Ellen Van Den Heuvel1, Evi Jonkers2, Ellen Rombouts3, Eric Manders3, Inge Zink4, Ann Swillen5. 1. KU Leuven, Faculty of Medicine, Department of Neurosciences, Research Group Experimental Oto-Rhino-Laryngology (ExpORL), Leuven, Belgium; University Hospitals Leuven, Department of Oto-Rhino-Laryngology, Head & Neck Surgery, MUCLA, Leuven, Belgium. Electronic address: ellen.vandenheuvel@kuleuven.be. 2. KU Leuven, Faculty of Psychology and Educational Sciences, Leuven, Belgium. 3. KU Leuven, Faculty of Medicine, Department of Neurosciences, Research Group Experimental Oto-Rhino-Laryngology (ExpORL), Leuven, Belgium. 4. KU Leuven, Faculty of Medicine, Department of Neurosciences, Research Group Experimental Oto-Rhino-Laryngology (ExpORL), Leuven, Belgium; University Hospitals Leuven, Department of Oto-Rhino-Laryngology, Head & Neck Surgery, MUCLA, Leuven, Belgium. 5. KU Leuven, Faculty of Medicine, Department of Human Genetics, Leuven, Belgium; University Hospitals Leuven, Center for Human Genetics, Leuven, Belgium.
Abstract
BACKGROUND: Development of cognitive skills and social responsiveness are areas of concern in children with 22q11.2 deletion syndrome (22q11DS). It remains unclear if the cognitive and social profiles and trajectories are syndrome-specific or similar to those of children with idiopathic intellectual disabilities (IID) with or without comorbid autism spectrum disorder (ASD). AIMS AND METHODS: In this exploratory study, we examined and compared five broad cognitive abilities (BCAs) and the social responsiveness in primary school-aged children with 22q11DS (age 6-13, n = 21) and IQ-matched peers with IID (n = 21). The relative strengths and weaknesses of both groups were re-evaluated after 19 to 30 months. OUTCOMES AND RESULTS: Four different cognitive trajectories (i.e. absolute progress, stability, growing into deficit, and absolute decline) were demonstrated in both groups. Most children showed combined types of trajectories across BCAs resulting in a complex changing cognitive profile. In the 22q11DS group, social responsiveness problems increased, whereas no significant change was observed in the IID group. CONCLUSIONS AND IMPLICATIONS: Results reflect similar cognitive and social responsiveness profiles and trajectories across groups with children with 22q11DS being more at risk for growing into a social deficit. We recommend repeated monitoring of social skills development to adapt the environmental demands to the child's individual social capacities.
BACKGROUND: Development of cognitive skills and social responsiveness are areas of concern in children with 22q11.2 deletion syndrome (22q11DS). It remains unclear if the cognitive and social profiles and trajectories are syndrome-specific or similar to those of children with idiopathic intellectual disabilities (IID) with or without comorbid autism spectrum disorder (ASD). AIMS AND METHODS: In this exploratory study, we examined and compared five broad cognitive abilities (BCAs) and the social responsiveness in primary school-aged children with 22q11DS (age 6-13, n = 21) and IQ-matched peers with IID (n = 21). The relative strengths and weaknesses of both groups were re-evaluated after 19 to 30 months. OUTCOMES AND RESULTS: Four different cognitive trajectories (i.e. absolute progress, stability, growing into deficit, and absolute decline) were demonstrated in both groups. Most children showed combined types of trajectories across BCAs resulting in a complex changing cognitive profile. In the 22q11DS group, social responsiveness problems increased, whereas no significant change was observed in the IID group. CONCLUSIONS AND IMPLICATIONS: Results reflect similar cognitive and social responsiveness profiles and trajectories across groups with children with 22q11DS being more at risk for growing into a social deficit. We recommend repeated monitoring of social skills development to adapt the environmental demands to the child's individual social capacities.