Rett syndrome: establishing a novel outcome measure for walking activity in an era of clinical trials for rare disorders

Title

Rett syndrome: establishing a novel outcome measure for walking activity in an era of clinical trials for rare disorders

Creator

Downs J; Leonard H; Jacoby P; Brisco L; Baikie G; Hill K

Publisher

Disability and Rehabilitation

Date

2015

Subject

Rehabilitation; validity; injury; Rett syndrome; walking; disease; sample; accelerometry; accuracy; activity monitors; Outcome measure; physical activity; physical-activity; step; test-retest reliability; tone and motor problems; tool development; scale development; StepWatch

Description

Background: Rett syndrome is a pervasive neurological disorder with impaired gait as one criterion. This study investigated the capacity of three accelerometer-type devices to measure walking activity in Rett syndrome. Methods: Twenty-six participants (mean 18 years, SD 8) wore an Actigraph, ActivPAL and StepWatch Activity Monitor (SAM) during a video-taped session of activities. Agreement was determined between step-counts derived from each accelerometer and observation. Repeatability of SAM-derived step counts was determined using pairs of one-minute epochs during which the same participant was observed to walk with the same cadence. Results: The mean difference (limit of agreement) for the Actigraph, ActivPAL and SAM were -41 (SD 33), -16 (SD 21) and -1 (SD 16) steps/min, respectively. Agreement was influenced by a device/cadence interaction (p < 0.001) with greater under-recording at higher cadences. For SAM data, repeatability of step-count pairs was excellent (intraclass correlation coefficient 0.91, 95% CI 0.79-0.96). The standard error of measurement was 6 steps/min and we would be 95% confident that a change >= 17 steps/min would be greater than within-subject measurement error. Conclusions: The capacity of the SAM to measure physical activity in Rett syndrome allows focus on participation-based activities in clinical practice and clinical trials.

Rights

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).

Citation

Downs J; Leonard H; Jacoby P; Brisco L; Baikie G; Hill K, “Rett syndrome: establishing a novel outcome measure for walking activity in an era of clinical trials for rare disorders,” Pediatric Palliative Care Library, accessed September 24, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16635.

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