Document Detail


Spinal deformity and disability in patients with Rett syndrome.
MedLine Citation:
PMID:  21501153     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Aim  Rett syndrome is a neurodevelopmental disorder starting in early childhood with devastating consequences both on the brain and on systemic neurons. Scoliosis is a common complication in most of these patients. The aim of the study was to describe the prevalence of scoliosis, classify spinal deformity, and evaluate the association between disability and scoliosis in patients with Rett syndrome. Method  Twenty-nine female patients, mean age 14 years 8 months (range 4y 10mo-33y) were included. Outcome parameters were the Barthel Index for evaluation of function, pain, analgesics, and radiological evaluation of the spine including curve size (Cobb's method) and curve type. None of the patients had surgery before assessment. Results  Twenty-five (87%) patients had radiographically verified scoliosis. The median curve was 41° (range 15-77). The curve was classified as C-shaped (neuromuscular) in 16 patients and double curve-shaped in nine. Function was poorer in patients with C-shaped curve (p<0.01). Walking on a level surface (r=0.9) and the ability to ascend or descend stairs (r=0.8) were highly correlated with the Barthel Index. Curve size was moderately correlated with function (r=0.5). Interpretation  Function level in females with Rett syndrome measured according to the Barthel Index showed significant correlation to scoliosis curve type. A low score was associated with a C-shaped curve involving all of the spine and pelvis.
Authors:
Rolf Riise; Jens Ivar Brox; Roger Sorensen; Ola H Skjeldal
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-18
Journal Detail:
Title:  Developmental medicine and child neurology     Volume:  -     ISSN:  1469-8749     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-4-19     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0006761     Medline TA:  Dev Med Child Neurol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© The Authors. Developmental Medicine & Child Neurology © 2011 Mac Keith Press.
Affiliation:
 Department of Orthopedic Surgery, Oslo University Hospital, Rikshospitalet, Oslo.  Section of Neurology, Department of Pediatrics, Oslo University Hospital, Rikshospitalet, Oslo.  Department of Research, Innlandet Hospital Thrust, Brumunddal, Norway.
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