Boys with fragile X syndrome: investigating temperament in early childhood.
Attention and self-control gaps in fragile X syndrome are measurable before kindergarten.
01Research in Context
What this study did
The team compared 50 boys with fragile X syndrome to 50 boys without the condition. All kids were 3 to 7 years old. Parents filled out a short checklist about each boy’s daily behavior.
What they found
Boys with FXS had weaker effortful control. They showed more shyness and constant motion. Negative mood rose with age in the FXS group only.
How this fits with other research
Wynne et al. (1988) first noticed hyperactivity in every FXS boy they saw. Eussen et al. (2016) now prove these traits show up before school starts.
Laugeson et al. (2014) found older FXS kids read faces poorly and avoid people. The new data link the same social caution to preschool shyness.
Leezenbaum et al. (2019) showed preschoolers with ASD also fail delay tasks. Both studies point to early self-control problems across neurodevelopmental disorders.
Why it matters
You can spot poor regulation in FXS boys as young as three. Use brief wait games and visual timers to build effortful control early. Share the checklist with parents so they see the same targets at home.
Get CEUs on This Topic — Free
The ABA Clubhouse has 60+ on-demand CEUs including ethics, supervision, and clinical topics like this one. Plus a new live CEU every Wednesday.
Start each session with a 10-second wait game and praise still hands.
02At a glance
03Original abstract
BACKGROUND: Fragile X syndrome (FXS) is an x-linked genetic disorder that represents the most common hereditary cause of Intellectual Disability (ID). Very specific behavioural features (e.g. attention deficit hyperactivity disorder and stereotyped behaviour) are associated with FXS in adolescents and adults, yet research on temperament and behavioural characteristics in young children with FXS has been more limited and less conclusive. METHOD: This study investigated temperament differences in young boys (3-7 years old) with FXS (N = 26) recruited from a national FXS centre and controls (N = 26) matched on age, gender and race. RESULTS: Compared with controls, boys with FXS exhibited less overall surgency/extraversion and effortful control. Boys with FXS also displayed significantly greater activity and shyness and less attentional focusing, inhibitory control, soothability and high intensity pleasure (tendency to enjoy intense/complex activities), relative to comparison children. A significant interaction between age and diagnosis (FXS or control) was observed for negative affectivity only. CONCLUSIONS: 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.
Journal of intellectual disability research : JIDR, 2016 · doi:10.1111/jir.12304