Open Access Research article

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

Alberto Lerario19, Serena Bonfiglio19, MariaPia Sormani29, Andrea Tettamanti39, Sarah Marktel49, Sara Napolitano49, Stefano Previtali59, Marina Scarlato59, MariaGrazia Natali-Sora59, Eugenio Mercuri69, Nereo Bresolin19, Tiziana Mongini79, Giancarlo Comi59, Roberto Gatti39, Fabio Ciceri49, Giulio Cossu89 and Yvan Torrente19*

Author Affiliations

1 Dipartimento di Fisiopatologia medico-chirurgica e dei Trapianti, Centro Dino Ferrari, Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Università di Milano, Fondazione, Ospedale Maggiore Policlinico di Milano, Via F. Sforza n35, Milano, 20122, Italy

2 Biostatistics Unit, Dep. of Health Sciences, University of Genoa, Genoa, Italy

3 School of Physiotherapy, University Vita-Salute San Raffaele, Milan, Italy

4 Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Milan, Italy

5 Division of Neuroscience, Department of Neurology and INSPE, San Raffaele Scientific Institute, Milan, Italy

6 Department of Pediatric Neurology, Catholic University, Policlinico Gemelli, Largo Gemelli, 00168, Rome, Italy

7 Neuromuscular Center "P. Peirolo", Department of Neuroscience, S.Giovanni Battista Hospital of Turin, Turin, Italy

8 Division of Regenerative Medicine, San Raffaele Scientific Institute, Milan, Italy

9 Department of Cell and Developmental Biology, University College London, Rm 545, Rockefeller Bldg. 21 University Street, London, WC1E 6DE, UK

For all author emails, please log on.

BMC Neurology 2012, 12:91  doi:10.1186/1471-2377-12-91

Published: 13 September 2012

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.