Funcionalidade de membro superior em pacientes deambuladores e não deambuladores com distrofia muscular de Duchenne

Abstract

RESUMO Novos tratamentos propostos para os pacientes com distrofia muscular de Duchenne (DMD) têm sua eficácia avaliada por testes de membros inferiores. Contudo, os testes funcionais de membros superiores (MMSS) avaliam tanto deambuladores (D) como não deambuladores (ND). Assim, este estudo se propôs a comparar a funcionalidade de MMSS de pacientes D e ND com DMD e correlacionar o estadiamento da doença e a função de MMSS. Trata-se de um estudo transversal no qual os pacientes foram divididos em D e ND de acordo com a escala Vignos. Posteriormente, a funcionalidade dos MMSS foi avaliada pela escala performace of upper limb (PUL). Foi realizado o teste t independente, teste qui-quadrado, teste Mann-Whitney, regressão linear e teste de correlação de Spearman pelo programa SPSS, versão 22. A pesquisa foi composta de 51 pacientes, sendo 20 D e 31 ND. Houve diferença entre os grupos em relação à idade (p=0,001), ao índice de massa corporal (IMC) (p=0,016), à escolaridade (p=0,011), quanto ao escore na escala Vignos (p<0,001) e na função dos MMSS (p<0,001). Na análise de regressão linear observamos que o paciente ser D ou ND influencia na função dos MMSS em 18 pontos na escala PUL. Houve forte correlação entre o estadiamento da doença e a função de MMSS (r2=−0,769, p<0,001). A condição funcional dos MMSS depende se o paciente é D ou ND, sendo pior nos ND. Concluiu-se que a função de MMSS tem forte correlação com o estadiamento da doença.

Motor Compensation Strategies for Reduced Upper Limb Function Among Individuals With Duchenne Muscular Dystrophy

“…After its recent update, PUL version 2.0, the PUL even provides a score for the examinee's motor compensations during task execution of (Pane et al, 2018). But a notorious problem with these measures, particularly for non-walkers, is their reliance on weight bearing task performance (Santos et al, 2020). Thus, the JTT has been used to assess upper limb function among patients with DMD (Artilheiro et al, 2017a(Artilheiro et al, , 2017bMaciel et al, in press, 2021;Santos et al, 2020).…”

“…But a notorious problem with these measures, particularly for non-walkers, is their reliance on weight bearing task performance (Santos et al, 2020). Thus, the JTT has been used to assess upper limb function among patients with DMD (Artilheiro et al, 2017a(Artilheiro et al, , 2017bMaciel et al, in press, 2021;Santos et al, 2020). But the JTT allows examinees to execute its tasks in whatever compensatory fashion they may employ, leading us, over time, to realize through repeated JTT evaluations that examinees' scores on the JTT were not reflecting the level of their qualitative declines in motor movements.…”

“…But the JTT allows examinees to execute its tasks in whatever compensatory fashion they may employ, leading us, over time, to realize through repeated JTT evaluations that examinees' scores on the JTT were not reflecting the level of their qualitative declines in motor movements. Maciel et al (2020) evaluated UL performance with the JTT over a period of one year and found that, six months after their initial evaluation, patients with DMD showed decreased performance on subtests 2, 3 and 5; and, 12 months after their initial evaluation, they showed a worsened subtest 4 performance. Of note, they maintained their performance on subtests 2, 3 and 5 during the period between six and 12 months after the initial evaluation, and their performances on subtests 1 and 6 were not different at these three later evaluations.…”

Responsiveness of Upper Limb Scales and Trunk Control for the Evolution of Patients With Duchenne Muscular Dystrophy

“…6,7 A study using the JTT showed that the test involves the recruitment and control of the proximal, medium, and distal levels. 25 Thus, compensatory movements were observed because muscle weakness impairs UL performance and restricts movements, which stimulates the selection of new motor strategies. 26,27 Functional motor loss in DMD usually occurs from proximal to distal, and a lack of proximal stability may impair the timed performance of the ULs.…”