TY - JOUR
T1 - Reliability of the Performance of Upper Limb assessment in Duchenne muscular dystrophy
AU - Pane, Marika
AU - Mazzone, Elena S.
AU - Fanelli, Lavinia
AU - De Sanctis, Roberto
AU - Bianco, Flaviana
AU - Sivo, Serena
AU - D'Amico, Adele
AU - Messina, Sonia
AU - Battini, Roberta
AU - Scutifero, Marianna
AU - Petillo, Roberta
AU - Frosini, Silvia
AU - Scalise, Roberta
AU - Vita, Gianluca
AU - Bruno, Claudio
AU - Pedemonte, Marina
AU - Mongini, Tiziana
AU - Pegoraro, Elena
AU - Brustia, Francesca
AU - Gardani, Alice
AU - Berardinelli, Angela
AU - Lanzillotta, Valentina
AU - Viggiano, Emanuela
AU - Cavallaro, Filippo
AU - Sframeli, Maria
AU - Bello, Luca
AU - Barp, Andrea
AU - Bonfiglio, Serena
AU - Rolle, Enrica
AU - Colia, Giulia
AU - Catteruccia, Michela
AU - Palermo, Concetta
AU - D'Angelo, Grazia
AU - Pini, Antonella
AU - Iotti, Elena
AU - Gorni, Ksenija
AU - Baranello, Giovanni
AU - Morandi, Lucia
AU - Bertini, Enrico
AU - Politano, Luisa
AU - Sormani, MariaPia
AU - Mercuri, Eugenio
PY - 2014/3
Y1 - 2014/3
N2 - 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.
AB - 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.
KW - Duchenne muscular dystrophy
KW - Upper limb
UR - http://www.scopus.com/inward/record.url?scp=84894248468&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84894248468&partnerID=8YFLogxK
U2 - 10.1016/j.nmd.2013.11.014
DO - 10.1016/j.nmd.2013.11.014
M3 - Article
C2 - 24440357
AN - SCOPUS:84894248468
VL - 24
SP - 201
EP - 206
JO - Neuromuscular Disorders
JF - Neuromuscular Disorders
SN - 0960-8966
IS - 3
ER -