Boys with fragile X syndrome: investigating temperament in early childhood

Abstract

Attention difficulties commonly found in adolescents and adults with FXS appear to also be characteristic of young boys with FXS, as reflected by lower effortful control. Age-related findings concerning negative affectivity may be particularly significant, leading to improved intervention/preventative efforts.

Infant Temperament in the FMR1 Premutation and Fragile X Syndrome

“…In the surgency domain, infants with FXS were generally rated as less active, less likely to seek novel environmental situations, less vocally reactive, and reduced in indices of positive affect such as smiling and laughter. In the negative affect domain, we replicated previous studies suggesting negative affect is reduced low in early childhood, despite being elevated in school-aged children (Low Kapalu & Gartstein, 2016;Shanahan et al, 2008), with particularly low distress to limitations in our sample. Notably, despite lower overall levels of negative affect in young children with FXS relative to controls, previous within-group studies have reported that higher negative affect is prospectively associated with anxiety in later childhood (Tonnsen et al, 2013).…”

“…First, infants with the FXpm could exhibit similar temperament profiles to those observed in older children with FXS, reflecting shared genetic vulnerabilities and qualitatively similar yet less frequent comorbid conditions in the FXpm relative to FXS (Bailey et al, 2008). From this perspective, we would expect infants with the FXpm to exhibit reduced surgency, negative affect, and regulatory capacity, paralleling previous work in older samples with FXS (Low Kapalu & Gartstein, 2016;Shanahan et al, 2008). This outcome would suggest that infants with the FXpm may benefit from similar surveillance and intervention approaches as FXS.…”

“…This trend toward suppressed negative affect in the FXpmparticularly in the domains of sadness and distress in response to environmental limitationsdeviates from the general population literature, whereby problem behaviors in later development are associated with enhanced negative affect in early childhood (Gartstein et al, 2010). Instead, this trend is similar to the non-linear patterns of reactivity associated with maladaptive outcomes in FXS, whereby negative affect is atypically low in early childhood but elevated in school-aged children (Low Kapalu & Gartstein, 2016;Shanahan et al, 2008). Notably, this shift between suppressed and elevated reactivity in FXS is most pronounced in children with higher rates of problem behaviors; for example, children with FXS with elevated autism features have been shown to display enhanced shifts between hypo-and hyper-responsiveness, as measured by heart activity during a toy play task (Roberts, Tonnsen, Robinson, & Shinkareva, 2012).…”

“…Although no studies to date have reported temperament features in infants with the FXpm, the temperament profile in older children with FXS is relatively well-characterized. Studies using the three-factor psychobiological model of temperament employed in the present study (Rothbart & Derryberry, 1981) have reported lower surgency and regulatory capacity in FXS relative to non-FXS controls (Low Kapalu & Gartstein, 2016) and negative affect that is low in early childhood but increases with age (Low Kapalu & Gartstein, 2016;Shanahan, Roberts, Hatton, Reznick, & Goldsmith, 2008). Using other theoretical models of temperament, children with FXS have been reported to display elevated activity and lower adaptability, intensity, persistence, and approach (Bailey, Hatton, Mesibov, Ament, & Skinner, 2000;Hatton, Bailey, Hargett-Beck, Skinner, & Clark, 1999;Roberts, Boccia, Hatton, Skinner, & Sideris, 2006).…”

Frontal electroencephalogram (EEG) asymmetry reactivity: Exploring changes from baseline to still face procedure response

“…Mothers with infants 6–12 months of age were recruited via social media (i.e., Facebook) advertisements, local birth centers/parent–infant programs, and pamphlets distributed in locations frequented by families with infants (e.g., pediatricians, local mall, farmers market) in two Inland Northwest communities. Children with significant medical or birth complications, including infants born preterm (<37 weeks of gestation) and/or identified as developmentally delayed/disabled, were excluded, as these conditions can be expected to result in altered patterns of temperament development (e.g., Gartstein, Marmion, & Swanson, 2006; LowKapalu & Gartstein, 2016), and children experiencing such difficulties are generally not recruited for temperament-related investigations (e.g., Gartstein & Rothbart, 2003; Gartstein et al, 2018). Non-English-speaking caregivers were also excluded, as instruments utilized for the project in addition to the IBQ-R are not available in languages other than English.…”