The 6 minute walk test and performance of upper limb in ambulant duchenne muscular dystrophy boys

Marika Pane, Elena Stacy Mazzone, Serena Sivo, Lavinia Fanelli, Roberto De Sanctis, Adele D'Amico, Sonia Messina, Roberta Battini, Flaviana Bianco, Marianna Scutifero, Roberta Petillo, Silvia Frosini, Roberta Scalise, Gian Luca Vita, Claudio Bruno, Marina Pedemonte, Tiziana Mongini, Elena Pegoraro, Francesca Brustia, Alice Gardani, Angela Berardinelli, Valentina Lanzillotta, Emanuela Viggiano, Filippo Cavallaro, Maria Sframeli, Luca Bello, Andrea Barp, Fabio Busato, Serena Bonfiglio, Enrica Rolle, Giulia Colia, Annamaria Bonetti, Concetta Palermo, Alessandra Graziano, Grazia D'Angelo, Antonella Pini, Alice Corlatti, Ksenija Gorni, Giovanni Baranello, Laura Antonaci, Enrico Bertini, Luisa Politano, Eugenio Mercuri
PLoS Currents 2014, 6
The Performance of Upper Limb (PUL) test was specifically developed for the assessment of upper limbs in Duchenne muscular dystrophy (DMD). The first published data have shown that early signs of involvement can also be found in ambulant DMD boys. The aim of this longitudinal Italian multicentric study was to evaluate the correlation between the 6 Minute Walk Test (6MWT) and the PUL in ambulant DMD boys. Both 6MWT and PUL were administered to 164 ambulant DMD boys of age between 5.0 and 16.17 years (mean 8.82). The 6 minute walk distance (6MWD) ranged between 118 and 557 (mean: 376.38, SD: 90.59). The PUL total scores ranged between 52 and 74 (mean: 70.74, SD: 4.66). The correlation between the two measures was 0.499. The scores on the PUL largely reflect the overall impairment observed on the 6MWT but the correlation was not linear. The use of the PUL appeared to be less relevant in the very strong patients with 6MWD above 400 meters, who, with few exceptions had near full scores. In patients with lower 6MWD the severity of upper limb involvement was more variable and could not always be predicted by the 6MWD value or by the use of steroids. Our results confirm that upper limb involvement can already be found in DMD boys even in the ambulant phase.

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