Reliability of the Performance of Upper Limb assessment in Duchenne muscular dystrophy

Marika Pane, Elena S. Mazzone, Lavinia Fanelli, Roberto De Sanctis, Flaviana Bianco, Serena Sivo, Adele D'Amico, Sonia Messina, Roberta Battini, Marianna Scutifero, Roberta Petillo, Silvia Frosini, Roberta Scalise, Gianluca Vita, Claudio Bruno, Marina Pedemonte, Tiziana Mongini, Elena Pegoraro, Francesca Brustia, Alice GardaniAngela Berardinelli, Valentina Lanzillotta, Emanuela Viggiano, Filippo Cavallaro, Maria Sframeli, Luca Bello, Andrea Barp, Serena Bonfiglio, Enrica Rolle, Giulia Colia, Michela Catteruccia, Concetta Palermo, Grazia D'Angelo, Antonella Pini, Elena Iotti, Ksenija Gorni, Giovanni Baranello, Lucia Morandi, Enrico Bertini, Luisa Politano, MariaPia Sormani, Eugenio Mercuri

Research output: Contribution to journalArticlepeer-review

Abstract

The Performance of Upper Limb was specifically designed to assess upper limb function in Duchenne muscular dystrophy. The aim of this study was to assess (1) a cohort of typically developing children from the age of 3. years onwards in order to identify the age when the activities assessed in the individual items are consistently achieved, and (2) a cohort of 322 Duchenne children and young adults to establish the range of findings at different ages. We collected normative data for the scale validation on 277 typically developing subjects from 3 to 25. years old. A full score was consistently achieved by the age of 5. years. In the Duchenne cohort there was early involvement of the proximal muscles and a proximal to distal progressive involvement. The scale was capable of measuring small distal movements, related to activities of daily living, even in the oldest and weakest patients. Our data suggest that the assessment can be reliably used in both ambulant and non ambulant Duchenne patients in a multicentric setting and could therefore be considered as an outcome measure for future trials.

Original languageEnglish
Pages (from-to)201-206
Number of pages6
JournalNeuromuscular Disorders
Volume24
Issue number3
DOIs
Publication statusPublished - Mar 2014

Keywords

  • Duchenne muscular dystrophy
  • Upper limb

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Genetics(clinical)
  • Neurology

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