T.P.13: Upper limb performance changes during a one-year follow-up in non-ambulant patients with Duchenne muscular dystrophy.

Upper limb evaluation of patients with Duchenne Muscular Dystrophy is crucially important to evaluate efficacy of new treatments in non-ambulant patients. Adequate outcome measures are scarce for patients who have lost ambulation. In addition, longitudinal data demonstrating sensitivity to clinical evolution of outcome measures on a short term period are lacking. We report here the results of a one-year multicenter study using specifically designed tools to assess grip and key pinch strength and hand function in wheelchair bound patients. Our study recruited 53 non-ambulant patients with Duchenne muscular atrophy aged 17.1 ± 4.8 years (range: 9–28.1 years). The Brooke functional score was 4.6 ± 1.1. The average forced vital capacity was 44.5 % and nineteen patients used non-invasive ventilation. Patients were assessed at baseline, 6 months and one year using the Motor Function Measure and innovative devices (namely the MyoSet composed of MyoGrip, MyoPinch and MoviPlate). Our study confirmed the preliminary data previously reported regarding feasibility, reliability of the MyoSet and correlation between the distal strength and clinical variables. We demonstrated that the use of sensitive dynamometers could capture a 12-month change in non-ambulant Duchenne muscular dystrophy patients of all ages. [ABSTRACT FROM AUTHOR]