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Journal of NeuroEngineering and Rehabilitation
Published in: Journal of NeuroEngineering and Rehabilitation 1/2019

Open Access 01-12-2019 | Research

Complexity based measures of postural stability provide novel evidence of functional decline in fragile X premutation carriers

Authors: Clodagh O’Keeffe, Laura P. Taboada, Niamh Feerick, Louise Gallagher, Timothy Lynch, Richard B. Reilly

Published in: Journal of NeuroEngineering and Rehabilitation | Issue 1/2019

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Abstract

Background

Fragile X Associated Tremor/Ataxia Syndrome (FXTAS) is a neurodegenerative movement disorder characterized by tremor, ataxic gait, and balance issues resulting from a premutation of the Fragile X Mental Retardation 1 (FMR1) gene. No biomarkers have yet been identified to allow early diagnosis of FXTAS, however, recent studies have reported subtle issues in the stability of younger premutation carriers, before disease onset. This study investigates the efficacy of multiscale entropy analysis (MSE) in detecting early changes in the motor system of premutation carriers without FXTAS.

Methods

Sway complexity of 12 female Premutation carriers and 15 healthy Controls were measured under four conditions: eyes open, closed, and two dual-task conditions. A Sustained Attention Response Task (SART) and a working memory based N-Back task were employed to increase cognitive load while standing on the forceplate. A Complexity Index (Ci) was calculated for anterior-posterior (AP) and mediolateral (ML) sway. Independent t-tests were used to assess between-group differences and Oneway repeated measures ANOVA were used to assess within group differences with Bonferroni corrections to adjust for multiple comparisons.

Results

Group performances were comparable with eyes open and closed conditions. The Carrier group’s Ci was consistent across tasks and conditions while the Control group’s AP Ci increased significantly during the cognitive dual-task (p = 0.001). There was also a strong correlation between CGG repeat length and complexity for the Carrier group (p = 0.004).

Significance

Increased sway complexity is believed to stem from reallocation of attention to facilitate the increased cognitive demands of dual-tasks. Carriers’ complexity did not change during dual-tasks, possibly indicating capacity interference and inefficient division of attention. Lower sway complexity in carriers suggests diminished adaptive capacity under stress as well as degradation of motor functioning. Therefore, sway complexity may be a useful tool in identifying early functional decline in FMR1 premutation carriers as well as monitoring progression towards disease onset.
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Metadata
Title
Complexity based measures of postural stability provide novel evidence of functional decline in fragile X premutation carriers
Authors
Clodagh O’Keeffe
Laura P. Taboada
Niamh Feerick
Louise Gallagher
Timothy Lynch
Richard B. Reilly
Publication date
01-12-2019
Publisher
BioMed Central
Published in
Journal of NeuroEngineering and Rehabilitation / Issue 1/2019
Electronic ISSN: 1743-0003
DOI
https://doi.org/10.1186/s12984-019-0560-6

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