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Beyond Contractures in Spinal Muscular Atrophy: Identifying Lower-Limb Joint Hypermobility.
Beyond Contractures in Spinal Muscular Atrophy: Identifying Lower-Limb Joint Hypermobility. Journal of clinical medicine Harding, E. R., Kanner, C. H., Pasternak, A., Glanzman, A. M., Dunaway Young, S., Rao, A. K., McDermott, M. P., Zolkipli-Cunningham, Z., Day, J. W., Finkel, R. S., Darras, B. T., De Vivo, D. C., Montes, J. 2024; 13 (9)Abstract
Background: The natural history of spinal muscular atrophy (SMA) is well understood, with progressive muscle weakness resulting in declines in function. The development of contractures is common and negatively impacts function. Clinically, joint hypermobility (JH) is observed but is poorly described, and its relationship with function is unknown. Methods: Lower-limb ROM (range of motion) assessments of extension and flexion at the hip, knee, and ankle were performed. ROMs exceeding the published norms were included in the analysis. The functional assessments performed included the six-minute walk test (6 MWT) and the Hammersmith Functional Motor Scale-Expanded (HFMSE). Results: Of the 143 participants, 86% (n = 123) had at least one ROM measure that was hypermobile, and 22% (n = 32) had three or more. The HFMSE scores were inversely correlated with hip extension JH (r = -0.60, p = 0.21; n = 6) and positively correlated with knee flexion JH (r = 0.24, p = 0.02, n = 89). There was a moderate, inverse relationship between the 6 MWT distance and ankle plantar flexion JH (r = -0.73, p = 0.002; n = 15). Conclusions: JH was identified in nearly all participants in at least one joint in this study. Hip extension, knee flexion and ankle plantar flexion JH was associated with function. A further understanding of the trajectory of lower-limb joint ROM is needed to improve future rehabilitation strategies.
View details for DOI 10.3390/jcm13092634
View details for PubMedID 38731167
View details for PubMedCentralID PMC11084694