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OBJECTIVE: Identify culturally insensitive tests and assessment practices based on a survey of neuropsychologists and neuropsychology trainees. METHOD: A survey was distributed to neuropsychology and psychology listservs asking for respondents to report tests, stimuli, and/or assessment practices perceived as being culturally insensitive and for which populations. A total of 100 participants provided responses, which were coded by three raters to identify commonly reported themes. Frequencies of themes (i.e. different issues related to culturally insensitive tests and practices) and how often specific tests were reported as culturally insensitive were determined. RESULTS: Lack of exposure due to items being biased toward U.S./Westernized culture or being unfamiliar based on age cohort, regional differences, and language background was the most commonly reported theme (20.1%), followed by tests and stimuli that were considered to be triggering or culturally offensive (17.4%). Among responses that mentioned specific tests, the Boston Naming Test was most frequently reported (43.2%), followed by the Wechsler Adult Intelligence Scale - Verbal subtests (20.3%), and Story B from the Wechsler Memory Scale-IV Logical Memory subtest (10.1%). CONCLUSIONS: Beyond the Boston Naming Test noose item, which was recently replaced, survey respondents identified several other culturally insensitive tests and assessment practices that may negatively impact an examinee's performance and their assessment experience. These results emphasize the need for more research to inform test revisions, updated normative data, and increased consideration for cultural differences to provide more equitable neuropsychological assessment services.

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KAT6B and KAT6A belong to the MYST family of lysine acetyltransferases, and regulate gene expression via histone modification. Although both proteins share similar structure and epigenetic regulatory functions, it remains unclear if KAT6A/6B mutation disorders, both very rare conditions, yield the same neurocognitive presentation and thus benefit from similar treatment approaches. This study provides a preliminary overview of neuropsychological functioning of 13 individuals with KAT6B disorder (Mean age = 9.01 years, SD = 5.46), which was compared to that of a recently published sample of 15 individuals with KAT6A syndrome (Mean age = 10.32 years, SD = 4.12). Participants completed a neuropsychological test battery to assess non-verbal cognition, and caregivers completed a series of standardized rating inventories to assess daily behavioral functioning. Results reveal those with KAT6B disorders present with severe adaptive deficits (92.3%) and autism-related behaviors (83.3%), juxtaposed with relatively low concerns with externalizing behaviors (7.6%), a pattern shared by the KAT6A group. Those with KAT6B disorders present with high levels of autistic features, including reduced affiliative interest, whereas social motivation is less affected within the KAT6A group. Overall, the levels of impairment in nonverbal cognition and receptive language were comparable among those with KAT6B disorders, a trend also seen in the KAT6A group. In brief, KAT6B and KAT6A disorders yield analogous neuropsychological profiles. Findings implicate common molecular pathophysiological mechanisms for these epigenetic disorders, such that similar therapies may have shared effect across diseases.

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Objective: The coronavirus disease-2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), has had a profound global impact on individual health and well-being in adults and children. While most fully recover from COVID-19, a relatively large subgroup continues to experience persistent physical, cognitive, and emotional/behavioral symptoms beyond the initial infection period. The World Health Organization has termed this phenomenon "Post-COVID-19 Condition" (PCC), better known as "Long COVID." Due to the cognitive and psychosocial symptoms, neuropsychologists often assess and recommend treatment for individuals with Long COVID. However, guidance for neuropsychologists' involvement in clinical care, policy-making, and research has not yet been developed. The authors of this manuscript convened to address this critical gap and develop guidance for clinical neuropsychologists working with patients presenting with Long COVID. Method: Authors include pediatric and adult neuropsychologists with expertise in Long COVID and behavioral health. All authors have been engaged in clinical and research efforts examining the impact of COVID-19. Authors summarized the literature-to-date pertinent to the neuropsychiatric sequelae of Long COVID and developed guidance for neuropsychologists working with individuals with Long COVID. Conclusions: Research findings regarding neuropsychiatric symptoms associated with Long COVID are mixed and limited by methodological differences. As they practice and conduct research, neuropsychologists should remain mindful of the evolving and tenuous nature of the literature.

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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.

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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.

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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.

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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.

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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.

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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.

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OBJECTIVE: When socioeconomic status is measured at the individual and/or family level, it has long been associated with cognition in children. However, the association between neighborhood deprivation, an index of community-level socioeconomic status, and child cognition is not fully understood. The goal of this study was to investigate (1) the relationship between neighborhood deprivation and child cognitive functioning and (2) whether child age moderates the relationship between cognitive functioning and neighborhood deprivation. METHODS: This study included 9878 children, ages 3 through 17 years (M = 10.4 yrs, SD = 3.4 yrs). Data were gathered from children referred for and evaluated at an urban, outpatient neuropsychology assessment clinic between 2006 and 2022, located in the Mid-Atlantic region of the United States. Neighborhood socioeconomic status was measured at the census block level using the Area Deprivation Index composite. RESULTS: There was a 20-point median difference in overall intelligence between the neighborhoods with the lowest and highest levels of deprivation. Overall intelligence and verbal comprehension, compared with working memory, fluid reasoning, and processing speed, demonstrated the strongest negative association with neighborhood deprivation (all p < 0.05). Older children had lower overall intelligence scores compared with younger children in neighborhoods with high levels of deprivation ( p < 0.01), suggesting a cumulative influence of poverty exposure. CONCLUSION: This study demonstrates the stark disparities in child cognitive functioning across levels of neighborhood deprivation. Findings support the importance of access to early interventions and services that promote intellectual growth and verbal capacity among children who live in neighborhoods with great deprivation.

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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.

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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.

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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.

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Many early-career neuroscientists with diverse identities may not have mentors who are more advanced in the neuroscience pipeline and have a congruent identity due to historic biases, laws, and policies impacting access to education. Cross-identity mentoring relationships pose challenges and power imbalances that impact the retention of diverse early career neuroscientists, but also hold the potential for a mutually enriching and collaborative relationship that fosters the mentee's success. Additionally, the barriers faced by diverse mentees and their mentorship needs may evolve with career progression and require developmental considerations. This article provides perspectives on factors that impact cross-identity mentorship from individuals participating in Diversifying the Community of Neuroscience (CNS)-a longitudinal, National Institute of Neurological Disorders and Stroke (NINDS) R25 neuroscience mentorship program developed to increase diversity in the neurosciences. Participants in Diversifying CNS were comprised of 14 graduate students, postdoctoral fellows, and early career faculty who completed an online qualitative survey on cross-identity mentorship practices that impact their experience in neuroscience fields. Qualitative survey data were analyzed using inductive thematic analysis and resulted in four themes across career levels: (1) approach to mentorship and interpersonal dynamics, (2) allyship and management of power imbalance, (3) academic sponsorship, and (4) institutional barriers impacting navigation of academia. These themes, along with identified mentorship needs by developmental stage, provide insights mentors can use to better support the success of their mentees with diverse intersectional identities. As highlighted in our discussion, a mentor's awareness of systemic barriers along with active allyship are foundational for their role.

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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.

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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.

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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).

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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.

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