Scoliosis in Rett Syndrome: Progression, Comorbidities, and Predictors.

ABSTRACT:

Background

Scoliosis is prominent in Rett syndrome (RTT). Following the prior report from the US Natural History Study, the onset and progression of severe scoliosis (≥40° Cobb angle) and surgery were examined regarding functional capabilities and specific genotypes, addressing the hypothesis that abnormal muscle tone, poor oral feeding, puberty, and delays or absence of sitting balance and ambulation may be responsible for greater risk in RTT.

Methods

The multicenter RTT Natural History Study gathered longitudinal data for classic RTT, including mutation type, scoliosis, muscle tone, sitting, ambulation, hand function, and feeding. Cox regression models were used to examine the association between scoliosis and functional characteristics. All analyses utilized SAS 9.4; two-

SUBMITTER: Killian JT 

PROVIDER: S-EPMC5461984 | biostudies-literature | 2017 May

REPOSITORIES: biostudies-literature