Building the repertoire of measures of walking in Rett syndrome
children; Rehabilitation; Rett syndrome; girls; walking; cerebral-palsy; test-retest reliability; adults; 2-minute; 6-minute; disability inventory; functional mobility scale; pediatric evaluation; two-minute walk test; tone and motor problems; tool development; scale development; Modified two-minute walk test; Rett syndrome specific functional mobility scale; RSGMS
Background: The repertoire of measures of walking in Rett syndrome is limited. This study aimed to determine measurement properties of a modified two-minute walk test (2MWT) and a modified Rett syndrome-specific functional mobility scale (FMS-RS) in Rett syndrome. Methods: Forty-two girls and women with Rett syndrome (median 18.4 years, range 2.4-60.9 years) were assessed for clinical severity, gross motor skills, and mobility. To measure walking capacity, 27 of this group completed a 2MWT twice on two different assessment days. To assess walking performance, the FMS-RS was administered to the total sample of parents (n=42) on two occasions approximately one week apart. Results: There were negative correlations between clinical severity and 2MWT (r=-0.48) and FMS-RS (r=-0.60-0.66). There were positive correlations between gross motor skills and mobility and 2MWT (r=0.51, 0.43) and FMS-RS (r=0.71-0.93, 0.74-0.94), respectively. Test-retest reliability for the 2MWT was good with high intraday and interday correlations (ICC=0.86-0.98). For the 2MWT, the standard error of measurement was 13.8 m and we would be 95% confident that changes greater than 38 m would be greater than within subject error. There was good test-retest reliability for all three distances on the FMS-RS (ICC=0.94-0.99). Conclusions: Walking capacity as measured by the 2MWT showed expected but limited relationships with measures of different constructs, providing some support for concurrent validity. Walking performance as measured with the FMS-RS was more strongly consistent with other clinical measures supporting its concurrent validity. Test-retest reliability was good for both the FMS-RS and the 2MWT. Therefore, these measures have the potential to be used in clinical practice and research.
Stahlhut M; Downs J; Leonard H; Bisgaard A M; Nordmark E
Disability and Rehabilitation
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1080/09638288.2016.1212280" target="_blank" rel="noreferrer noopener">10.1080/09638288.2016.1212280</a>
Validating the Rett Syndrome Gross Motor Scale
tone and motor problems; Rett syndrome; tool development; scale development; RSGMS
© 2016 Downs et al. Rett syndrome is a pervasive neurodevelopmental disorder associated with a pathogenic mutation on the MECP2 gene. Impaired movement is a fundamental component and the Rett Syndrome Gross Motor Scale was developed to measure gross motor abilities in this population. The current study investigated the validity and reliability of the Rett Syndrome Gross Motor Scale. Video data showing gross motor abilities supplemented with parent report data was collected for 255 girls and women registered with the Australian Rett Syndrome Database, and the factor structure and relationships between motor scores, age and genotype were investigated. Clinical assessment scores for 38 girls and women with Rett syndrome who attended the Danish Center for Rett Syndrome were used to assess consistency of measurement. Principal components analysis enabled the calculation of three factor scores: Sitting, Standing and Walking, and Challenge. Motor scores were poorer with increasing age and those with the p.Arg133Cys, p.Arg294* or p.Arg306Cys mutation achieved higher scores than those with a large deletion. The repeatability of clinical assessment was excellent (intraclass correlation coefficient for total score 0.99, 95% CI 0.930.98). The standard error of measurement for the total score was 2 points and we would be 95% confident that a change 4 points in the 45-point scale would be greater than within-subject measurement error. The Rett Syndrome Gross Motor Scale could be an appropriate measure of gross motor skills in clinical practice and clinical trials.
Downs J; Stahlhut M; Wong K; Syhler B; Bisgaard A; Jacoby P; Leonard H
PLoS ONE
2016
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1371/journal.pone.0147555" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0147555</a>