The social phenotype associated with Wiedemann-Steiner syndrome: Autistic traits juxtaposed with high social drive and prosociality.

The aim of this study was to provide a descriptive overview of the social characteristics associated with Wiedemann-Steiner syndrome (WSS). A total of 24 parents of children/adults with WSS (11F, mean age = 12.94 years, SD = 8.00) completed the Social Responsiveness Scale 2nd Edition (SRS-2); Colorado Learning Difficulties Questionnaire (CLDQ) and Strengths and Difficulties Questionnaire (SDQ). Almost half our sample reported a diagnosis of autism spectrum disorder (ASD) and 70% had intellectual disability. On the SDQ, over 90% of participants were rated in borderline/clinical ranges in Peer Problems, yet the majority fell within normal limits in Prosocial Behaviors. Most fell in the moderate/severe difficulties ranges across SRS-2 Social Cognition, Communication, and Restricted/Repetitive Behaviors scales (all >70%); whereas substantially less participants met these ranges for deficits in Social Awareness (50%) and Social Motivation (33.33%). A pattern of relatively strong prosocial skills and social drive in the context of difficulties with inflexible behaviors, social cognition, and communication was observed, regardless of gender, ASD or intellectual disability diagnosis. The social phenotype associated with WSS is characterized by some autistic features paired with unusually high social motivation and prosocial tendencies.

Anxiety in Wiedemann-Steiner syndrome.

This study examined anxiety in Wiedemann-Steiner syndrome (WSS). Eighteen caregivers and participants with WSS completed the parent- and self-report versions of the Screen for Child Anxiety Related Disorder or the adapted version of the Screen for Adult Anxiety Related Disorder. Approximately 33.33% of parents and 65% of participants with WSS rated in the clinical range for overall anxiety. Across anxiety subtypes, parents primarily indicated concerns with Separation Anxiety (72%), which was also endorsed by the majority of participants with WSS (82%). The emergent trend showed Total Anxiety increased with age based on parent-informant ratings. The behavioral phenotype of WSS includes elevated anxiety. Clinical management should include incorporating early behavioral interventions to bolster emotion regulation given the observed risk of anxiety with age.

Individuals with Wiedemann-Steiner syndrome show nonverbal reasoning and visuospatial defects with relative verbal skill sparing.

OBJECTIVES: Wiedemann-Steiner syndrome (WSS) is a rare Mendelian disorder of the epigenetic machinery caused by heterozygous pathogenic variants in KMT2A. Currently, the specific neurocognitive profile of this syndrome remains unknown. This case series provides insight into the cognitive phenotype of WSS. METHODS: This study involves a retrospective medical chart review of 10 pediatric patients, each with a molecularly confirmed diagnosis of WSS who underwent clinical neuropsychological evaluation at an academic medical center. RESULTS: The majority of patients performed in the below average to very low ranges in Nonverbal Reasoning, Visual/Spatial Perception, Visuoconstruction, Visual Memory, Attention, Working Memory and Math Computation skills. In contrast, over half the sample performed within normal limits on Receptive Vocabulary, Verbal Memory, and Word Reading. Wilcoxon signed rank test showed weaker Nonverbal versus Verbal Reasoning skills (p = .005). Most caregivers reported deficits in executive functioning, most notably in emotion regulation. CONCLUSIONS: Nonverbal reasoning/memory, visuospatial/construction, attention, working memory, executive functioning, and math computation skills are areas of weakness among those with WSS. These findings overlap with research on Kabuki syndrome, which is caused by variants in KMT2D, and suggest disruption in the neurogenesis of the hippocampal formation may drive shared pathogenesis of the two syndromes.

Tumor region associated with specific processing speed outcomes.

OBJECTIVE: Processing speed (PS) is a vulnerable cognitive skill in pediatric cancer survivors as a consequence of treatments and, less consistently, tumor region. Studies conventionally examine graphomotor PS; emerging research suggests other aspects of PS may be impacted. This study examined types of PS in pediatric brain tumor survivors to determine which aspects are impaired. Given discordance across studies, we additionally investigated the relationship between brain region and PS. METHODS: The sample consisted of 167 pediatric brain tumor patients (100 supratentorial). PS (oral naming, semantic fluency, phonemic fluency, motor speed, graphomotor speed, visual scanning) was gathered via clinical neuropsychological assessment. To examine PS by region, infratentorial and supratentorial groups were matched on age at diagnosis and neuropsychological assessment, and time since diagnosis. RESULTS: The whole sample performed below normative means on measures of oral naming (p < .001), phonemic fluency (p < .001), motor speed (p = .03), visual scanning (p < .001), and graphomotor speed (p < .001). Only oral naming differed by region (p = .03), with infratentorial tumors associated with slower performance. After controlling for known medical and demographic risk factors, brain region remained a significant predictor of performance (p = .04). Among the whole sample, greater than expected proportions of patients with impairment (i.e., >1 standard deviation below the normative mean) were seen across all PS measures. Infratentorial tumors had higher rates of impairments across all PS measures except phonemic fluency. CONCLUSIONS: Results indicate pediatric brain tumor survivors demonstrate weaknesses in multiple aspects of PS, suggesting impairments are not secondary to peripheral motor slowing alone. Additionally, tumor region may predict some but not all neuropsychological outcomes in this population.

Working Memory and Processing Speed Predict Math Skills in Pediatric Brain Tumor Survivors.

OBJECTIVE: Childhood brain tumor (BT) survivors are at risk for working memory (WM) and processing speed (PS) deficits, which impact other cognitive domains. This study aimed to characterize WM, PS, and untimed mathematics calculation performance in pediatric BT survivors at least 2 years post-diagnosis, identify medical factors associated with deficits in mathematics, and examine whether WM and/or PS predict mathematics performance in this clinical sample. METHODS: Retrospective data were gathered from 72 BT survivors between 7 and 21 years of age ( M =13.64 y, SD =4.01 y) for a clinical neuropsychologic evaluation. All participants completed Wechsler measures of WM and PS and a measure of untimed mathematics calculation. RESULTS: WM, PS, and the mathematics calculation were significantly lower than the normative mean. Math scores were not correlated with any of the examined medical factors. PS was negatively correlated with the Neurological Predictor Scale and positively correlated with age at diagnosis. Both WM and PS were associated with math outcomes and accounted for 30.4% and 19.2% of the variance, respectively. CONCLUSIONS: The findings indicate that WM and PS contribute to mathematics performance in pediatric BT survivors. Examining mathematics performance should be a part of clinical neuropsychological evaluations. Interventions to improve mathematics performance in this population should also focus on WM and compensatory strategies for slowed PS.

Predictors of behavioral dysregulation and social dysfunction in pediatric patients with epilepsy and obesity: Developmental timing matters.

Childhood obesity has been associated with greater risks of psychopathology, including externalizing behaviors and social dysfunction. However, there has been limited research on the effect of obesity on psychosocial functioning among pediatric patients with epilepsy. Accordingly, this study aimed to examine the association between overweight/obesity and behavioral and social functioning among pediatric patients with epilepsy, as function of their developmental status. We completed a retrospective chart review of data (seizure factors, intellectual functioning, caregiver ratings of aggression/defiance and social problems, sex- and age-adjusted body mass index (BMI) percentile) from 30 school-age children and 30 adolescents with epilepsy evaluated through our institute. Body mass index percentile and overweight/obesity status were not associated with seizure variables. Despite controlling for intellectual functioning, overweight/obesity was associated with greater aggression/defiance among child patients but not adolescents. In contrast, adolescence, but not weight status, was associated with social dysfunction. In brief, while early interventions focused on behavior and weight management among children with epilepsy may mitigate risk of aggression and oppositional behavior, among adolescent patients, therapies directed towards promoting interpersonal skills, sense of social competency, and expansion of social support system may reduce psychosocial maladjustment. Findings highlight need to understand the developmental effects of common medical comorbidities on neurobehavioral functioning among youth with epilepsy.

Abnormalities in early visual processes are linked to hypersociability and atypical evaluation of facial trustworthiness: An ERP study with Williams syndrome.

Accurate assessment of trustworthiness is fundamental to successful and adaptive social behavior. Initially, people assess trustworthiness from facial appearance alone. These assessments then inform critical approach or avoid decisions. Individuals with Williams syndrome (WS) exhibit a heightened social drive, especially toward strangers. This study investigated the temporal dynamics of facial trustworthiness evaluation in neurotypic adults (TD) and individuals with WS. We examined whether differences in neural activity during trustworthiness evaluation may explain increased approach motivation in WS compared to TD individuals. Event-related potentials were recorded while participants appraised faces previously rated as trustworthy or untrustworthy. TD participants showed increased sensitivity to untrustworthy faces within the first 65-90 ms, indexed by the negative-going rise of the P1 onset (oP1). The amplitude of the oP1 difference to untrustworthy minus trustworthy faces was correlated with lower approachability scores. In contrast, participants with WS showed increased N170 amplitudes to trustworthy faces. The N170 difference to low-high-trust faces was correlated with low approachability in TD and high approachability in WS. The findings suggest that hypersociability associated with WS may arise from abnormalities in the timing and organization of early visual brain activity during trustworthiness evaluation. More generally, the study provides support for the hypothesis that impairments in low-level perceptual processes can have a cascading effect on social cognition.

The neurobiology of self-processing in abused depressed adolescents.

Maltreatment is associated with chronic depression, high negative self-attributions, and lifetime psychopathology. Adolescence is a sensitive period for the formation of self-concept. Identifying neurobiomarkers of self-processing in depressed adolescents with and without maltreatment may parse the effects of trauma and depression on self-development and chronic psychopathology. Depressed adolescents (n = 86) maltreated due to omission (DO, n = 13) or commission (DCM, n = 28) or without maltreatment (DC, n = 45), and HCs (HC, n = 37) appraised positive and negative self-descriptors in the scanner. DCM and DO showed hypoactivity in the dorsal anterior cingulate cortex (dACC) while processing positive versus negative self-descriptors compared to DC youth, who in turn showed reduced dACC recruitment versus HC. HC youth showed the highest activation in the dACC and striatum during positive self-descriptors; these regions showed a linear decline in activity across DC, DO, and DCM. Low dACC activity to positive versus negative self-descriptors was linked to inadequate coregulation of children's emotions by parents. Negative self-cognitions prevalent in DCM and DO adolescents may be perpetuated by activity in the dACC and striatum. Reduced activation of the dACC and striatum for positive self-descriptors, coupled with enhanced activity for negative self-descriptors, may heighten the risk for persistent depression.

IMPACT OF JOINT ATTENTION ON SOCIAL-COMMUNICATION SKILLS IN INTERNATIONALLY ADOPTED CHILDREN.

Internationally adopted (IA) children have often experienced early adversity and are at risk for long-term deficiencies in multiple developmental domains. This study examined the association between IA children's joint attention (JA) soon after arrival and later cognitive, communicative, and socioemotional competency 6 months' postadoption. We expected a child's initial JA would positively predict later cognitive, communication, and social ability. IA children (n = 63) adopted from Eastern Europe were seen soon after their arrival into the United States to assess their JA. Their socioemotional competency, social communication, and cognitive abilities were measured at a follow-up session 6 months' postadoption. We found that higher order JA was positively associated with measures of social relatedness. Furthermore, individual hierarchical regressions of each measure of JA (higher order JA, initiating JA, responding to JA, and initiating behavior requests [BR]) considered with age-at-adoption showed that each measure was an independent and positive predictor of Mullen outcomes in the receptive and expressive language domains. These results suggest that JA may be a sensitive predictor of subsequent functioning in the social, communicative, and cognitive domains. Thus, assessing JA soon after arrival has the potential to identify at-risk IA children, and interventions targeting JA may support those children in overcoming the negative impacts of early adversity.

Social functioning and autonomic nervous system sensitivity across vocal and musical emotion in Williams syndrome and autism spectrum disorder.

Both Williams syndrome (WS) and autism spectrum disorders (ASD) are associated with unusual auditory phenotypes with respect to processing vocal and musical stimuli, which may be shaped by the atypical social profiles that characterize the syndromes. Autonomic nervous system (ANS) reactivity to vocal and musical emotional stimuli was examined in 12 children with WS, 17 children with ASD, and 20 typically developing (TD) children, and related to their level of social functioning. The results of this small-scale study showed that after controlling for between-group differences in cognitive ability, all groups showed similar emotion identification performance across conditions. Additionally, in ASD, lower autonomic reactivity to human voice, and in TD, to musical emotion, was related to more normal social functioning. Compared to TD, both clinical groups showed increased arousal to vocalizations. A further result highlighted uniquely increased arousal to music in WS, contrasted with a decrease in arousal in ASD and TD. The ASD and WS groups exhibited arousal patterns suggestive of diminished habituation to the auditory stimuli. The results are discussed in the context of the clinical presentation of WS and ASD.

Neuropsychological profile associated with KAT6A syndrome: Emergent genotype-phenotype trends.

BACKGROUND: KAT6A (Arboleda-Tham) syndrome is a Mendelian disorder of the epigenetic machinery caused by pathogenic variants in the lysine acetyltransferase 6 A (KAT6A) gene. Intellectual disability and speech/language impairment (e.g., minimally verbal) are common features of the disorder, with late-truncating variants associated with a more severe form of intellectual disability. However, much of the cognitive phenotype remains elusive given the dearth of research. PARTICIPANTS AND METHODS: This study examined non-verbal and social skills of 15 individuals with molecularly-confirmed diagnoses of KAT6A syndrome (Mean age = 10.32 years, SD = 4.12). Participants completed select subtests from the DAS-II, the NEPSY-II, and the Beery Buktenica Developmental Test of Visual Motor Integration 6th Edition, and their caregivers completed an assortment of behavior rating inventories. RESULTS: Findings suggest global cognitive impairment with nonverbal cognition scores similar to those for receptive language. Autism-related features, particularly restricted interests and repetitive behaviors, and broad adaptive deficits were common in our sample juxtaposed with a relatively strong social drive and low frequency of internalizing and externalizing behavioral problems. A general trend of lower performance scores on nonverbal and receptive language measures was observed among those with protein-truncating variants vs. missense variants; however, no effect was observed on caregiver rating inventories of daily behaviors. Late and early truncating variants yielded comparable neuropsychological profiles. CONCLUSIONS: Overall, study results show the cognitive phenotype of KAT6A syndrome includes equally impaired nonverbal cognition and receptive language functioning, paired with relatively intact social drive and strengths in behavior regulation. Emergent genotype-phenotype correlations suggest cognition may be more affected in protein-truncating than missense mutations although similar neurobehavioral profiles were observed.

Associations Between Executive Functioning, Behavioral Functioning, and Adaptive Functioning Difficulties in Wiedemann-Steiner Syndrome.

OBJECTIVES: Wiedemann-Steiner syndrome (WSS) is a neurogenetic disorder caused by heterozygous variants in KMT2A. Recent investigations suggest increased anxiety and behavior regulation challenges among those with WSS although the neurobehavioral phenotype remains largely unknown. This study aims to examine the pattern of and associations between executive functioning (EF) and behavior functioning among those with WSS. METHOD: This study involved utilizing caregiver-report inventories (Behavior Rating Inventory of Executive Function 2nd Edition, BRIEF-2; Adaptive Behavior Assessment 3rd Edition, ABAS-3; Strengths and Difficulties Questionnaire, SDQ) to assess day-to-day behavior functioning among those with WSS (N = 24; mean age = 10.68 years, SD = 3.19). Frequency of clinical elevations in daily difficulties in EF, adaptive behaviors, and behavior regulation were reported. Correlations and hierarchical linear regressions were used to determine the relationships between EF with behavior and adaptive functioning. RESULTS: Out of our sample, 63% met clinical levels of executive functioning difficulties on the BRIEF-2, and 75% with Hyperactivity and 54% with Emotional Problems on the SDQ. In addition, 33% were rated >2 SD below the normative mean in overall adaptive functioning on the ABAS-3. Elevated ratings in BRIEF-2 Shift, reflective of challenges with mental flexibility, predicted more Emotional Problems and accounted for 33.5% of its variance. More difficulties in Emotional Control were related to greater adaptive deficits, accounting for 33.3% of its variance. CONCLUSIONS: Those with WSS are at risk for EF deficits, hyperactivity, and emotional dysregulation. EF correlates with adaptive and affective behaviors, highlighting the promise of behavioral interventions to target cognitive flexibility, emotional awareness, and reactivity in this population.

Neurobehavioral and developmental profiles: genotype-phenotype correlations in individuals with Cornelia de Lange syndrome.

BACKGROUND: Cornelia de Lange (CdLS) is a rare genetic disorder that affects most body systems. Variants in multiple genes including NIPBL and SMC1A, can cause the syndrome. To date, literature on genotype-phenotype associations in individuals with CdLS is extremely limited, although studies suggest some differences in clinical phenotype severity across variants. This study aimed to examine and compare neurobehavioral differences and developmental variability across CdLS genes, specifically NIPBL and SMC1A, and identify genotype-phenotype correlations. PARTICIPANTS AND METHODS: This patient-reported outcomes study included accessing data from the Coordination of Rare Diseases registry at Sanford. Parents of a total of 26 children/adults with CdLS and a known variant in NIPBL (Mean age = 20.46 years, SD = 11.21) and 12 with a known variant in SMC1A (Mean age = 11.08 years, SD = 9.04) completed a series of questionnaires regarding their child's developmental history. This included attainment of common language and motor milestones, intervention history, and behavior functioning. Developmental history and reported behavior regulation difficulties were compared across variant groups. RESULTS: Overall, individuals with a pathogenic variant in NIPBL or SMC1A were similarly delayed across motor and language milestones with about 70% not using phrase speech and 30-50% not walking by 5 years of age. However, those with NIPBL variants showed more severity in behavioral phenotype, namely with more repetitive behaviors, tantrums, and withdrawn behaviors. In addition, these individuals were more likely than those with SMC1A variants to demonstrate self-injurious behaviors, and anxiety. Both groups yielded a similar proportion of participants who participated in speech and occupational therapy, however those with SMC1A variants were more likely to engage in physical therapy. Both clinical groups report low rate of communicative or assistive device use despite a large proportion of participants never mastering single word or sentence use. CONCLUSIONS: Study results are consistent with recent investigations highlighting more severe behavioral phenotype, particularly autistic features, anxiety, and behavior regulation challenges, among those with NIPBL variants albeit comparable developmental milestones. Both groups endorsed very elevated attention problems. Findings highlight importance of early interventions, including behavioral health services.

Neuropsychological functioning of pediatric patients with long COVID.

Objective: To determine the neurocognitive profile for youth with long COVID presenting with cognitive concerns. Method: This study is a case series of 54 pediatric patients (65% female, Mage = 13.48, SDage = 3.10, 5-19) with long COVID who were referred for neuropsychological testing from a post-COVID-19 multidisciplinary clinic. The outcomes of interest were neuropsychological test scores and parent ratings of mood, attention, and executive functioning. The percentage of patients with neuropsychological test scores below the 9th percentile (below average range) and those with at-risk or clinically significant scores (T-scores > 59) on parent-informant inventories were computed. Results: A portion of children with long COVID showed weaknesses in sustained attention (29%) and divided attention (35%). This portion of patients did not significantly differ when comparing patients with and without pre-existing attention and mood concerns. A high percentage of parents reported at-risk to clinically significant concerns for cognitive regulation (53%), depression (95%), anxiety (85%), and inattention (66%) on standardized questionnaires. Conclusions: The present case series showed that approximately a third of children with long COVID demonstrate objective weaknesses on sustained and divided attention tasks but were largely intact in other domains of neuropsychological functioning. Importantly, children with long COVID had similar difficulties in attention, regardless of pre-existing attention or mood concerns. Parents reported high rates of mood, anxiety, and executive functioning difficulties which likely impact daily functioning. Attention and emotional regulation should be closely monitored and treated as necessary in pediatric patients with long COVID to aid functional recovery.

To confirm your appointment, please press one: Examining demographic and health system interface factors that predict missed appointments in a pediatric outpatient neuropsychology clinic.

Objective: Missed patient appointments have a substantial negative impact on patient care, child health and well-being, and clinic functioning. This study aims to identify health system interface and child/family demographic characteristics as potential predictors of appointment attendance in a pediatric outpatient neuropsychology clinic. Method: Pediatric patients (N = 6,976 across 13,362 scheduled appointments) who attended versus missed scheduled appointments at a large, urban assessment clinic were compared on a broad array of factors extracted from the medical record, and the cumulative impact of significant risk factors was examined. Results: In the final multivariate logistic regression model, health system interface factors that significantly predicted more missed appointments included a higher percentage of previous missed appointments within the broader medical center, missing pre-visit intake paperwork, assessment/testing appointment type, and visit timing relative to the COVID-19 pandemic (i.e. more missed appointments prior to the pandemic). Demographic characteristics that significantly predicted more missed appointments in the final model included Medicaid (medical assistance) insurance and greater neighborhood disadvantage per the Area Deprivation Index (ADI). Waitlist length, referral source, season, format (telehealth vs. in-person), need for interpreter, language, and age were not predictive of appointment attendance. Taken together, 7.75% of patients with zero risk factors missed their appointment, while 22.30% of patients with five risk factors missed their appointment. Conclusions: Pediatric neuropsychology clinics have a unique array of factors that impact successful attendance, and identification of these factors can help inform policies, clinic procedures, and strategies to decrease barriers, and thus increase appointment attendance, in similar settings.

Epigenetics of cognition and behavior: insights from Mendelian disorders of epigenetic machinery.

Epigenetics, one mechanism by which gene expression can change without any changes to the DNA sequence, was described nearly a century ago. However, the importance of epigenetic processes to neurodevelopment and higher order neurological functions like cognition and behavior is only now being realized. A group of disorders known as the Mendelian disorders of the epigenetic machinery are caused by the altered function of epigenetic machinery proteins, which consequently affects downstream expression of many genes. These disorders almost universally have cognitive dysfunction and behavioral issues as core features. Here, we review what is known about the neurodevelopmental phenotypes of some key examples of these disorders divided into categories based on the underlying function of the affected protein. Understanding these Mendelian disorders of the epigenetic machinery can illuminate the role of epigenetic regulation in typical brain function and can lead to future therapies and better management for a host of neurodevelopmental and neuropsychological disorders.

Clinical utility of teleneuropsychology among pediatric patients with broadly average and low intellectual functioning.

The reliability of teleneuropsychology (teleNP) within pediatric populations, particularly those with low intellectual functioning (LIF; i.e., Intellectual Quotient <80), is largely unknown. This repeated-measures study compared performance on WISC-V and WAIS-IV subtests administered in-person before the COVID-19 pandemic and via teleNP during the pandemic in individuals with LIF versus broadly average (BA) intellectual functioning in a clinically referred pediatric cohort. Data were collected from a retrospective chart review of 35 pediatric patients who underwent in-person neuropsychological evaluation at an academic medical center before the pandemic (Mage = 10.10 years, SD = 2.93) and videoconference teleNP assessment during the pandemic (Mage = 13.47 years, SD = 2.88). Participants completed the Similarities, Matrix Reasoning, and Digit Span subtests from the WISC-V or WAIS-IV at both time points. After controlling for test-retest time interval, partial correlations showed relatively strong associations in test-retest performance across subtests in the whole sample and among the subset of LIF patients. Distribution of significant reliable change indices (RCI) between the LIF and BA groups were similar. Strong correlations were observed between performances on select Wechsler subtests administered in-person and via teleNP. Results lend initial support toward the utility of teleNP administration of these measures in children with a broad range of intellectual functioning.

Performing pediatric neuropsychological evaluation with Chinese patients: A narrative review of the literature and recommendations for practice.

Objective: This paper offers a narrative overview of performance-based cognitive tests and behavior rating inventories that can be considered when working with Chinese youth in assessment settings. Methods: A total of 46 articles that focused on assessment tools and normative data in Chinese-speaking patients were reviewed. Based on the gaps in research and patterns of strengths/weaknesses across reviewed articles, we provide recommendations for selection of tests and norms while utilizing the ECLECTIC framework. Results: Our review of literature suggests extant research on neuropsychological tools for Chinese pediatric patients have largely been focused on translated or adapted measures. Findings highlight the need for the development of indigenous measures across multiple cognitive and behavioral domains to optimally integrate cultural considerations in the assessment process. Conclusions: Specifically, cultural factors that may impact test and norm selection, patients' test performance, and diagnostic considerations are discussed. Finally, gaps in literature are highlighted for future research directions.

Mind-mindedness in a high-risk sample: Differential benefits for developmental outcomes based on child maltreatment.

Mind-mindedness is associated with positive developmental outcomes. However, much of the literature uses mostly White, middle to high socioeconomic status (SES) samples despite evidence that the benefits of mind-mindedness may vary based on degree of social risk. Additionally, few studies have examined relations between mind-mindedness and language development. The current study investigates whether mind-mindedness predicts children's language development and behavioral functioning and if family history of childhood maltreatment moderates the relation of mind-mindedness to these outcomes. Participants were 98 mothers (49.0% Black, 24.5% White, 13.3% Latina, 7.2% multiracial; 81.6% low SES per Hollingshead classifications) and their children (49 boys, 49 girls) from the Rochester, New York area recruited at Time 1 (Mage = 13.34 months) and followed up twice (Mages = 27.51 [Time 2] and 39.31 months [Time 3]). Mother-child dyads participated in videotaped free play interactions at Times 1 and 2. Using transcripts of these interactions, we coded mind-mindedness at Times 1 and 2 and children's internal state language at Time 2. Mothers reported on children's behavioral functioning at Time 3. Findings revealed that Time 2 mind-mindedness predicted fewer Time 3 behavioral difficulties in children from maltreating families but did not predict behavioral difficulties in children from nonmaltreating families. Additionally, Time 1 mind-mindedness predicted children's Time 2 use of decontextualized internal state language. Findings highlight the importance of examining mind-mindedness in higher risk populations and how mind-mindedness can be leveraged as a protective factor to prevent future maladjustment in children at risk for adverse outcomes. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Cognitive disengagement syndrome in pediatric patients with long COVID: associations with mood, anxiety, and functional impairment.

Children with long COVID often report symptoms that overlap with cognitive disengagement syndrome (CDS, previously sluggish cognitive tempo (SCT)), a set of behaviors distinct from attention-deficit/hyperactivity disorder (ADHD) including excessive daydreaming, mental fogginess, and slowed behavior and thinking. Those with long COVID also frequently report low mood and anxiety, which are linked to CDS. The relationships between cognitive difficulties, mood, and functional impairment have yet to be explored in pediatric long COVID. Specifically, it is unclear how much cognitive difficulties (CDS, inattention) contribute to functional impairment, when accounting for mood/anxiety symptoms in this population. Retrospective parent-reported data was collected from 34 patients with long COVID (22 females, Mage = 14.06 years, SD = 2.85, range 7-19) referred for neuropsychological consultation through a multidisciplinary Post-COVID-19 clinic. Compared to community and clinically referred samples, on average, long COVID patients showed elevated CDS symptoms, including Sluggish/sleepy (e.g., fatigue) and Low Initiation subscales (e.g., difficulty performing goal directed behaviors). Low Initiation, mood, anxiety, and inattention were associated with functional impairment. In multiple hierarchical regression models, after controlling for mood and anxiety, Low Initiation and inattention were no longer predictive of functional impairment. Instead, anxiety remained the sole predictor of functional impairment. Our results demonstrate that children with long COVID have high levels of CDS symptoms. The association between cognitive difficulties and functional impairment dissipated with the inclusion of mood and anxiety, suggesting behavioral health interventions targeting anxiety may help improve daily functioning and quality of life in pediatric long COVID patients.