Medical Issues in Adults with Rett Syndrome – A National Survey. (17th February 2020)

• Record Type: Journal Article
• Authors: Henriksen, Mari Wold; Breck, Hilde; von Tetzchner, Stephen; Paus, Benedicte; Skjeldal, Ola H.
• Is Part Of: Developmental neurorehabilitation. Volume 23:Number 2(2020)
• Page Start: 106
• Page End: 112
• Publication Date: 2020-02-17
• Subjects: Rett syndrome -- ageing -- adulthood -- morbidity -- clinical management; Children with disabilities -- Rehabilitation -- Periodicals; Developmentally disabled children -- Rehabilitation -- Periodicals; 615.542
• DOI: 10.1080/17518423.2019.1646341
• Languages: English
• ISSNs: 1751-8423

Abstract

ABSTRACT: Objectives : To examine main health issues in a population of females with Rett syndrome, with a focus on individuals aged 36 or older. Methods : A national survey including 85 females, divided into a younger (1–20 years), a middle (21–35 years) and an older group (36–66 years). Data include clinical examination, medical records and parental interviews. Prevalences of six main medical issues (scoliosis, ambulation, growth, respiration, gastrointestinal dysmobility and epilepsy) and severity scores in the three groups were compared. Results : Mean severity scores were 11.8, 15.1 and 13.7 (from younger to older), and the difference between the younger and the middle group was significant. No other major significant prevalence differences were observed. Conclusions : Most main medical issues in Rett syndrome continued to be a major concern in adulthood, but health did not seem to decline with increasing age. The results emphasize the need for clinical follow-up throughout adulthood.