Literature DB >> 25495774

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

Jenny Downs1, Helen Leonard, Peter Jacoby, Lauren Brisco, Gordon Baikie, Kylie Hill.   

Abstract

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. Implications for Rehabilitation Many girls and women with Rett syndrome are able to walk on their own or with assistance but with altered movement patterns. Validated measures of physical activity, such as step counts, have potential to monitor function during daily life. Compared with other forms of accelerometer-type devices, such as ActiGraph and ActivPAL, the StepWatch Activity Monitor (SAM) measured step counts with good accuracy and repeatability. The capacity of the SAM to measure physical activity in Rett syndrome allows focus on participation-based activities in clinical practice and clinical trials.

Entities:  

Keywords:  Outcome measure; Rett syndrome; physical activity; walking

Mesh:

Year:  2015        PMID: 25495774     DOI: 10.3109/09638288.2014.993436

Source DB:  PubMed          Journal:  Disabil Rehabil        ISSN: 0963-8288            Impact factor:   3.033


  6 in total

1.  A Psychometric Evaluation of the Motor-Behavioral Assessment Scale for Use as an Outcome Measure in Rett Syndrome Clinical Trials.

Authors:  Melissa Raspa; Carla M Bann; Angela Gwaltney; Timothy A Benke; Cary Fu; Daniel G Glaze; Richard Haas; Peter Heydemann; Mary Jones; Walter E Kaufmann; David Lieberman; Eric Marsh; Sarika Peters; Robin Ryther; Shannon Standridge; Steven A Skinner; Alan K Percy; Jeffrey L Neul
Journal:  Am J Intellect Dev Disabil       Date:  2020-11-01

2.  Quantification of walking-based physical activity and sedentary time in individuals with Rett syndrome.

Authors:  Jenny Downs; Helen Leonard; Kingsley Wong; Nikki Newton; Kylie Hill
Journal:  Dev Med Child Neurol       Date:  2017-02-06       Impact factor: 5.449

Review 3.  Clinical Trial Design for Disease-Modifying Therapies for Genetic Epilepsies.

Authors:  Dylan C Brock; Scott Demarest; Tim A Benke
Journal:  Neurotherapeutics       Date:  2021-09-30       Impact factor: 6.088

4.  Abnormal Foot Position and Standing and Walking Ability in Rett Syndrome: an Exploratory Study.

Authors:  Hanneke E Borst; Gillian S Townend; Mirjam van Eck; Eric Smeets; Mariëlle van den Berg; Aleid Laan; Leopold M G Curfs
Journal:  J Dev Phys Disabil       Date:  2018-01-02

5.  Implementing telehealth support to increase physical activity in girls and women with Rett syndrome-ActivRett: protocol for a waitlist randomised controlled trial.

Authors:  Jenny Downs; Meir Lotan; Cochavit Elefant; Helen Leonard; Kingsley Wong; Nicholas Buckley; Michelle Stahlhut
Journal:  BMJ Open       Date:  2020-12-29       Impact factor: 2.692

6.  Observational Gait Assessment Scales in Patients with Walking Disorders: Systematic Review.

Authors:  Carmen Ridao-Fernández; Elena Pinero-Pinto; Gema Chamorro-Moriana
Journal:  Biomed Res Int       Date:  2019-10-31       Impact factor: 3.411

  6 in total

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