Syndrome-Specific Attention Profiles in Infants with Fragile X syndrome and Down Syndrome

Malorie Webb, Public Health; Elizabeth Will, Ph.D., Psychology

Background
From as early as 4 months, typically developing (TD) infants explore objects with their eyes, mouths, and hands
(Rochat, 1989). This exploration requires substantial fine and gross motor skills and helps to develop an infant’s
understanding of their environment (Kaur, Srinivasan, Bhat, 2015). In children with a diagnosis of fragile X
syndrome (FXS) or Down syndrome (DS), object attention is often impaired as a result of dysregulated
attention, hypotonia (i.e., low muscle tone), and impaired motor development (Hagerman et al., 2017; Iverson.
2010). FXS and DS children often present with reduced or atypical object exploration, such as becoming hyper
focused on an object or on social stimuli (Kaur et al., 2015; Lansink, 1997). This is referred to as “sticky”
attention, in other words the child has difficulty disengaging from one object in order to move on to the next,
which constrains information gathered from the environment (Fischer et al. 2016). The same is true if a child
becomes “stuck” on social stimuli and misses object-learning opportunities. Regulation of visual attention is
essential to learning about the environment, and children with a neurodevelopmental disorder associated with
impaired attentional control experience decreased exploration and social interactions (Sacrey, Bryson,
Zwaigenbaum, 2013; Kaufmann et al., 2017). These missed learning opportunities may lead to compromised
cognitive and developmental outcomes that are unique across each disorder. This project aims to identify early
markers of delayed development and how they differ between groups so future researchers can develop
intervention strategies.

Research Question
Are there group differences in object and social attention between children with DS or FXS compared to
typically developing children?

Project Goals and Objectives

The goal of this project is to look at the underlying mechanisms of developmental differences in children with
FXS or DS relative to typically developing children. To achieve this goal, the project objectives are:
• Code collected data from the keys task using Noldus XT software. Videos will be coded for key
variables of interest including duration of object and social attention;
• Work with a mentor to analyze group differences across key variables of interest;
• Contribute to mentor’s ongoing research project through data reduction, analysis, and dissemination
efforts;
• Disseminate findings in research papers and Discover USC.

Project Significance
Certain patterns of object and social attention, such as “sticky” attention, may serve as indicators of attentional
difficulties and potentially other co-occurring psychopathology, such as autism. Early exploration is an essential
foundation for cognitive development in which an infant learns to interact with and understand their
environment, as well as learn to interact with social partners. Being able to recognize these indicators is a
crucial first step for researchers in developing and implementing strategies to promote optimal learning and
social communication. Thus, this research will help to identify potential areas for targeted and early
intervention.

Project Design
The Neurodevelopmental Disorders (NDD) Lab is conducting a prospective longitudinal study with infants
diagnosed with DS or FXS to increase understanding of how these disorders present at an early age. All
research staff has undergone CITI training for human research and the larger project has been approved by the
USC IRB for human subject research (Pro00001966). Participants were seen at 6, 9, 12, and 24-months old, and
are now in the process of completing 36, 48, and 60-month follow ups. The proposed project will utilize
collected data from 12-month old infants with FXS (n=27) and make a novel contribution through coding new
DS infants (n=30) for social attention. Coding is completed as part of the proposed project, and with these
additional efforts, the data will be sufficient for analysis and dissemination.

Object and social attention is indexed using a 3-minute exploration task, in which infants are given a set of baby
keys. Using Noldus XT software, the keys task is coded for duration of visual object attention (i.e. to the keys)
and duration of visual social attention (i.e. to examiner or parent). The primary mentor will conduct primary
analyses, with preliminary analyses (participant characteristics and correlations) conducted by Miss Webb as
part of the Magellan project.

Project Timeline
Project Months
Task Description

April

June
May
Aug

Nov
Dec

July
Mar
Sep

Feb
Oct

Jan
Coding data with Noldus XT X X X X X

Analysis of group differences X X X

Preparation for presentation at Discover USC X

Prepare publication X X X X

Anticipated Results and Dissemination

We expect our research will show that both groups, DS and FXS, will differ from the TD groups. In regard to
social attention, we hypothesize that children diagnosed with DS will show significantly higher rates of social
attention than TD kids. We also anticipate that the FXS group will show significantly lower rates of social
attention than both DS and TD groups. We expect the inverse results for object attention, with children with
FXS coding highest followed by the TD group, and then the DS group.

I plan to present this research at Discover USC in April 2020, as well as contribute to my mentor’s research
paper to be submitted for publication by July 2020. This research will also be used in future projects in the
Neurodevelopmental Disorders lab.

Personal Statement
I have been working in the Neurodevelopmental Disorders lab since my freshman year. I originally joined in
order to gain research experience with neurodevelopmental disorders for my future goals of attending medical
school. Through my experience so far, I have gained a better understanding of different neurodevelopmental
disorders and the unique needs across each. I have also come to recognize the importance of research in
developing treatment and I plan to stay with the lab for the remainder of my undergraduate career. I am excited
for the opportunity to work with Dr. Will on her research papers and to present at conferences. As I get more
involved with this research, I have developed a strong interest in working with this community. I plan to use
this research for Graduation with Leadership Distinction as well as for my honors thesis. I want to look at how
people diagnosed with a neurodevelopmental disorder are treated in the medical community, as well as
investigate what kind of training physicians receive in order to work with patients on the autism spectrum. I
want to be a pediatrician, so this research is an especially important experience for me as I work with these
children and their families.
 References

Fischer J., Smith H., Martinez-Pedraza F., Carter A.S., Kanwisher N., Kaldy Z. (2016). Unimpaired attentional
disengagement in toddlers with autism spectrum disorder. Developmental Science, 19(6), 1095-1103.

Hagerman R.J., Berry-Kravis E., Hazlett H.C., Bailey D.B., Moine H., Kooy R. F., … Hagerman P.J. (2017).
Fragile X syndrome. Nature Reviews Disease Primers, 3(17065). doi 10.1038/nrdp.2017.65

Iverson J. (2010) Developing language in a developing body: the relationship between motor development and
language development. Journal of Child Language 37, 229–261.

Kaufmann W.E., Kidd S.A., Andrews H.F., Budimirovic D.B., Esler A., Haas-Givler B., … Berry-Kravis E.
(2017). Autism spectrum disorder in fragile X syndrome: Cooccurring condition and current treatment.
Pediatrics, 139(3), 194-206.

Kaur M., Srinivasan S.M., Bhat A.N. (2015) Atypical object exploration in infants at-risk for autism during the
first year of life. Frontiers in Psychology, 6(798), 1-15.

Lansink J.M., Richards J.E., (1997). Heart rate and behavioral measures of attention in six-, nine-, and twelve-
month old infants during object exploration. Child Development, 68(4), 610-620.

Rochat P. (1989) Object manipulation and exploration in 2- to 5-month-old infants. Developmental Psychology,
25, 871-884.

Sacrey L.R., Bryson S.E., Zwaigenbaum L, (2013). Prospective examination of visual attention during play in
infants at high-risk for autism spectrum disorder: a longitudinal study from 6 to 36 months of age.
Behavioural Brain Research, 256, 441-450.