Scoliosis in girls with Rett syndrome impairs functioning


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.

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John Northfield

After qualifying as a social worker, John worked in community learning disability teams before getting involved in a number of long-stay hospital closure programmes, working to develop individual plans for people moving into their own homes. He worked for BILD, helping to develop the Quality Network and was editorial lead for the NHS electronic library learning disabilities specialist collection. This led him to found the Learning Disabilities Elf site with Andre Tomlin as a way of making the evidence accessible to practitioners in health and social care. Most recently he has worked as part of Mencap's national quality team and also been involved in a number of national website developments, including the General Medical Council's learning disabilities site.

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