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Upper Limb Strength and Function Changes during a One-Year Follow-Up in Non-Ambulant Patients with Duchenne Muscular Dystrophy: An Observational Multicenter Trial
Authors:Andreea Mihaela Seferian  Amélie Moraux  Mélanie Annoussamy  Aurélie Canal  Valérie Decostre  Oumar Diebate  Anne-Ga?lle Le Moing  Teresa Gidaro  Nicolas Deconinck  Frauke Van Parys  Wendy Vereecke  Sylvia Wittevrongel  Michèle Mayer  Kim Maincent  Isabelle Desguerre  Christine Thémar-No?l  Jean-Marie Cuisset  Vincent Tiffreau  Severine Denis  Virginie Jousten  Susana Quijano-Roy  Thomas Voit  Jean-Yves Hogrel  Laurent Servais
Abstract:IntroductionUpper limb evaluation of patients with Duchenne Muscular Dystrophy is crucially important to evaluations of efficacy of new treatments in non-ambulant patients. In patients who have lost ambulation, there are few validated and informative outcome measures. In addition, longitudinal data demonstrating sensitivity to clinical evolution of outcome measures over short-term periods are lacking.ResultsOur study confirmed preliminary data previously reported regarding feasibility of use and of reliability of the MyoSet and the correlation at baseline between distal strength and clinical outcomes such as FVC, Brooke score, age, and duration since loss of ambulation. A significant correlation was observed between the distal upper limb strength and clinical variables. The sensitive dynamometers (MyoGrip and MyoPinch) and MoviPlate captured a 12-month change in non-ambulant Duchenne muscular dystrophy patients of all ages.

Trial Registration

ClinicalTrials.gov NCT00993161 NCT00993161
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