4 results
64 Sluggish Cognitive Tempo in Pediatric Patients with Post-Acute Sequelae of COVID-19: Moderating Role of Depression on Functional Impairment
- Rowena Ng, Gray Vargas, Dasal Tenzin Jashar, Amanda Morrow, Laura A Malone
-
- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 59-60
-
- Article
-
- You have access Access
- Export citation
-
Objective:
Children with post-acute sequelae of COVID-19 (PASC) often report fatigue, attention problems, anxiety, and low mood. Sluggish cognitive tempo (SCT) is a constellation of behavioral symptoms (e.g., drowsiness, moving slowly, mental fogginess, daydreaming, confusion, or inattention) often associated with but distinct from attention-deficit/hyperactivity disorder (ADHD), executive function deficits and depressive symptoms. Given the apparent overlapping symptoms of PASC and SCT, this retrospective chart review aimed to 1) characterize SCT symptoms among pediatric patients with PASC relative to published normative and clinically referred samples, and 2) examine associations between subscales of SCT with ADHD symptoms, depression, anxiety, and functional impairment in this clinical sample.
Participants and Methods:This study included retrospective data from 25 patients with PASC (17 females; Mean age=13.73 years, SD=2.07, range=8-19) who were referred for a neuropsychological evaluation following a multidisciplinary visit at a post-COVID-19 rehabilitation clinic within an academic medical center. Patients’ caregivers completed the SCT Scale, ADHD Rating Scale 5 (ADHD-RS-V),
Conners Comprehensive Behavior Rating Scale (CBRS), and Impairment Rating Scale (IRS). Higher scores on the SCT, CBRS, and IRS total reflect more problems in the specified area. Welch’s t-tests were utilized to compare SCT scores from our cohort of pediatric patients with PASC relative to a normative community sample (Penny et al., 2009) and a heterogeneous clinically-referred sample (Koriakin et al., 2015). Bivariate correlations were computed to examine associations between SCT (Daydreamy, Low Initiation, Sluggish/Sleepy), ADHD (Inattention and Hyperactivity subscales from the ADHD-RS-V), affective symptoms (Major Depressive Episode (MDE) and Generalized Anxiety Disorder (GAD) scales from the CBRS), and functional impairment (average score from IRS). Multiple linear regressions were used to determine whether SCT factors independently contribute to variance in functional deficits after accounting for age of evaluation, low mood, and anxiety.
Results:Sluggish/Sleepy and Low Initiation were elevated in our cohort with PASC as compared to normative and mixed clinical samples from Penny et al. and Koriakin et al. (t>4.36, p<0.001). Patients with PASC had lower scores on the Daydreamy SCT scale than the clinically referred cohort (t=2.06, p=0.049), but similar to the normative sample (t=1.48, p=0.15). After controlling for age of testing, of the SCT subscales, only Low Initiation was associated with MDE (r=0.62, p=0.005), GAD (r=0.56, p=0.01) and overall Functional Impairment (r=0.48, p=0.04). Low Initiation was not correlated with Inattention or Hyperactivity. Notably, multiple regressions revealed Low Initiation scores were not associated with functional impairment when accounting for depression and anxiety symptoms(Low Initiation: ß=0.48, p=0.04; Low Initiation when depression and anxiety are included in independent regression models: ßs=0.13 and 0.29, ps=0.58 and 0.27 respectively).
Conclusions:Children and adolescents with PASC demonstrate more sluggish/sleepy presentation and difficulties with initiating activities or directing effort, as compared to normative and mixed clinically referred samples. Low initiation was associated with symptoms of MDE and GAD and functional impairment, but not with symptoms of ADHD. Depression and anxiety may moderate the association between poor initiation with functional impairment, highlighting the importance of psychological interventions to address mental health among youth with PASC and behavioral/cognitive concerns.
57 Executive Functioning Correlates with Adaptive Behaviors in Wiedemann-Steiner Syndrome
- Rowena Ng, Hans Tomas Bjornsson, Jill A Fahrner, Jacqueline Harris
-
- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 662-663
-
- Article
-
- You have access Access
- Export citation
-
Objective:
Wiedemann-Steiner syndrome (WSS) is a rare Mendelian disorder of epigenetic machinery caused by a mutation in KMT2A, with hallmark features that include intellectual disability and developmental delay. Animal models have helped identify the critical roles KMT2A plays in prefrontal neuron maturation and executive function (i.e. working memory) development. However, the neurobehavioral phenotype of individuals with WSS, including executive functioning, remains poorly characterized. Accordingly, this study aimed to 1.) examine the neurobehavioral profile (adaptive, psychosocial, and executive functioning) associated with WSS and 2.) the correlations between executive functioning and these domains.
Participants and Methods:A total of 25 mothers of individuals with WSS (13 females, Mean age=12.78 years, SD=7.88) completed a combination of parent-informant questionnaires. The caregivers completed the Adaptive Behavior Assessment System 3rd Edition (ABAS-3), the Strengths and Difficulties Questionnaire (SDQ), and a version of the Behavior Rating Inventory of Executive Function (BRIEF). Descriptive analyses were conducted to examine proportion of the sample with clinically significant concerns on the BRIEF and SDQ, and low to very low adaptive skills based on ratings on the ABAS-3. Partial correlations were computed to examine the relationships between overall executive functioning (BRIEF General Executive Composite, GEC) with adaptive domains (ABAS-3 Conceptual, Practical, Social), and psychosocial functioning (SDQ Emotional Problems, Conduct Problems, Hyperactivity, Peer Relations, Prosocial Behaviors) while accounting for age. Associations that survived Benjamin Hochberg correction are reported.
Results:Of our sample, 64% were rated in the very elevated range for executive functioning problems (BRIEF GEC), with a greater proportion endorsing clinically significant concerns with behavior regulation (68%) relative to cognitive regulation (48%). Majority of participants was rated in the low or very low range for ABAS-3 General Adaptive Composite (72%), with a greater proportion showing problems in Practical (64%) and Conceptual Domains (64%) relative to Social Domain (44%). Among those who completed the SDQ, caregiver ratings implicate elevated Total Problems (90%) with greater concerns observed in Emotional Problems (62%), Hyperactivity (81%) and Peer Relationship difficulties (95%). After controlling for age, executive functioning difficulties were associated with weaker skills in the Conceptual (r=-0.56, p=0.003), Social (r=-0.44, p=0.028) and Practical domains (r=-0.51, p=0.009); as well as more Hyperactivity (r=0.49, p=0.025) and Conduct problems (r=0.58, p=0.007). Specifically, day-to-day challenges with executive functions were related to weaker adaptive skills in Self-Care (r=-0.54, p=0.006), Self-Direction (r=-0.53, p=0.007), and Communication (r=-0.49, p=0.01).
Conclusions:Individuals with WSS are at risk for executive functioning deficits, which in turn may impact the development of and/or day-today application of adaptive skills and behavior regulation. Future clinical research should further explore the development and neurophysiology of executive functions among those with WSS with multidisciplinary methods, including behavioral, cognitive and neurobiological metrics. Those working with individuals with WSS may consider executive functioning interventions, which may yield indirect benefits to self-regulation and daily use of life skills.
Individuals with Wiedemann-Steiner syndrome show nonverbal reasoning and visuospatial defects with relative verbal skill sparing
- Rowena Ng, Jacqueline Harris, Jill A. Fahrner, Hans Tomas Bjornsson
-
- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue 5 / June 2023
- Published online by Cambridge University Press:
- 05 September 2022, pp. 512-518
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
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.
The neurobiology of self-processing in abused depressed adolescents
- Karina Quevedo, Rowena Ng, Hannah Scott, Garry Smyda, Jennifer H. Pfeifer, Sandra Malone
-
- Journal:
- Development and Psychopathology / Volume 29 / Issue 3 / August 2017
- Published online by Cambridge University Press:
- 20 October 2016, pp. 1057-1073
-
- Article
- Export citation
-
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.