Quantitative muscle strength assessment in duchenne muscular dystrophy: longitudinal study and correlation with functional measures

Alberto Lerario, Serena Bonfiglio, MariaPia Sormani, Andrea Tettamanti, Sarah Marktel, Sara Napolitano, Stefano Previtali, Marina Scarlato, MariaGrazia Natali-Sora, Eugenio Mercuri, Nereo Bresolin, Tiziana Mongini, Giancarlo Comi, Roberto Gatti, Fabio Ciceri, Giulio Cossu, Yvan Torrente

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    Abstract

    Background: The aim of this study was to perform a longitudinal assessment using Quantitative Muscle Testing (QMT) in a cohort of ambulant boys affected by Duchenne muscular dystrophy (DMD) and to correlate the results of QMT with functional measures. This study is to date the most thorough long-term evaluation of QMT in a cohort of DMD patients correlated with other measures, such as the North Star Ambulatory Assessment (NSAA) or thee 6-min walk test (6MWT).Methods: This is a single centre, prospective, non-randomised, study assessing QMT using the Kin Com® 125 machine in a study cohort of 28 ambulant DMD boys, aged 5 to 12 years. This cohort was assessed longitudinally over a 12 months period of time with 3 monthly assessments for QMT and with assessment of functional abilities, using the NSAA and the 6MWT at baseline and at 12 months only. QMT was also used in a control group of 13 healthy age-matched boys examined at baseline and at 12 months.Results: There was an increase in QMT over 12 months in boys below the age of 7.5 years while in boys above the age of 7.5 years, QMT showed a significant decrease. All the average one-year changes were significantly different than those experienced by healthy controls. We also found a good correlation between quantitative tests and the other measures that was more obvious in the stronger children.Conclusion: Our longitudinal data using QMT in a cohort of DMD patients suggest that this could be used as an additional tool to monitor changes, providing additional information on segmental strength. © 2012 Lerario et al.; licensee BioMed Central Ltd.
    Original languageEnglish
    Article number91
    JournalBMC Neurology
    Volume12
    DOIs
    Publication statusPublished - 13 Sep 2012

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