Impact of proximal muscle weakness of lower limbs on balance and functionality of individuals with Charcot-Marie-Tooth type 2 disease

Authors

DOI:

https://doi.org/10.33448/rsd-v11i12.33108

Keywords:

Charcot-Marie-Tooth; Muscle strength; Postural balance; Functionality.

Abstract

Introduction: Charcot-Marie-Tooth disease (CMT) is the most prevalent genetically determined neurological condition in the world, individuals have muscle weakness, atrophy and sensory deficit. Objective: Assess lower limb and trunk muscle strength in individuals with CMT type 2 and its correlations with balance and functionality. Methods: Cross-sectional study, with a sample of fifteen individuals in the CMT2 group (CMT2G) and fifteen in the control group (CG). To assess muscle strong (MS), the dynamometer was used; balance through stabilometry (Velocity of the Center of Pressure Oscillation (VCPo) anteroposterior (AP) and latero-lateral (LL); and the Berg Balance Scale (BSE); and for functional performance, the Time Up and Go (TUG). Results: There was a significant reduction in proximal MS of lower limbs, abdominal and paravertebral; lower BSE score and lower TUG of CMT2G compared to CG. When analyzing the correlations of LL and trunk strength with VCPoAP in CMT2G (individuals with a mild score), the muscles with the highest correlation were: hip and paravertebral extensors/abductors/adductors, while in moderate/severe individuals, there was a regular correlation with hip and paravertebral flexors. In the correlations with BSE (light score), the paravertebral muscles had a regular correlation and in the most affected there was a strong correlation with the hip extensors/flexors/abductors and regular with the abdominals. In the correlations with the TUG (light score), there was a regular correlation with the hip, abdominal and paravertebral flexors. In moderate/severe individuals, the hip and paravertebral flexors had a slight correlation.

References

Albiero J et al. (2010). Doença de Charcot-Marie-Tooth: relato de caso e revisão literária de tratamento fisioterapêutico. Trabalho apresentado no XIII Congresso Estadual das APAEs IV Fórum de Autodefensores 28 a 30 de março de 2010 Parque Vila Germânica, Setor 2 – Blumenau (SC), BRASIL. https://www.tecnoevento.com.br/eve9/arq/DOENCA%20DE%20CHARCOT%20MARIE%20TOOTH.pdf

Berg K O, et al. (1992). Measuring balance in the elderly: validation of an instrument. Can J Public Health; 83 (Suppl), S7-11.

Bragadin M M et al. (2011). Outcome measures and a rehabilitation treatment in patients affected by Charcot-Marie-Tooth Neuropathy: A Pilot Study. Am J Phys Med Rehabil. 2011 Aug 8; 90, 628-637. PMid:21681064.

Braz N et al. (2018). Effectiveness of Nintendo Wii in functional and health outcomes of individuals with Parkinson’s disease: a systematic review. Fisioter Pesqui. 2018; 25(1), 100-106. DOI: 10.1590/1809-2950/17131825012018.

De França Costa et al. (2018). Evaluation of muscle strength, balance and functionality of individuals with type 2 Charcot-Marie-Tooth Disease. Gait & Posture. May, 62, 463-467.

Dick P J, & Lambert E H. (2014). Lower motor and primary sensory neuron diseases with peroneal muscular atrophy. I. Neurologic, genetic, and electrophysiologic findings in hereditary polyneuropathies. Arch Neurol 1968; 18:603-18. apud GONDIM, Francisco de Assis Aquino, et al, Fenótipos Raros de Neuropatia Hereditária: Charcot-Marie-Tooth Tipo 4. Rev Neurocienc; 22(1), 84-94.

Don R et al. (2007). Foot drop and plantar flexion failure determine different gait strategies in Charcot-Marie-Tooth patients. Clin Biomech (Bristol, Avon). Oct; 22(8), 905-16.

Fávero R A et al. (2010). Análise da marcha e funcionalidade na doença de Charcot-Marie Tooth: relato de caso. Rev Neurocienc 18(1), 44-49.

Ferla F L et al. (2015). Fisioterapia no tratamento do controle de tronco e equilíbrio de pacientes pós AVC. Rev Neurocienc 23(2), 211-217.

Kennedy R et al. (2016). Gait in children and adolescents with Charcot-Marie-Tooth disease: a systematic review. J Peripher Nerv Syst. Dec; 21(4), 317-328. doi: 10.1111/jns.12183.

Kuciel N M et al. (2016). Lower extremity muscles activity in standing and sitting position with use of sEMG in patients suffering from Charcot–Marie–Tooth syndrome. Neurol Neurochir Pol. 2016; 50(3), 195-9. Doi: 10.1016/j.pjnns.2016.01.011.

Lemos L F C et al. (2009). Uma revisão sobre centro de gravidade e equilíbrio corporal. R. bras. Ci. e Mov; 17(4), 83-90.

Lencioni T et al. (2014). Postural stabilization and balance assessment in Charcot–Marie–Tooth 1A subjects. Gait & Posture 40, 481–86.

Lencioni T et al. (2016). The influence of somatosensory and muscular deficits on postural stabilization: Insights from an instrumented analysis of subjects affected by different types of Charcot–Marie–Tooth disease. Neuromuscular Disorders 25, 640–45.

Lima et al., (2008).Versão brasileira da Escala de Comprometimento do Tronco: um estudo de validade em sujeitos pós-acidente vascular encefálico. Fisioter. Pesqui. 15(3).

Martín L A M et al. (2005). Actualidad genética y clinica en las polineuropatias sensoriomotoras hereditárias. Rev. habanera cienc. Méd. 2005, 4(5).

Martinelli A R et al. (2014). Changes of the gait parameters and sensory-motor deficit associated with peripheral diabetic neuropathy. Acta Fisiatr; 21(1), 36-40.

Miniou P, & Fontes M. T (2021). herapeutic Development in Charcot Marie Tooth Type 1 Disease. Int J Mol Sci. 2021 Jun 23; 22(13), 6755. doi: 10.3390/ijms22136755. PMID: 34201736; PMCID: PMC8268813.

Morena J, Gupta A, & Hoyle J C. (2019). Charcot-Marie-Tooth: From Molecules to Therapy. Int J Mol Sci. 2019 Jul 12; 20(14), 3419. doi: 10.3390/ijms20143419. PMID: 31336816; PMCID: PMC6679156.

Neves E L A, & Kok F. (2011). Clinical and neurophysiological investigation of a large family with dominant Charcot-Marie-Tooth type 2 disease with pyramidal signs. Arq Neuropsiquiatr 69(3), 424-30.

Newman C J et al. (2007). The characteristics of gait in Charcot-Marie-Tooth disease types I and II. Gait & Posture. 2, 120-127.

Pareyson D, & Marchesi C. (2009). Diagnosis, natural history, and management of Charcot-Marie-Tooth disease. Lancet Neurol; 8, 654–67.

Park R et al. (2013). Changes in Regional Activity of the Psoas Major and Quadratus Lumborum With Voluntary Trunk and Hip Tasks and Different Spinal Curvatures in Sitting. J Orthop Sports Phys Ther. Feb; 43(2), 74-82.

Piovesan A C et al. (2015). Avaliação do Teste de Tinetti e Mini-Exame do Estado Mental em idosas moradoras da comunidade Roberto Binatto, Santa Maria (RS). Revista Kairós Gerontologia. 2015; 18(1), 341-352. ISSN 1516-2567. ISSNe 2176-901X. São Paulo (SP), Brasil: FACHS/NEPE/PEPGG/PUC-SP.

Ramdharry G M et al. (2014). A pilot study of proximal strength training in Charcot-Marie-Tooth disease. J. Peripher Nerv Syst. 2014. Dec; 19(4), 328-32.

Ramdharry G M et al. (2009). Hip flexor fatigue limits walking in Charcot-Marie-Tooth disease. Muscle & Nerve, 40, 103-111,.

Sautreuil P, Delorme D, Baron A, Mane M, Missaoui B, & Thoumie P. (2017). Maladie de Charcot-Marie-Tooth - Éléments de rééducation fonctionnelle, kinésithérapie, ergothérapie [Charcot Marie Tooth disease: principles of rehabilitation, physiotherapy and occupational therapy]. Med Sci (Paris). 2017 Nov;33 Hors série n°1:49-54. French. doi: 10.1051/medsci/201733s110. Epub Nov 15. PMID: 29139387.

Silva D L et al. (2007). Otorhinolaryngological Clinical Features of Charcot-Marie-Tooth Disease. Arq. Int. Otorrinolaringol./Intl. Arch. Otorhinolaryngol., São Paulo, 11(4), 472-476.

Silva R et al. (2011). Alterações neuromusculares no quadril associadas a entorses do tornozelo: revisão de literatura. Fisioter. Mov., Curitiba, jul./set; 24(3), 503-511.

Stavrou M, Sargiannidou I, Georgiou E, Kagiava A, Kleopa K A. (2021). Emerging Therapies for Charcot-Marie- Tooth Inherited Neuropathies. Int J Mol Sci. Jun 3; 22(11), 6048. doi: 10.3390/ijms22116048. PMID: 34205075; PMCID: PMC8199910.

Teixeira C L. (2010). Equilibrio e controle postural. Brazilian Journal of Biomechanics, 11(20).

Published

07/09/2022

How to Cite

VASCONCELOS , J. M. de A. e; SILVA, J. P. R. .; OLIVEIRA, P. M. P. .; NEVES, E. L. de A. .; COELHO DE SOUZA, C.; BARRETO, L. C. L. S. .; CAJUEIRO, C. A. G.; COSTA, I. M. P. de F.; ARAUJO, A. A. de S.; NUNES, P. S. . Impact of proximal muscle weakness of lower limbs on balance and functionality of individuals with Charcot-Marie-Tooth type 2 disease . Research, Society and Development, [S. l.], v. 11, n. 12, p. e55111233108, 2022. DOI: 10.33448/rsd-v11i12.33108. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/33108. Acesso em: 19 nov. 2024.

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Section

Health Sciences