pane, Eugenio Mercuri, iSMAC Consortium Group

Research output: Contribution to journalArticlepeer-review

24 Citations (Scopus)

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.
Original languageEnglish
Pages (from-to)426-430
Number of pages5
JournalMuscle & Nerve
Volume59
Issue number4
Early online date7 Feb 2019
DOIs
Publication statusPublished - 1 Apr 2019

Keywords

  • Functional assessment
  • Longitudinal Data
  • Neuromuscular disorders
  • Outcome measures
  • Spinal Muscular Atrophy
  • Upper limb

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