Rett syndrome affects girls and is characterized by progressive motor disablement, loss of acquired skills, seizures and autism. Scoliosis is also a commonly seen as a complication and this study set out to describe its prevalence, classify spinal deformity, and evaluate associations between disability and scoliosis.
29 patients took part in the study, ages ranging from 4 to 33.
The study found that nearly 90% of those who took part had radiographically verified scoliosis, with the median curve being 41° They found that function was poorer in patients with C-shaped curve
Using the Barthel index (10 items measuring daily functioning for activities of daily living and mobility) the function level in females with Rett syndrome showed significant correlation to scoliosis curve type
Spinal deformity and disability in patients with Rett syndrome, Riise R et al, in Developmental Medicine & Child Neurology, 53: 653–657.