North Star Ambulatory Assessment, 6-minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy

Elena Mazzone, Diego Martinelli, Angela Berardinelli, Sonia Messina, Adele D'Amico, Gessica Vasco, Marion Main, Luca Doglio, Luisa Politano, Filippo Cavallaro, Silvia Frosini, Luca Bello, Adelina Carlesi, Anna Maria Bonetti, Elisabetta Zucchini, Roberto De Sanctis, Marianna Scutifero, Flaviana Bianco, Francesca Rossi, Maria Chiara Motta, Annalisa Sacco, Maria Alice Donati, Tiziana Mongini, Antonella Pini, Roberta Battini, Elena Pegoraro, Marika Pane, Elisabetta Pasquini, Claudio Bruno, Giuseppe Vita, Chiara de Waure, Enrico Bertini, Eugenio Mercuri
Neuromuscular Disorders: NMD 2010, 20 (11): 712-6
The North Star Ambulatory Assessment is a functional scale specifically designed for ambulant boys affected by Duchenne muscular dystrophy (DMD). Recently the 6-minute walk test has also been used as an outcome measure in trials in DMD. The aim of our study was to assess a large cohort of ambulant boys affected by DMD using both North Star Assessment and 6-minute walk test. More specifically, we wished to establish the spectrum of findings for each measure and their correlation. This is a prospective multicentric study involving 10 centers. The cohort included 112 ambulant DMD boys of age ranging between 4.10 and 17 years (mean 8.18±2.3 DS). Ninety-one of the 112 were on steroids: 37/91 on intermittent and 54/91 on daily regimen. The scores on the North Star assessment ranged from 6/34 to 34/34. The distance on the 6-minute walk test ranged from 127 to 560.6 m. The time to walk 10 m was between 3 and 15 s. The time to rise from the floor ranged from 1 to 27.5 s. Some patients were unable to rise from the floor. As expected the results changed with age and were overall better in children treated with daily steroids. The North Star assessment had a moderate to good correlation with 6-minute walk test and with timed rising from floor but less with 10 m timed walk/run test. The 6-minute walk test in contrast had better correlation with 10 m timed walk/run test than with timed rising from floor. These findings suggest that a combination of these outcome measures can be effectively used in ambulant DMD boys and will provide information on different aspects of motor function, that may not be captured using a single measure.

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