Associations Between Medical History, Cognition, and Behavior in Youth With Down Syndrome: A Report From the Down Syndrome Cognition Project

Abstract

The cause of the high degree of variability in cognition and behavior among individuals with Down syndrome (DS) is unknown. We hypothesized that birth defects requiring surgery in the first years of life (congenital heart defects and gastrointestinal defects) might affect an individual's level of function. We used data from the first 234 individuals, age 6-25 years, enrolled in the Down Syndrome Cognition Project (DSCP) to test this hypothesis. Data were drawn from medical records, parent interviews, and a cognitive and behavior assessment battery. Results did not support our hypothesis. That is, we found no evidence that either birth defect was associated with poorer outcomes, adjusting for gender, race/ethnicity, and socioeconomic status. Implications for study design and measurement are discussed.

The relationship between chronic health conditions and cognitive deficits in children, adolescents, and young adults with down syndrome: A systematic review

“…In contrast, studies conducted in older children and young adults observed no impact of CHD on IQ, motor skills, language ability, spatial learning, sensorimotor function, and performance-based and parent-reported aspects of executive function [33,37,39]. In the largest of these cohorts (n = 234), there was no association between CHD and either performance-based or parental ratings of cognitive performance in children ages 6-25 years [39], suggesting that corrected CHD does not have a significant impact on long-term cognitive development. Although lower DQ was reported in one study that included children three to six years old [38], cognitive deficits were not noted among children with DS and CHD age �3 years, albeit these conclusions are limited by the cross-sectional nature of these studies.…”

“…Seven out of fifteen articles (47%) reported on cardiovascular disorders in association with cognitive performance, all of which focused on congenital heart disease (CHD) [33][34][35][36][37][38][39]. Again, a wide range of performance-based cognitive assessments were used to measure IQ, developmental quotients (DQ), visual-motor abilities, gross/fine motor skills, language, behavior, cognition, learning and memory, and aspects of executive function including set-shifting, behavioral and emotional control, working memory, and planning/organizing skills.…”

Multiorgan involvement and management in children with Down syndrome

“…Over eighty conditions are associated with trisomy 21, but all do not occur within the one individual thus epigenetic differences are likely to be involved. 33,34 Visuo-spatial processing and perception are relative strengths in people with Down syndrome, where verbal short term memory appears to be an area of difficulty. 22,35 This variation in the level of intellectual disability can prove challenging when counselling families.…”

“…A few studies have looked at the impact of congenital heart disease, structural GI abnormalities and infantile spasms on the neurodevelopmental outcome on children with DS. 34,36,37…”

Altered Relationship between Soluble TREM2 and Inflammatory Markers in Young Adults with Down Syndrome

“…Assessment of subtle cognitive changes is challenging in DS, partly because of the presence and variability of intellectual disability 7,8 . Therefore, defining biomarkers of dementia progression are critical in this population of individuals.…”