The relationship of cumulative motor asymmetries to scoliosis in Rett syndrome.

Abstract

Interrelationships between Rett syndrome scoliosis and symmetric and asymmetric motor pull, ambulation, and advancement of age were investigated in order to provide a treatment rationale for slowing the progression of scoliosis. Questionnaires (N = 262) completed by International Rett Syndrome Association families were analyzed with logistic regression, odds ratio, Kruskal-Wallis one-way, and Fisher's exact test analyses. Rett syndrome scoliosis was found to be significantly related to orthopedic risk factors of asymmetric movements and positions (odds ratio = 4.5, p < .001). The odds ratio for asymmetric upper-body positioning (4.4), nonambulation (3.1), and age advancement (14.1) indicated that each was a significant predictor of scoliosis by logistic regression analysis. Univariate Kruskal-Wallis one-way analysis identified a significant relationship between scoliosis and asymmetric higher shoulder positioning (p < .001) and bodyside movements (p = .03). Hemisyndrome aspects of Rett syndrome were identified as the increased prevalence of symptoms in either right or left bodyside. A significant relationship was found between the prevalence of Rett syndrome scoliosis and orthopedic risk factors. These findings suggest a treatment approach for Rett syndrome scoliosis that focuses on balancing bilateral muscular pull.