A comparison of gait in spinal muscular atrophy, type II and Duchenne muscular dystrophy

Armand, Stéphane; Mercier, M.; Watelain, Éric; Patte, Karine; Pélissier, J.; Rivier, François

doi:10.1016/j.gaitpost.2004.04.006

Cited by 60 publications

(24 citation statements)

References 22 publications

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“…7,43 Furthermore, PF strength has been implicated as a key muscle in maintaining gait in boys with DMD. 44 Interestingly, we did not observe any relationships between DF muscle strength and functional ability, although a previous study 45 found that ankle DF muscle strength of less than grade 4 was a key prognostic factor in determining time to wheelchair dependence in boys with DMD. Because all subjects in our study were ambulatory, DF torque may not have played a role in their current walking ability.…”

Section: Discussioncontrasting

confidence: 69%

Age-Related Differences in Lower-Limb Muscle Cross-Sectional Area and Torque Production in Boys With Duchenne Muscular Dystrophy

Mathur

Lott

Senesac

et al. 2010

Archives of Physical Medicine and Rehabilitation

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Objective To examine the relationship between lower-extremity muscle cross-sectional area, muscle strength, specific torque, and age in ambulatory boys with Duchenne muscular dystrophy (DMD) compared with controls. Design Observational cross-sectional study. Setting University research setting. Participants Volunteer sample of boys with DMD (n = 22) and healthy control boys (n = 10), ages 5 through 14 years. Interventions Not applicable. Main Outcome Measures Maximal muscle cross-sectional area (CSAmax) assessed by magnetic resonance imaging of quadriceps, plantarflexors (PFs) and dorsiflexors (DFs), peak isometric torque from dynamometry, and timed functional tests. Results The average CSAmax of the triceps surae muscle group was approximately 60% higher in boys with DMD compared with controls (39.1 ± 13.6 cm2 vs 24.5±9.3 cm2; P = .002), while the tibialis anterior muscle showed age-appropriate increases in CSAmax. The increase in quadriceps CSAmax was also distinctly different in boys with DMD compared with controls. Specific torque (ie, peak torque/CSAmax) was impaired in all 3 muscles groups, with the knee extensor (KE) and PF muscles showing 4-fold, and the DF muscles 2-fold, higher values in controls compared with boys with DMD. Large age-related gains in specific torque were observed in all 3 muscle groups of control subjects, which were absent in ambulatory boys with DMD. Correlations were observed between performance on functional tasks and quadriceps and PF torque production (r= −.45 to − 57, P < .05), but not with DF strength. Conclusions Age-related changes in muscle cross-sectional area and specific torque production in lower-extremity muscles showed distinctly different patterns in the KE, PF, and DF muscles of boys with DMD compared with controls.

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Section: Discussioncontrasting

confidence: 69%

Age-Related Differences in Lower-Limb Muscle Cross-Sectional Area and Torque Production in Boys With Duchenne Muscular Dystrophy

Mathur

Lott

Senesac

et al. 2010

Archives of Physical Medicine and Rehabilitation

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“…In DMD patients, the lack of knee flexion during weight acceptance phase of the gait cycle is generally attributed to muscle weakness [22–24]. Because GRMD dogs are weaker than normal controls [25], we speculate that affected dogs make adaptations analogous to gait adaptations observed in DMD patients.…”

Section: Discussionmentioning

confidence: 93%

Kinematics of gait in Golden Retriever Muscular Dystrophy

Marsh

Eggebeen

Kornegay

et al. 2010

Neuromuscular Disorders

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The goal of this study was to quantify the two-dimensional kinematics of pathologic gait during overground walking at a self-selected speed at the stifle (knee) and hock (ankle) joints in six Golden Retriever Muscular Dystrophy (GRMD) dogs and six carrier littermates (controls). We found that GRMD dogs walked significantly slower than controls (p < 0.01). At the stifle joint, both groups displayed similar ROM (range of motion), but compared to controls, GRMD dogs walked with the stifle joint relatively more extended. At the hock joint, GRMD dogs displayed less ROM (range of motion) and walked with the joint relatively less flexed compared to controls. We controlled for gait speed in all analyses, so the differences we observed in joint kinematics between groups cannot be attributed solely to the slower walking speed of the GRMD dogs. This is the first kinematic study of gait in the GRMD dog, an important step in using this model in pre-clinical trials.

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“…Leg muscle weakness is most severe in the hip adductors, hip flexors, and quadriceps, and less severe in hamstrings and other pelvic girdle muscles 16. Although the distal musculature is relatively spared, weakness of the gastrocnemius muscle has been reported among ambulatory patients with SMA, with a resultant impact on force production at the ankle and step length 17. The relatively uniform pattern of involvement in SMA is reflected in our observations of fatigue‐related changes in performance during the 6MWT.…”

Section: Discussionmentioning

confidence: 99%