Nonverbal Executive Functioning in Relation to Vocabulary and Morphosyntax in Preschool Children With and Without Developmental Language Disorder.

PURPOSE: Developmental language disorder (DLD) is characterized by persistent and unexplained difficulties in language development. Accumulating evidence shows that children with DLD also present with deficits in other cognitive domains, such as executive functioning (EF). There is an ongoing debate on whether exclusively verbal EF abilities are impaired in children with DLD or whether nonverbal EF is also impaired, and whether these EF impairments are related to their language difficulties. The aims of this study were to (a) compare nonverbal performance of preschoolers with DLD and typically developing (TD) peers, (b) examine how nonverbal EF and language abilities are related, and (c) investigate whether a diagnosis of DLD moderates the relationship between EF and language abilities. METHOD: A total of 143 children (nDLD = 65, nTD = 78) participated. All children were between 3 and 6.5 years old and were monolingual Dutch. We assessed nonverbal EF with a visual selective attention task, a visuospatial short-term and working memory task, and a task gauging broad EF abilities. Vocabulary and morphosyntax were each measured with two standardized language tests. We created latent variables for EF, vocabulary, and morphosyntax. RESULTS: Analyses showed that children with DLD were outperformed by their TD peers on all nonverbal EF tasks. Nonverbal EF abilities were related to morphosyntactic abilities in both groups, whereas a relationship between vocabulary and EF skills was found in the TD group only. These relationships were not significantly moderated by a diagnosis of DLD. CONCLUSIONS: We found evidence for nonverbal EF impairments in preschool children with DLD. Moreover, nonverbal EF and morphosyntactic abilities were significantly related in these children. These findings may have implications for intervention and support the improvement of prognostic accuracy. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.24121287.

Behaviors related to autism spectrum disorder in children with developmental language disorder and children with 22q11.2 deletion syndrome.

Background and Aim: Children with Developmental Language Disorder (DLD) are at an increased risk to develop behaviors associated with Autism Spectrum Disorder (ASD). The relationship between early language difficulties and the occurrence of ASD-related behaviors in DLD is poorly understood. One factor that may hinder progress in understanding this relationship is the etiological heterogeneity of DLD. We therefore study this relationship in an etiologically homogeneous group of children, who share phenotypic characteristics with children with DLD: children with the 22q11.2 Deletion Syndrome (22q11DS). We compare children with 22q11DS, to children with DLD and age-matched typically developing children (TD). Method: 44 children with 22q11DS, 65 children with DLD and 81 TD children, between 3.0-6.5 years old, participated in a longitudinal cohort study that included a baseline measure and a follow-up measure with a 1-year interval. A parental questionnaire (SRS-2) was used to measure the incidence of behaviors in two key behavioral domains associated with ASD: Social Communication and Interaction and Restricted Repetitive Behaviors and Interests. At baseline, we assessed children's expressive and receptive language abilities as well as their intellectual functioning with standardized tests. We compared the distribution of ASD-related behaviors between the three groups. We used regression analyses to investigate whether language abilities at baseline predict ASD-related behavior at follow-up, accounting for ASD-related behavior at baseline, demographic variables and intellectual functioning. Results: Both the children with 22q11DS and the children with DLD displayed significantly more ASD-related behaviors than the TD children. Over 30% of children in both clinical groups had scores exceeding the subclinical threshold for ASD in both behavioral domains. Both in 22q11DS and DLD, baseline receptive language scores were negatively correlated with ASD-related behaviors 1 year later, when controlling for baseline SRS-scores. However, this association was statistically significant only in children with 22q11DS, even when controlled for IQ-scores, and it was significantly stronger as than in the TD group. The strength of the association did not differ significantly between 22q11DS and DLD. Conclusion: Both children with 22q11DS and children with DLD present with elevated rates of ASD-related behaviors at a preschool-age. Only in children with 22q11DS we observed that weaker receptive language skills were related to increased behavioral problems in the domain of social communication and interaction one year later. Implications: Our findings indicate that relations between early language impairment and other behavioral phenotypes may be more feasible to detect in a subgroup of children with a homogeneous etiology, than in a group of children with a heterogeneous etiology (such as children with DLD). Our results in 22q11DS reveal that receptive language is especially important in predicting the occurrence of ASD-related behaviors. Future research is needed to determine to what extent receptive language predicts the occurrence of ASD-related behaviors in children with DLD, especially among those children with DLD with the weakest receptive language. Clinically, screening for ASD-related behaviors in children with developmental language difficulties is recommended from a young age, especially among children with receptive language difficulties.

Executive functioning in preschoolers with 22q11.2 deletion syndrome and the impact of congenital heart defects.

BACKGROUND: Executive functioning (EF) is an umbrella term for various cognitive functions that play a role in monitoring and planning to effectuate goal-directed behavior. The 22q11.2 deletion syndrome (22q11DS), the most common microdeletion syndrome, is associated with a multitude of both somatic and cognitive symptoms, including EF impairments in school-age and adolescence. However, results vary across different EF domains and studies with preschool children are scarce. As EF is critically associated with later psychopathology and adaptive functioning, our first aim was to study EF in preschool children with 22q11DS. Our second aim was to explore the effect of a congenital heart defects (CHD) on EF abilities, as CHD are common in 22q11DS and have been implicated in EF impairment in individuals with CHD without a syndromic origin. METHODS: All children with 22q11DS (n = 44) and typically developing (TD) children (n = 81) were 3.0 to 6.5 years old and participated in a larger prospective study. We administered tasks measuring visual selective attention, visual working memory, and a task gauging broad EF abilities. The presence of CHD was determined by a pediatric cardiologist based on medical records. RESULTS: Analyses showed that children with 22q11DS were outperformed by TD peers on the selective attention task and the working memory task. As many children were unable to complete the broad EF task, we did not run statistical analyses, but provide a qualitative description of the results. There were no differences in EF abilities between children with 22q11DS with and without CHDs. CONCLUSION: To our knowledge, this is the first study measuring EF in a relatively large sample of young children with 22q11DS. Our results show that EF impairments are already present in early childhood in children with 22q11DS. In line with previous studies with older children with 22q11DS, CHDs do not appear to have an effect on EF performance. These findings might have important implications for early intervention and support the improvement of prognostic accuracy.

The Language Profile of Preschool Children With 22q11.2 Deletion Syndrome and the Relationship With Speech Intelligibility.

PURPOSE: Young children with 22q11.2 deletion syndrome (22q11DS) often have impaired language development and poor speech intelligibility. Here, we report a comprehensive overview of standardized language assessment in a relatively large sample of preschool-aged children with 22q11DS. We furthermore explored whether speech ability explained variability in language skills. METHOD: Forty-four monolingual Dutch preschoolers (3-6 years) with a confirmed genetic 22q11DS diagnosis participated in this prospective cohort study. Standardized tests (Clinical Evaluation of Language Fundamentals Preschool-2-NL and Peabody Picture Vocabulary Test-III-NL) were administered. Speech intelligibility was rated by two expert speech and language therapists using a standardized procedure. RESULTS: Most children had impaired language skills across all tested domains. The composite score for expressive language was significantly lower than that for receptive language, but the two were strongly correlated. Only small differences between the mean scores on the various subtests were observed, with the lowest scores for expressive morphosyntactic skills. Language scores showed a moderate positive relation with speech intelligibility, but language abilities varied greatly among the children with intelligible speech. CONCLUSIONS: We show that the majority of preschool children with 22q11DS have a broad range of language problems. Other than the relatively larger impairment in expressive than in receptive language skills, our results do not show a clearly delineated language profile. As many of the children with intelligible speech still had below-average language scores, we highlight that language problems require a broad assessment and care in all young children with 22q11DS. Future research using spontaneous language and detailed speech analysis is recommended, to provide more in-depth understanding of children's language profile and the relationship between speech and language in 22q11DS.

Narrative comprehension and production abilities of children with 22q11.2 deletion syndrome.

BACKGROUND: The 22q11.2 Deletion Syndrome (22q11DS) is associated with language deficits and weak intellectual functioning. In other clinical groups, linguistic and cognitive difficulties have been associated with impaired acquisition of narrative abilities. However, little is known about the narrative abilities of children with 22q11DS. AIMS: To describe the ability of children with 22q11DS to produce and comprehend narrative macrostructure. Additionally, to examine the role of intellectual functioning in explaining their narrative difficulties. METHODS AND PROCEDURES: Narrative skills of 14 school-aged children with 22q11DS were compared to those of younger typically developing (TD) children matched on mental age and same-aged peers with Developmental Language Disorder (DLD). OUTCOMES AND RESULTS: Children with 22q11DS had significantly lower scores on narrative comprehension than younger TD children. No significant differences emerged on narrative production. Children with 22q11DS and children with DLD did not differ significantly on any of the narrative measures. CONCLUSIONS AND IMPLICATIONS: Narrative comprehension in children with 22q11DS seems more affected than production. Narrative comprehension difficulties cannot be entirely explained by a low level of intellectual functioning. Narrative comprehension and production abilities in 22q11DS require further consideration.

Reduced brain activation during spoken language processing in children with developmental language disorder and children with 22q11.2 deletion syndrome.

Language difficulties of children with Developmental Language Disorder (DLD) have been associated with multiple underlying factors and are still poorly understood. One way of investigating the mechanisms of DLD language problems is to compare language-related brain activation patterns of children with DLD to those of a population with similar language difficulties and a uniform etiology. Children with 22q11.2 deletion syndrome (22q11DS) constitute such a population. Here, we conducted an fMRI study, in which children (6-10yo) with DLD and 22q11DS listened to speech alternated with reversed speech. We compared language laterality and language-related brain activation levels with those of typically developing (TD) children who performed the same task. The data revealed no significant differences between groups in language lateralization, but task-related activation levels were lower in children with language impairment than in TD children in several nodes of the language network. We conclude that language impairment in children with DLD and in children with 22q11DS may involve (partially) overlapping cortical areas.