Abstract
Background
We sought to examine fundamental aspects of attention in children with Rett syndrome,
a severely disabling neurodevelopmental disorder caused by spontaneous mutations in
the X-linked MECP2 gene. To gauge their attention, we used eye tracking, which bypasses
the profound impairments in expressive language and hand use in Rett syndrome. We
report two aspects of attention—shifting and sustaining—basic abilities known to drive
cognitive growth.
Methods
Two groups were compared: those with Rett syndrome (N = 20; 3-15 years) and a typically
developing comparison group (N = 14; 3-16 years), using a task in which an attractive
central stimulus was followed, after a short gap, by a dynamic target presented to
one side. Time to shift to the target location (reactive and anticipatory saccades)
and time fixating the target were assessed.
Results
Children with Rett syndrome were consistently slower to shift (largely because of
fewer anticipations); their reactive saccades were also slower than those of typically
developing children, but not significantly so. The Rett syndrome group spent considerable
time looking at the target (over 75% of available time), although significantly less
so than the typically developing group.
Conclusions
These findings indicate that children with Rett syndrome could maintain attention
on a stimulus and orient relatively quickly to the appearance of a target in the visual
field. However, they had difficulty in anticipating predictable events, a difficulty
in endogenous attention that is likely to have deleterious implications for executive
functioning.
Keywords
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Article info
Publication history
Published online: January 20, 2016
Accepted:
January 12,
2016
Received:
August 21,
2015
Footnotes
Drs. Rose and Djukic share first authorship of this article.
Disclosure: None of the authors had any financial interests or benefit arising from this work.
Identification
Copyright
© 2016 Elsevier Inc. Published by Elsevier Inc. All rights reserved.