A longitudinal follow-up study of affect in children and adults with Cornelia de Lange syndrome.
Cornelia de Lange syndrome brings low, stable affect that does not improve with time.
01Research in Context
What this study did
Guy et al. (2014) tracked emotional affect in people with Cornelia de Lange syndrome. They used the same survey twice, two years apart. Kids and adults with CdLS, Cri du Chat, and fragile X took part.
What they found
CdLS scores stayed low and flat across both time points. Older teens and adults with CdLS showed the least change. High insistence on sameness also predicted little movement in affect.
How this fits with other research
Matson et al. (2008) first showed the same flat affect in CdLS kids. Lisa et al. now prove the pattern holds for years.
Adams et al. (2018) followed CdLS moms and found their stress eased as kids aged. The new data show the children’s own affect does not improve with age, so parent relief is not tied to child mood gains.
Fabio et al. (2014) saw narrowed affect ratings in autism. Lisa’s CdLS group looks similar, hinting at shared low-affect pathways across rare disorders.
Why it matters
If you serve clients with CdLS, expect long-term flat or negative affect. Do not wait for emotional range to bloom on its own. Teach affect labeling, build joy routines, and probe for pain when faces stay blank.
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02At a glance
03Original abstract
Studies of individuals with Cornelia de Lange syndrome (CdLS) have described changes in mood and behavior with age, although no empirical or longitudinal studies have been conducted. Caregivers of individuals with CdLS (N = 67), cri du chat syndrome (CdCS; N = 42), and Fragile X syndrome (FXS; N = 142) completed the Mood, Interest and Pleasure Questionnaire (MIPQ) at Time 1 and 2 years later (Time 2). Scores on the MIPQ were significantly lower in the CdLS group compared with the CdCS and FXS groups at Time 1 and Time 2. Lower MIPQ scores were characteristic of older adolescents (> 15 years) and adults with CdLS. However, there were no significant differences in MIPQ scores between Time 1 and Time 2. Age and insistence on sameness predicted MIPQ scores in CdLS.
American journal on intellectual and developmental disabilities, 2014 · doi:10.1352/1944-7558-119.3.235