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

Marika Pane, Eugenio Maria Mercuri, Roberto De Sanctis, Flaviana Bianco, Serena Sivo, Adele D'Amico, Elena S. Mazzone, Lavinia Fanelli, Sonia Messina, Roberta Battini, Marianna Scutifero, Roberta Petillo, Silvia Frosini, Roberta Scalise, Gianluca Vita, Claudio Bruno, Marina Pedemonte, Tiziana Mongini, Elena Pegoraro, Francesca BrustiaAlice Gardani, Angela 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

Risultato della ricerca: Contributo in rivistaArticolo in rivista

58 Citazioni (Scopus)

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 3years 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 25years old. A full score was consistently achieved by the age of 5years. 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.
Lingua originaleEnglish
pagine (da-a)201-206
Numero di pagine6
RivistaNeuromuscular Disorders
Volume24
DOI
Stato di pubblicazionePubblicato - 2014

Keywords

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Disease Progression
  • Duchenne muscular dystrophy
  • Humans
  • Male
  • Muscular Dystrophy, Duchenne
  • Symptom Assessment
  • Upper Extremity
  • Upper limb
  • Young Adult

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