Posture and gait abilities in patients with myotonic dystrophy (Steinert disease). Evaluation on the short-term of a rehabilitation program

Abstract

A rehabilitation program focused on strength, gait and balance allowed for significant improvements in some parameters of myotonic dystrophy. These results attest to the relevance of a short-term rehabilitation protocol for these patients in the framework of a multidisciplinary therapeutic care. The disparity observed in the results measured for these patients suggest the contribution of cognitive involvement in the limitations felt by patients with myotonic dystrophy in the areas of gait and balance.

Gait pattern in myotonic dystrophy (Steinert disease): A kinematic, kinetic and EMG evaluation using 3D gait analysis

“…Specific clinical features are highly characteristic muscle involvement with early bifacial weakness, bilateral mild ptosis, neck flexion weakness and pronounced distal weakness of finger flexors as well as ankle dorsiflexors with foot drop. As the disease progresses, the myotonia becomes accompanied by distal more proximal muscle weakness, more pronounced in the upper than in the lower extremities [9][10][11].…”

“…Wiles et al [12] investigated falls and risk factors in patients with DM1 using clinical assessments, lower limb muscle strength and spatio-temporal parameters during gait. Recently, Missaoui et al [11] evaluated the significant improvement in patients' balance capacities after a rehabilitation program focused on strength, gait and balance in 20 patients with DM1.…”

Évaluation de la capacité fonctionnelle chez des patients atteints de la dystrophie myotonique de type 1 /

“…Il faut cependant nuancer cette affirmation puisque plusieurs études dont l'entraînement n'était pas supervisé ont démontré peu ou aucune amélioration de l'endurance musculaire dans cette population (Lindeman et al, 1995;Voet, van der Kooi et al, 2010;. Étant donné l'atteinte systémique de la maladie et ses implications au niveau de l'autonomie physique et de la cognition, il est possible que cette clientèle ne soit pas apte à comprendre et à appliquer un programme d'entraînement spécifique et qu'elle nécessite non seulement une intervention avant et après, mais pendant toute la durée de l'étude (Johnson et al, 1995;Deater-Deckard & Mayr, 2005;Missaoui et al, 2010).…”

“…Chez des individus sains, l'entraînement des qualités musculaires donne des résultats mesurables très rapidement et il en est de même pour les personnes atteintes de DM1 Harper, 2001;Missaoui et al, 2010;Voet, van der Kooi et al,. 2010).…”

Análise das curvas de torque isocinético em movimentos do ombro

“…It quantifies the mentioned variables as well as the functional deficit through a large scale of angle velocities [5][6][7] . Among all these variables, the peak torque presents the highest representativeness in research and clinics 5 , in the evaluation of athletes 12 or different pathological conditions [13][14][15][16] . The graphic representation of an isokinetic curve exposes the behavior of the muscle during the articular activity and can be obtained in any movement degree such as torque, peak torque and other important variables, such as work and the antagonist/agonist ratio 1,7,8,17 .…”