Abstract
BackgroundChromosome 22q11.2 deletion syndrome (22q11.2DS), fragile X syndrome (FXS), and Turner syndrome (TS) are complex and variable developmental syndromes caused by different genetic abnormalities; yet, they share similar cognitive impairments in the domains of numbers, space, and time. The atypical development of foundational neural networks that underpin the attentional system is thought to result in further impairments in higher-order cognitive functions. The current study investigates whether children with similar higher-order cognitive impairments but different genetic disorders also show similar impairments in alerting, orienting, and executive control of attention.MethodsGirls with 22q11.2DS, FXS, or TS and typically developing (TD) girls, aged 7 to 15 years, completed an attention network test, a flanker task with alerting and orienting cues. Exploration of reaction times and accuracy allowed us to test for potential commonalities in attentional functioning in alerting, orienting, and executive control. Linear regression models were used to test whether the predictors of group and chronological age were able to predict differences in attention indices.ResultsGirls with 22q11.2DS, FXS, or TS demonstrated unimpaired function of the alerting system and impaired function of the executive control system. Diagnosis-specific impairments were found such that girls with FXS made more errors and had a reduced orienting index, while girls with 22q11.2DS showed specific age-related deficits in the executive control system.ConclusionsThese results suggest that the control but not the implementation of attention is selectively impaired in girls with 22q11.2DS, TS or FXS. Additionally, the age effect on executive control in girls with 22q11.2DS implies a possible altered developmental trajectory.
Highlights
Chromosome 22q11.2 deletion syndrome (22q11.2DS), fragile X syndrome (FXS), and Turner syndrome (TS) are complex and variable developmental syndromes caused by different genetic abnormalities; yet, they share similar cognitive impairments in the domains of numbers, space, and time
There was no significant difference in simple motor reaction time (SRT) between the four groups (F(3, 114) = 1.73, P = 0.16)
In the experiment reported in this paper, we found a consistent pattern of results that supports the view that girls with chromosome 22q11.2 deletion syndrome, fragile X syndrome, or 45,X Turner syndrome suffer from both common and diagnosis-specific impairments in visuospatial attention
Summary
Chromosome 22q11.2 deletion syndrome (22q11.2DS), fragile X syndrome (FXS), and Turner syndrome (TS) are complex and variable developmental syndromes caused by different genetic abnormalities; yet, they share similar cognitive impairments in the domains of numbers, space, and time. The current study investigates whether children with similar higher-order cognitive impairments but different genetic disorders show similar impairments in alerting, orienting, and executive control of attention. Behavioral, anatomical, and neuroimaging studies support the notion that the output of visual attention networks underpins a broad to continuous genetic influence, affords valuable insights into the typical development of the cognitive subsystems that underpin attention, and can provide clarity as to the specific nature of cognitive impairments in the respective NDDs [5]. Cross-syndrome comparisons provide insight into the neurobiological nature of cognitive skills by linking atypically developing behaviors to genetically modulated cellular and anatomical changes. Direct behavioral cross-syndrome comparisons have been limited to a single three-way combination of FXS, WS, and Down’s syndrome [9] and a small number of two-way combinations [10,11,12,13,14,15,16]
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