Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
6041423 | Neuromuscular Disorders | 2014 | 6 Pages |
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 3Â 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 25Â years old. A full score was consistently achieved by the age of 5Â 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.
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Authors
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,