Suitability of north star ambulatory assessment in young boys with Duchenne muscular dystrophy

Roberto De Sanctis, Marika Pane, Serena Sivo, Valeria Ricotti, Giovanni Baranello, Silvia Frosini, Elena Mazzone, Flaviana Bianco, Lavinia Fanelli, Marion Main, Alice Corlatti, Adele D'Amico, Giulia Colia, Roberta Scalise, Concetta Palermo, Chiara Alfonsi, Giovanna Tritto, Domenico M. Romeo, Alessandra Graziano, Roberta BattiniLucia Morandi, Enrico Bertini, Francesco Muntoni, Eugenio Mercuri

Research output: Contribution to journalArticle

Abstract

The aim of this study was to establish the suitability of the North Star Ambulatory Assessment for use in young boys with Duchenne muscular dystrophy. We studied 147 typically developing and 144 boys affected by Duchenne muscular dystrophy between the ages of 3 and 5 years. More than 85% of the typically developing boys by the age of 4 years had full scores on all the items with total scores ≥33/34. Before the age of 4 years more than 15% of the typically developing boys did not achieve full scores on all the items. Some items, such as standing on one leg, showed significant improvement with age. In contrast, other activities were rarely achieved even in the older boys. Even if there was a progressive increase in scores with age, both total and individual item scores in Duchenne were still far from those obtained in the typically developing children of the same age. Our findings suggest that the North Star Ambulatory Assessment can be reliably used at least from the age of 4 years. Longitudinal natural history data studies are needed to assess possible changes over time and the possible effect of early steroids.

Original languageEnglish
Pages (from-to)14-18
Number of pages5
JournalNeuromuscular Disorders
Volume25
Issue number1
DOIs
Publication statusPublished - Jan 1 2015

Keywords

  • Duchenne muscular dystrophy
  • North star ambulatory assessment
  • Outcome measure
  • Typically developing boys

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Genetics(clinical)
  • Neurology

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  • Cite this

    De Sanctis, R., Pane, M., Sivo, S., Ricotti, V., Baranello, G., Frosini, S., Mazzone, E., Bianco, F., Fanelli, L., Main, M., Corlatti, A., D'Amico, A., Colia, G., Scalise, R., Palermo, C., Alfonsi, C., Tritto, G., Romeo, D. M., Graziano, A., ... Mercuri, E. (2015). Suitability of north star ambulatory assessment in young boys with Duchenne muscular dystrophy. Neuromuscular Disorders, 25(1), 14-18. https://doi.org/10.1016/j.nmd.2014.09.015