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Quantitative muscle strength assessment in Duchenne Muscular Dystrophy: longitudinal study and correlation with functional measures


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Publication Date: 
05/09/2012
Authors: 
Lerario A, Bonfiglio S, Sormani MP, Tettamanti A, Marktel S, Napolitano S, Previtali S, Scarlato M, Natali-Sora MG, Mercuri E, Bresolin N, Mongini T, Comi G, Gatti R, Ciceri F, Cossu G, Torrente Y
Citation: 
BMC Neurol. 2012 Sep 13;12:91
DOI: 
10.1186/1471-2377-12-91.
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 three 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.