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

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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.

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          Most cited references29

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          Reliability and validity of the Biodex system 3 pro isokinetic dynamometer velocity, torque and position measurements.

          This study quantitatively assessed the mechanical reliability and validity of position, torque and velocity measurements of the Biodex System 3 isokinetic dynamometer. Trial-to-trial and day-to-day reliability were assessed during three trials on two separate days. To assess instrument validity, measurement of each variable using the Biodex System 3 dynamometer was compared to a criterion measure of position, torque and velocity. Position was assessed at 5 degrees increments across the available range of motion of the dynamometer. Torque measures were assessed isometrically by hanging six different calibrated weights from the lever arm. Velocity was assessed (30 degrees/s to 500 degrees/s) across a 70 degrees arc of motion by manually accelerating the weighted lever arm. With the exception of a systematic decrease in velocity at speeds of 300 degrees/s and higher, the Biodex System 3 performed with acceptable mechanical reliability and validity on all variables tested.
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            Change in natural history of Duchenne muscular dystrophy with long-term corticosteroid treatment: implications for management.

            In 2005, the American Academy of Neurology and the Child Neurology Society published a practice parameter, based primarily on studies that involved 6 to 18 months of treatment, indicating that prednisone has a beneficial effect on muscle strength and function in patients with Duchenne muscular dystrophy and recommended that corticosteroids be offered (prednisone 0.75 mg/kg/d and deflazacort 0.9 mg/kg/d) as treatment. Recent reports emphasize that longer term treatment with corticosteroids (greater than 3 years) produces important sustained benefits in neuromuscular function without causing major side effects. This review highlights these reports and indicates that long-term corticosteroid therapy (1) prolongs ambulation by 2 to 5 years, (2) reduces the need for spinal stabilization surgery, (3) improves cardiopulmonary function, (4) delays the need for noninvasive nasal ventilation, and (5) increases survival and the quality of life of patients with Duchenne muscular dystrophy. Educational, vocational, and other social counseling is now a vital part of management for Duchenne muscular dystrophy.
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              One-repetition maximum strength test represents a valid means to assess leg strength in vivo in humans.

              Skeletal muscle strength is often determined to evaluate the adaptive response to an exercise intervention programme. Although dynamometry is considered the "gold standard" for the assessment of muscle strength in vivo, one-repetition maximum (1-RM) testing performed on training-specific equipment is more commonly applied. We assessed the validity of specific knee extension 1-RM testing by comparison with dynamometry in a heterogeneous population (n=55). All participants performed 1-RM tests on regular leg extension and leg press machines. Additionally, isometric (at seven different knee angles) and isokinetic (at four different velocities) knee extension peak torques were determined. Pearson's r was calculated for the relationship between 1-RM data and peak torques for the entire population and for subgroups defined by age and gender. One-repetition maximum strength correlated strongly with the dynamometer results. One-repetition maximum leg extension correlated more strongly with peak torques than did 1-RM leg press (0.78
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                Author and article information

                Journal
                BMC Neurol
                BMC Neurol
                BMC Neurology
                BioMed Central
                1471-2377
                2012
                13 September 2012
                : 12
                : 91
                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
                Article
                1471-2377-12-91
                10.1186/1471-2377-12-91
                3482602
                22974002
                cfcceba8-3832-4a77-a36f-8112d7145515
                Copyright ©2012 Lerario et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 September 2011
                : 16 August 2012
                Categories
                Research Article

                Neurology
                Neurology

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