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

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 i...

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Bibliographic Details
Published in:Neuromuscular disorders : NMD 2014-03, Vol.24 (3), p.201-206
Main Authors: Pane, Marika, Mazzone, Elena S, Fanelli, Lavinia, De Sanctis, Roberto, Bianco, Flaviana, Sivo, Serena, D’Amico, Adele, Messina, Sonia, Battini, Roberta, Scutifero, Marianna, Petillo, Roberta, Frosini, Silvia, Scalise, Roberta, Vita, Gianluca, Bruno, Claudio, Pedemonte, Marina, Mongini, Tiziana, Pegoraro, Elena, Brustia, Francesca, Gardani, Alice, Berardinelli, Angela, Lanzillotta, Valentina, Viggiano, Emanuela, Cavallaro, Filippo, Sframeli, Maria, Bello, Luca, Barp, Andrea, Bonfiglio, Serena, Rolle, Enrica, Colia, Giulia, Catteruccia, Michela, Palermo, Concetta, D’Angelo, Grazia, Pini, Antonella, Iotti, Elena, Gorni, Ksenija, Baranello, Giovanni, Morandi, Lucia, Bertini, Enrico, Politano, Luisa, Sormani, MariaPia, Mercuri, Eugenio
Format: Article
Language:English
Subjects:
Adolescent
Adult
Child
Child, Preschool
Disease Progression
Duchenne muscular dystrophy
Humans
Male
Muscular Dystrophy, Duchenne - physiopathology
Neurology
Symptom Assessment
Upper Extremity - physiopathology
Upper limb
Young Adult
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Summary: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.
ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2013.11.014