Skip to main content
Top
Published in: BMC Neurology 1/2012

Open Access 01-12-2012 | Research article

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

Authors: 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

Published in: BMC Neurology | Issue 1/2012

Login to get access

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.
Appendix
Available only for authorised users
Literature
1.
go back to reference Kakulas BA: Observations on the pathogenesis of duchenne muscular dystrophy in the light of recent progress in molecular genetics. Aust Paediatr J. 1988, 24 (Suppl 1): 4-8.PubMed Kakulas BA: Observations on the pathogenesis of duchenne muscular dystrophy in the light of recent progress in molecular genetics. Aust Paediatr J. 1988, 24 (Suppl 1): 4-8.PubMed
2.
go back to reference Drousiotou A, Ioannou P, Georgiou T, et al: Neonatal screening for duchenne muscular dystrophy: a novel semiquantitative application of the bioluminescence test for creatine kinase in a pilot national program in cyprus. Genet Test. 1998, 2: 55-60. 10.1089/gte.1998.2.55.CrossRefPubMed Drousiotou A, Ioannou P, Georgiou T, et al: Neonatal screening for duchenne muscular dystrophy: a novel semiquantitative application of the bioluminescence test for creatine kinase in a pilot national program in cyprus. Genet Test. 1998, 2: 55-60. 10.1089/gte.1998.2.55.CrossRefPubMed
3.
go back to reference Parsons EP, Bradley DM, Clarke AJ: Newborn screening for duchenne muscular dystrophy. Arch Dis Child. 2003, 88: 91-92. 10.1136/adc.88.1.91.CrossRef Parsons EP, Bradley DM, Clarke AJ: Newborn screening for duchenne muscular dystrophy. Arch Dis Child. 2003, 88: 91-92. 10.1136/adc.88.1.91.CrossRef
4.
go back to reference Siciliano G, Tessa A, Renna M, et al: Epidemiology of dystrophinopathies in North-West Tuscany: a molecular genetics-based revisitation. Clin Genet. 1999, 56: 51-58. 10.1034/j.1399-0004.1999.560107.x.CrossRefPubMed Siciliano G, Tessa A, Renna M, et al: Epidemiology of dystrophinopathies in North-West Tuscany: a molecular genetics-based revisitation. Clin Genet. 1999, 56: 51-58. 10.1034/j.1399-0004.1999.560107.x.CrossRefPubMed
5.
go back to reference Merlini L, Stagni SB, Marri E, Granata C: Epidemiology of neuromuscular disorders in the under-20 population in Bologna Province, Italy. Neuromuscul Disord. 1992, 2: 197-200. 10.1016/0960-8966(92)90006-R.CrossRefPubMed Merlini L, Stagni SB, Marri E, Granata C: Epidemiology of neuromuscular disorders in the under-20 population in Bologna Province, Italy. Neuromuscul Disord. 1992, 2: 197-200. 10.1016/0960-8966(92)90006-R.CrossRefPubMed
6.
go back to reference Giacanelli M, Gneo S, Liguori M, Tarsitani G, Tessarolo D: Clinico-epidemiologic study of duchenne muscular dystrophy. Nuovi Ann Ig Microbiol. 1988, 39: 129-135.PubMed Giacanelli M, Gneo S, Liguori M, Tarsitani G, Tessarolo D: Clinico-epidemiologic study of duchenne muscular dystrophy. Nuovi Ann Ig Microbiol. 1988, 39: 129-135.PubMed
7.
go back to reference McDonald CM, Henricson EK, Han JJ, et al: The 6-minute walk test in duchenne/becker muscular dystrophy: longitudinal observations. Muscle Nerve. 2010, 42 (6): 966-974. 10.1002/mus.21808.CrossRefPubMed McDonald CM, Henricson EK, Han JJ, et al: The 6-minute walk test in duchenne/becker muscular dystrophy: longitudinal observations. Muscle Nerve. 2010, 42 (6): 966-974. 10.1002/mus.21808.CrossRefPubMed
8.
go back to reference Law PK, Goodwin TG, Fang Q, et al: Feasibility, safety, and efficacy of myoblast transfer therapy on duchenne muscular dystrophy boys. Cell Transplant. 1992, 1: 235-244.PubMed Law PK, Goodwin TG, Fang Q, et al: Feasibility, safety, and efficacy of myoblast transfer therapy on duchenne muscular dystrophy boys. Cell Transplant. 1992, 1: 235-244.PubMed
9.
go back to reference Tremblay JP, Malouin F, Roy R, et al: Results of a triple blind clinical study of myoblast transplantations without immunosuppressive treatment in young boys with duchenne muscular dystrophy. Cell Transplant. 1993, 2: 99-112.PubMed Tremblay JP, Malouin F, Roy R, et al: Results of a triple blind clinical study of myoblast transplantations without immunosuppressive treatment in young boys with duchenne muscular dystrophy. Cell Transplant. 1993, 2: 99-112.PubMed
10.
go back to reference Verdijk LB, van Loon L, Meijer K, Savelberg HH: One-repetition maximum strength test represents a valid means to assess leg strength in vivo in humans. J Sports Sci. 2009, 27: 59-68. 10.1080/02640410802428089.CrossRefPubMed Verdijk LB, van Loon L, Meijer K, Savelberg HH: One-repetition maximum strength test represents a valid means to assess leg strength in vivo in humans. J Sports Sci. 2009, 27: 59-68. 10.1080/02640410802428089.CrossRefPubMed
11.
go back to reference Drouin JM, Valovich-mcLeod TC, Shultz SJ, Gansneder BM, Perrin DH: Reliability and validity of the biodex system 3 pro isokinetic dynamometer velocity, torque and position measurements. Eur J Appl Physiol. 2004, 91: 22-29. 10.1007/s00421-003-0933-0.CrossRefPubMed Drouin JM, Valovich-mcLeod TC, Shultz SJ, Gansneder BM, Perrin DH: Reliability and validity of the biodex system 3 pro isokinetic dynamometer velocity, torque and position measurements. Eur J Appl Physiol. 2004, 91: 22-29. 10.1007/s00421-003-0933-0.CrossRefPubMed
12.
go back to reference Moxley RT, Pandya S, Ciafaloni E, Fox DJ, Campbell K: Change in natural history of duchenne muscular dystrophy with long-term corticosteroid treatment: implications for management. J Child Neurol. 2010, 25: 1116-1129. 10.1177/0883073810371004.CrossRefPubMed Moxley RT, Pandya S, Ciafaloni E, Fox DJ, Campbell K: Change in natural history of duchenne muscular dystrophy with long-term corticosteroid treatment: implications for management. J Child Neurol. 2010, 25: 1116-1129. 10.1177/0883073810371004.CrossRefPubMed
13.
go back to reference Chakkalakal JV, Thompson J, Parks RJ, Jasmin BJ: Molecular, cellular, and pharmacological therapies for duchenne/becker muscular dystrophies. FASEB J. 2005, 19: 880-891. 10.1096/fj.04-1956rev.CrossRefPubMed Chakkalakal JV, Thompson J, Parks RJ, Jasmin BJ: Molecular, cellular, and pharmacological therapies for duchenne/becker muscular dystrophies. FASEB J. 2005, 19: 880-891. 10.1096/fj.04-1956rev.CrossRefPubMed
14.
go back to reference Scott OM, Hyde SA, Goddard C, Dubowitz V: Quantitation of muscle function in children: a prospective study in duchenne muscular dystrophy. Muscle Nerve. 1982, 5: 291-301. 10.1002/mus.880050405.CrossRefPubMed Scott OM, Hyde SA, Goddard C, Dubowitz V: Quantitation of muscle function in children: a prospective study in duchenne muscular dystrophy. Muscle Nerve. 1982, 5: 291-301. 10.1002/mus.880050405.CrossRefPubMed
15.
go back to reference Mathur S, Lott DJ, Senesac C, et al: Age-related differences in lower-limb muscle cross-sectional area and torque production in boys with duchenne muscular dystrophy. Arch Phys Med Rehabil. 2010, 91: 1051-1058. 10.1016/j.apmr.2010.03.024.CrossRefPubMed Mathur S, Lott DJ, Senesac C, et al: Age-related differences in lower-limb muscle cross-sectional area and torque production in boys with duchenne muscular dystrophy. Arch Phys Med Rehabil. 2010, 91: 1051-1058. 10.1016/j.apmr.2010.03.024.CrossRefPubMed
16.
go back to reference Mazzone E, Martinelli D, Berardinelli A, et al: North star ambulatory assessment, 6-minute walk test and timed items in ambulant boys with duchenne muscular dystrophy. Neuromuscul Disord. 2010, 20: 712-716. 10.1016/j.nmd.2010.06.014.CrossRefPubMed Mazzone E, Martinelli D, Berardinelli A, et al: North star ambulatory assessment, 6-minute walk test and timed items in ambulant boys with duchenne muscular dystrophy. Neuromuscul Disord. 2010, 20: 712-716. 10.1016/j.nmd.2010.06.014.CrossRefPubMed
17.
go back to reference Brussock CM, Haley SM, Munsat TL, Bernhardt DB: Measurement of isometric force in children with and without duchenne's muscular dystrophy. Phys Ther. 1992, 72: 105-114.PubMed Brussock CM, Haley SM, Munsat TL, Bernhardt DB: Measurement of isometric force in children with and without duchenne's muscular dystrophy. Phys Ther. 1992, 72: 105-114.PubMed
18.
go back to reference Buyse GM, Goemans N, Henricson E, et al: CINRG pilot trial of oxatomide in steroid-naive duchenne muscular dystrophy. Eur J Paediatr Neurol. 2007, 11: 337-340. 10.1016/j.ejpn.2007.02.009.CrossRefPubMed Buyse GM, Goemans N, Henricson E, et al: CINRG pilot trial of oxatomide in steroid-naive duchenne muscular dystrophy. Eur J Paediatr Neurol. 2007, 11: 337-340. 10.1016/j.ejpn.2007.02.009.CrossRefPubMed
19.
go back to reference Mayhew JE, Florence JM, Mayhew TP, et al: Reliable surrogate outcome measures in multicenter clinical trials of duchenne muscular dystrophy. Muscle Nerve. 2007, 35: 36-42. 10.1002/mus.20654.CrossRefPubMed Mayhew JE, Florence JM, Mayhew TP, et al: Reliable surrogate outcome measures in multicenter clinical trials of duchenne muscular dystrophy. Muscle Nerve. 2007, 35: 36-42. 10.1002/mus.20654.CrossRefPubMed
20.
go back to reference Fowler WM, Gardner GW: Quantitative strength measurements in muscular dystrophy. Arch Phys Med Rehabil. 1967, 48: 629-644.PubMed Fowler WM, Gardner GW: Quantitative strength measurements in muscular dystrophy. Arch Phys Med Rehabil. 1967, 48: 629-644.PubMed
21.
go back to reference Hogrel JY, Payan CA, Ollivier G, et al: Development of a french isometric strength normative database for adults using quantitative muscle testing. Arch Phys Med Rehabil. 2007, 88: 1289-1297. 10.1016/j.apmr.2007.07.011.CrossRefPubMed Hogrel JY, Payan CA, Ollivier G, et al: Development of a french isometric strength normative database for adults using quantitative muscle testing. Arch Phys Med Rehabil. 2007, 88: 1289-1297. 10.1016/j.apmr.2007.07.011.CrossRefPubMed
22.
go back to reference Mazzone ES, Messina S, Vasco G, et al: Reliability of the north star ambulatory assessment in a multicentric setting. Neuromuscul Disord. 2009, 19: 458-461. 10.1016/j.nmd.2009.06.368.CrossRefPubMed Mazzone ES, Messina S, Vasco G, et al: Reliability of the north star ambulatory assessment in a multicentric setting. Neuromuscul Disord. 2009, 19: 458-461. 10.1016/j.nmd.2009.06.368.CrossRefPubMed
23.
go back to reference McDonald CM, Henricson EK, Han JJ, et al: The 6-minute walk test as a new outcome measure in duchenne muscular dystrophy. Muscle Nerve. 2010, 41: 500-510. 10.1002/mus.21544.CrossRefPubMed McDonald CM, Henricson EK, Han JJ, et al: The 6-minute walk test as a new outcome measure in duchenne muscular dystrophy. Muscle Nerve. 2010, 41: 500-510. 10.1002/mus.21544.CrossRefPubMed
24.
go back to reference Munsat TL: Development of measurement techniques. Neurology. 1996, 47: S83-S85. 10.1212/WNL.47.4_Suppl_2.83S.CrossRefPubMed Munsat TL: Development of measurement techniques. Neurology. 1996, 47: S83-S85. 10.1212/WNL.47.4_Suppl_2.83S.CrossRefPubMed
25.
go back to reference Escolar DM, Henricson EK, Pasquali L, Gorni K, Hoffman EP: Collaborative translational research leading to multicenter clinical trials in duchenne muscular dystrophy: the cooperative international neuromuscular research group (CINRG). Neuromuscul Disord. 2002, 12 (Suppl 1): S147-S154.CrossRefPubMed Escolar DM, Henricson EK, Pasquali L, Gorni K, Hoffman EP: Collaborative translational research leading to multicenter clinical trials in duchenne muscular dystrophy: the cooperative international neuromuscular research group (CINRG). Neuromuscul Disord. 2002, 12 (Suppl 1): S147-S154.CrossRefPubMed
26.
go back to reference Wyse JP, Mercer TH, Gleeson NP: Time-of-day dependence of isokinetic leg strength and associated interday variability. Br J Sports Med. 1994, 28: 167-170. 10.1136/bjsm.28.3.167.CrossRefPubMedPubMedCentral Wyse JP, Mercer TH, Gleeson NP: Time-of-day dependence of isokinetic leg strength and associated interday variability. Br J Sports Med. 1994, 28: 167-170. 10.1136/bjsm.28.3.167.CrossRefPubMedPubMedCentral
27.
go back to reference Droste SK, Collins A, Lightman SL, Linthorst AC, Reul JM: Distinct, time-dependent effects of voluntary exercise on circadian and ultradian rhythms and stress responses of free corticosterone in the rat hippocampus. Endocrinology. 2009, 150: 4170-4179. 10.1210/en.2009-0402.CrossRefPubMedPubMedCentral Droste SK, Collins A, Lightman SL, Linthorst AC, Reul JM: Distinct, time-dependent effects of voluntary exercise on circadian and ultradian rhythms and stress responses of free corticosterone in the rat hippocampus. Endocrinology. 2009, 150: 4170-4179. 10.1210/en.2009-0402.CrossRefPubMedPubMedCentral
28.
go back to reference Ives JC, Shelley GA: Psychophysics in functional strength and power training: review and implementation framework. J Strength Cond Res. 2003, 17: 177-186.PubMed Ives JC, Shelley GA: Psychophysics in functional strength and power training: review and implementation framework. J Strength Cond Res. 2003, 17: 177-186.PubMed
29.
go back to reference Househam E, McAuley J, Charles T, Lightfoot T, Swash M: Analysis of force profile during a maximum voluntary isometric contraction task. Muscle Nerve. 2004, 29: 401-408. 10.1002/mus.10564.CrossRefPubMed Househam E, McAuley J, Charles T, Lightfoot T, Swash M: Analysis of force profile during a maximum voluntary isometric contraction task. Muscle Nerve. 2004, 29: 401-408. 10.1002/mus.10564.CrossRefPubMed
Metadata
Title
Quantitative muscle strength assessment in duchenne muscular dystrophy: longitudinal study and correlation with functional measures
Authors
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
Publication date
01-12-2012
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2012
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/1471-2377-12-91

Other articles of this Issue 1/2012

BMC Neurology 1/2012 Go to the issue