Upper limb function in ambulatory and non-ambulatory patients with Duchenne muscular dystrophy

Authors

DOI:

https://doi.org/10.1590/1809-2950/19017427022020

Keywords:

Muscular Dystrophy, Duchenne; Upper Extremity;, Physiotherapy

Abstract

New treatments proposed for patients with Duchenne muscular dystrophy (DMD) have their efficacy evaluated by lower limb tests. However, upper limb function tests evaluate both ambulatory (A) and non-ambulatory (NA) people. Thus, this work aimed to compare the upper limb function of patients A and NA with DMD and to correlate disease staging and upper limb function. This is a cross-sectional study in which patients were divided into A and NA according to the Vignos scale. Subsequently, the upper limb function was evaluated by the performance of upper limb (PUL) scale. The independent t-test, chi-square test, Mann-Whitney test, linear regression and Spearman’s correlation test were performed by SPSS, version 22. The research dealt with 51 patients, 20 A and 31 NA. There were differences between the groups reference age (p=0.001), body mass index (BMI) (p=0.016), schooling (p=0.011), Vignos score (p<0.001) and upper limb function (p<0.001). The linear regression analysis showed that whether the patient was A or NA influenced the upper limb function in 18 points on the PUL scale. There was a strong correlation between disease staging and upper limb function (r2=−0.769, p<0.001). The functional condition of the upper limb depends on whether the patient is A or NA, being worse function in NA patients. We concluded that the upper limb function has a strong correlation with the staging of the disease.

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References

Suthar R, Sankhyan N. Duchenne Muscular Dystrophy:

A Practice Update. Indian J Pediatr. 2018;85:276-81. doi:

1007/s12098-017-2397-y

Birnkrant DJ, Bushby K, Bann CM, Apkon SD, Blackwell A,

Brumbaugh D, et al. Diagnosis and management of Duchenne

muscular dystrophy, part 1: Diagnosis, and neuromuscular,

rehabilitation, endocrine, and gastrointestinal and nutritional

management. Lancet Neurol. 2018;17(3):251-67. doi: 10.1016/

s1474-4422(18)30024-3

Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Cripe L,

et al. Diagnosis and management of Duchenne muscular

dystrophy, part 2: Implementation of multidisciplinary care. Lancet

Neurol. 2010;9(2):177-89. doi: 10.1016/s1474-4422(09)70271-6.

Victor RG, Sweeney HL, Finkel R, McDonald CM, Byrne B, Eagle M,

et al. A phase 3 randomized placebo-controlled trial

of tadalafil for Duchenne muscular dystrophy. Neurology.

;89(17):1811-20. doi: 10.1212/wnl.0000000000004570

McDonald CM, Campbell C, Torricelli RE, Finkel RS, Flanigan

KM, Goemans N, et al. Ataluren in patients with nonsense

mutation Duchenne muscular dystrophy (ACT DMD):

A multicentre, randomised, double-blind, placebo-controlled,

phase 3 trial. Lancet. 2017;390(10101):1489-98. doi: 10.1016/

s0140-6736(17)31611-2

Araujo A, Nardes F, Fortes C, Pereira JA, Rebel MF, Dias CM,

et al. Brazilian consensus on Duchenne muscular dystrophy.

Part 2: Rehabilitation and systemic care. Arq Neuropsiquiatr.

;76(7):481-9. 10.1590/0004-282x20180062

Santos NM, Rezende M, Terni A, Hayashi MCB, Fávero FM, Quadros

AAJ, et al. Perfil clínico e funcional dos pacientes com distrofia

muscular de Duchenne assistidos na Associação Brasileira de

Distrofia Muscular (ABDIM). Rev Neurociênc. 2006;14(1):15-22.

Vignos PJ Jr., Archibald KC. Maintenance of ambulation in childhood

muscular dystrophy. J Chronic Dis. 1960;12(2):273-90.

doi: 10.1016/0021-9681(60)90105-3

Brucki SM, Nitrini R, Caramelli P, Bertolucci PH, Okamoto IH.

Sugestões para o uso do mini-exame do estado mental no

Brasil. Arq Neuropsiquiatr. 2003;61(3B):777-81. doi: 10.1590/

S0004-282X2003000500014

Voos M, Fávero FM, Dias K, Artiheiro M, Oliveira A, Caromano

F. Dissociation between motor and cognitive skills in patients

with Duchenne muscular dystrophy. Neuromuscul Disord.

;25(Suppl 2)S306-23. doi: 10.1016/j.nmd.2015.06.427

Mayhew A, Mazzone ES, Eagle M, Duong T, Ash M, Decostre

V, et al. Development of the performance of the upper limb

module for Duchenne muscular dystrophy. Dev Med Child

Neurol. 2013;55(11):1038-45. doi: 10.1111/dmcn.12213

Pane M, Mazzone ES, Fanelli L, de Sanctis R, Bianco F, Sivo S,

et al. Reliability of the performance of upper limb assessment

in Duchenne muscular dystrophy. Neuromuscul Disord.

;24(3):201-6. doi: 10.1016/j.nmd.2013.11.014.

Hulley SB, Cummings SR, Browner WS, Grady DG, Newman

TB. Delineando a pesquisa clinica: uma abordagem

epidemiológica. 3 ed. São Paulo: Artmed; 2008.

Emery AE. The muscular dystrophies. Lancet.

;359(9307):687-95. doi: 10.1016/s0140-6736(02)07815-7

Mattar FL, Sobreira C. Hand weakness in Duchenne muscular

dystrophy and its relation to physical disability. Neuromuscul

Disord. 2008;18(3):193-8. doi: 10.1016/j.nmd.2007.11.004

Pane M, Mazzone ES, Sivo S, Fanelli L, de Sanctis R, D’Amico

A, et al. The 6 minute walk test and performance of upper

limb in ambulant Duchenne muscular dystrophy boys. PLoS

Curr. 2014;6. doi: 10.1371/currents.md.a93d9904d57dcb08

f2ea89bca6fe6

Janssen MM, Hendriks JC, Geurts AC, de Groot IJ. Variables

associated with upper extremity function in patients with

Duchenne muscular dystrophy. J Neurol. 2016;263(9):1810-8.

doi: 10.1007/s00415-016-8193-1

Brogna C, Cristiano L, Tartaglione T, Verdolotti T, Fanelli L,

Ficociello L, et al. Functional levels and MRI patterns of

muscle involvement in upper limbs in Duchenne muscular

dystrophy. PLoS One. 2018;13(6):e0199222. doi:10.1371/journal.

pone.0199222

Janssen M, Harlaar J, Koopman B, de Groot IJM. Dynamic arm

study: Quantitative description of upper extremity function

and activity of boys and men with Duchenne muscular

dystrophy. J Neuroeng Rehabil. 2017;14(1):[14 p]. doi:10.1186/

s12984-017-0259-5

Deconinck N, Dan B. Pathophysiology of Duchenne muscular

dystrophy: Current hypotheses. Pediatr Neurol. 2007;36(1):1-7.

doi:10.1016/j.pediatrneurol.2006.09.016

Pane M, Coratti G, Brogna C, Mazzone ES, Mayhew A, Fanelli

L, et al. Upper limb function in Duchenne muscular dystrophy:

month longitudinal data. PLoS One. 2018;13(6):e0199223.

doi:10.1371/journal.pone.0199223

Birnkrant DJ, Bushby K, Bann CM, Apkon SD, Blackwell A,

Colvin MK, et al. Diagnosis and management of Duchenne

muscular dystrophy, part 3: Primary care, emergency

management, psychosocial care, and transitions of care across

the lifespan. Lancet Neurol. 2018;17(5):445-55. doi:10.1016/

s1474-4422(18)30026-7

Published

2020-03-03

Issue

Section

Original Research

How to Cite

Upper limb function in ambulatory and non-ambulatory patients with Duchenne muscular dystrophy. (2020). Fisioterapia E Pesquisa, 27(2), 188-193. https://doi.org/10.1590/1809-2950/19017427022020