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Dev Med Child Neurol. 2013 Apr;55(4):364-71. doi: 10.1111/dmcn.12085.

Rett syndrome: an eye-tracking study of attention and recognition memory.

Author information

1
Department of Pediatrics, Kennedy Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, NY 10461, USA. susan.rose@einstein.yu.edu

Abstract

AIM:

The aim of this study was to examine attention and recognition memory for faces and patterns in Rett syndrome, a severely disabling neurodevelopmental disorder caused by mutations in the X-linked MECP2 gene.

METHOD:

Because Rett syndrome impairs speech and hand use, precluding most neuropsychological testing, the visual paired-comparison paradigm (VPC) was used, together with eye tracking. In the VPC, two identical stimuli are presented for familiarization. On test, the familiar stimulus and a new one are paired, and recognition inferred from preferential looking to the novel target. Attention is measured by looking time, gaze dispersion, and number/length of fixations. Twenty-seven female patients with Rett syndrome (mean age 10y 6mo; SD 6y 8mo, age range 2-22y) from the Rett clinic at a children's hospital were assessed in this study, along with 30 age- and sex-matched typically developing participants (outpatients from the same hospital).

RESULTS:

Although patients with Rett syndrome showed recognition of both faces and patterns, with novelty scores greater than chance (50%), their performance was significantly poorer than that of the typically developing comparison group. Their attention to both was less mature and marked by a more narrowly focused gaze, with fewer and longer fixations. When inspecting faces, attention to the eyes was similar in both groups; however, patients with Rett syndrome tended to ignore the nose and mouth.

INTERPRETATION:

This is one of the first studies to characterize attention and memory in individuals with Rett syndrome. Visually based techniques, such as the VPC, open a new avenue for quantifying the cognitive phenotype associated with this syndrome.

PMID:
23488948
DOI:
10.1111/dmcn.12085
[Indexed for MEDLINE]
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