Adaptive Behavior Assessment System-Second Edition Research Articles

Adaptive behavior in primary agenesis of the corpus callosum

Background and aimsPrimary agenesis of the corpus callosum (ACC) is a congenital neurological disorder characterized by the absence, either partial or complete, of the corpus callosum in individuals who do not have intellectual disability and are otherwise neurologically asymptomatic. While mild to moderate neurocognitive deficits have been observed in individuals with primary ACC using neuropsychological assessments, the impact of this syndrome on adaptive behavior remains insufficiently understood. MethodsThis study used self- and informant-ratings on the Adaptive Behavior Assessment System, Second Edition (ABAS-II) to evaluate adaptive behavior in 35 adults diagnosed with primary ACC. ResultsWhile adults with primary ACC reported adaptive functioning comparable to an age-adjusted normative sample, family informants rated their adaptive ability below norms in several skill domains, particularly social skills. ConclusionsThis pattern of lower ratings by informants than self-ratings suggests adults with ACC may have poor understanding of their own behavior and its consequences. This study demonstrates that informants observe significant deficiencies in the conceptual, social, and practical aspects of adaptive behavior in persons with primary ACC, and that these deficiencies are not seen as clearly by the persons themselves.

Sleep disturbances and behavioral symptoms in pediatric Sotos syndrome.

Sotos syndrome (SoS) is a rare overgrowth genetic disease caused by intragenic mutations or microdeletions of the NSD1 gene located on chromosome 5q35. SoS population might present cognitive impairment and a spectrum of behavioral characteristics, with a worse profile in patients with microdeletion. Although patients with SoS are known to have impaired sleep habits, very little data are available. The present study aimed to assess the prevalence of sleep disorders (SDs) in a pediatric cohort of patients with SoS and their correlation with neuropsychiatric profiles. We included patients with a SoS diagnosis and age < 18 years; all patients underwent a comprehensive neuropsychological assessment, including evaluation of cognition, adaptive functions through the Adaptive Behavior Assessment System-Second Edition (ABAS-II), and behavioral problems using the Achenbach Child Behavior Checklist (CBCL) and Conners' Parent Rating Scale-Revised (CPRS-R:L) questionnaire. To investigate the presence of SD parents, the Sleep Disturbance Scale for Children (SDSC) was completed. Thirty-eight patients (M 61%, F 39%, mean age 11.1 ± 4.65 years) were included in the study. Although only two had a prior SD diagnosis, 71.1% (N = 27) exhibited pathological scores on SDSC. No statistically significant associations were found between positive SDSC results and genetic microdeletion, intellectual disability (ID), or other medical conditions/treatments. However, a positive correlation emerged between SDSC scores and Conners' Global Index (p = 0.048) and Restless/Impulsive (p = 0.01) scores, CBCL externalizing (p = 0.02), internalizing (p = 0.01), and total scores (p = 0.05). Conversely, a negative linear relationship was observed between the SDSC score and the ABAS GAC and ABAS CAD scores (p = 0.025). We detected an SD in 71.1% of our sample, with a positive relation between SD and internalizing and externalizing symptom levels, especially hyperactivity and impulsivity. Our study demonstrated a high prevalence of SD in pediatric patients with SoS, highlighting that all patients should be screened for this problem, which has a great impact on the quality of life of patients and their families.

Influence of parental education on the intelligence quotient profiles and socially adaptive behavior of school-age children with autism spectrum disorder in eastern China.

Intelligence quotient (IQ) and adaptive behavior are the influencing factors of autism spectrum disorder (ASD) in children entering mainstream schools. This study explored the association between parental education level, IQ, and adaptive behavior in ASD groups. A total of 257 school-age ASD children were enrolled in our study from January 2017 to June 2021. Their parents completed a standard demographic form, including age at autism diagnosis, gender, school placement, and parents' educational background. The Chinese version of the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV) was completed by a certified assessor for each enrolled child. Parents were interviewed on adaptive behavior using the Chinese version of the Adaptive Behavior Assessment System, Second Edition (ABAS-II). The average IQ of school-age ASD children was 76.88 (standard deviation (SD)=22.62) and boys had higher IQ levels than girls. The IQ was positively correlated with age. The General Adaptive Composite (GAC) score was 82.47 (SD=15.86) and adaptive behavior did not increase with age. ASD children who attended mainstream schools had better adaptive behavior profiles than other children. The mother's education level showed a significant correlation with the IQ and adaptive behavior of autistic children, while the father's education level did not. Consequently, better training and support for parents may help autistic children enter mainstream schools, with adaptive training being the most urgently required skill for parents.

The Adolescent/Young Adult Self-Management and Independence Scale (AMIS-II): Expanding evidence for validity and reliability.

The purpose of this longitudinal study was to evaluate the internal consistency reliability and construct validity of the Adolescent/Young Adult Self-Management and Independence Scale-II (AMIS-II), an interview-based measure of self-management for youth with chronic health conditions. A diverse sample of adolescents and young adults (AYA) with spina bifida (SB) (n = 64 AYA; mean 20.88; age range 18-25 years) completed an AMIS-II interview. Six years earlier, parents from 55 families completed questionnaires that assessed children's responsibility for SB-related care (Sharing of Spina Bifida Management Responsibilities) and their ability to perform skills across condition-related tasks (The Spina Bifida Independence Survey). Parents also reported on child's communication skills, adaptive behaviors, and independent management of finances (Adaptive Behavior Assessment System-Second Edition; Scales of Independent Behavior-Revised). Descriptive and correlational analyses were conducted to assess the construct validity and the internal consistency reliability of the AMIS-II. The AMIS-II demonstrated excellent internal consistency reliability (AMIS-II total scale α= 0.95; subscales α= 0.90 -0.91). Evidence in support of construct validity was found in associations between the AMIS-II and measures of child responsibility for SB-related care, ability to perform condition-related skills, and adaptive behaviors (r's = 0.378 -0.777; p's < 0.05). This study provides further evidence of strong reliability and validity for the AMIS-II. Additional research with this measure will facilitate a better understanding of factors related to self-management behaviors in adolescents and young adults with spina bifida.

The Floor Effect on the ABAS-II in Adolescents with Autism Spectrum Disorder.

The present study aimed at enhancingtheassessment of adaptive behaviorinadolescentswithAutism SpectrumDisordertroughthe Adaptive Behavior Assessment System-SecondEdition. Toovercomethe limits underpinning floor effect,which are commonly observed with the traditionalmethods, the statistical Hessl method and its revision by Orsini, Pezzuti and Hulbertwas adopted.The results showed a more variationusing the former method compared to the latter: a greater impairment in Socialversus Leisure skills emerged. Regarding the Conceptual domain, a greater deficit in Functional Academics was observed. In the Practical domain, we found a greater impairment in Self-Care skills. Conversely, Home Living skills appeared as a strength compared to other skills. The results are discussed in reference toliterature.

Infant repetition effects and change detection: Are they related to adaptive skills?

Repetition effects and change detection response have been proposed as neuro-electrophysiological correlates of fundamental learning processes. As such, they could be a good predictor of brain maturation and cognitive development. We recorded high density EEG in 71 healthy infants (32 females) aged between 3 and 9months, while they listened to vowel sequences (standard /a/a/a/i/ [80%] and deviant /a/a/a/a/ [20%]). Adaptive skills, a surrogate of cognitive development, were measured via the parent form of the Adaptive Behavior Assessment System Second Edition (ABAS-II). Cortical auditory-evoked potentials (CAEPs) analyses, time-frequency analyses and a statistical approach using linear mixed models (LMMs) and linear regression models were performed. Age and adaptive skills were tested as predictors. Age modulation of repetition effects and change detection response was observed in theta (3-5Hz), alpha (5-10Hz) and high gamma (80-90 Hz) oscillations and in all CAEPs. Moreover, adaptive skills modulation of repetition effects was evidenced in theta (3-5Hz), high gamma oscillations (80-90 Hz), N250/P350 peak-to-peak amplitude and P350 latency. Finally, adaptive skills modulation of change detection response was observed in the N250/P350 peak-to-peak amplitude. Our results confirm that repetition effects and change detection response evolve with age. Moreover, our results suggest that repetition effects and change detection response vary according to adaptive skills displayed by infants during the first year of life, demonstrating their predictive value for neurodevelopment.

Sex Differences in Autism Spectrum Disorder: Repetitive Behaviors and Adaptive Functioning.

Sex differences in restricted and repetitive behaviors (RRBs) in individuals with Autism Spectrum Disorder (ASD) have been explored with mixed findings. We aimed to investigate sex differences in RRBs through a specific measure—i.e., the Repetitive Behavior Scale Revised (RBS-R)—in a sample of preschool-age and school-age children with ASD. Additionally, we evaluated if RRBs were differently related to adaptive functioning within the male and the female age groups. A sample of 210 ASD individuals (3–18 years; 145 males, 65 females) underwent an in-depth assessment including a cognitive, adaptive functioning evaluation (i.e., the Adaptive Behavior Assessment System, Second Edition (ABAS-II)) and RRBs assessment (i.e., RBS-R). No significant sex differences on the RBS-R total score or any RBS-R subscale emerged. Within the group of older participants, RRBs were negatively associated with all adaptive skill domains independently from sex and age. Our results suggest a lack of sex differences in RRBs in our sample. Additionally, our findings highlight the possible negative impact of RRBs on adaptive skills in older individuals with autism, emphasizing the need for autistic individuals of both sexes to undergo an early intervention targeting RRBs, in order to improve their adaptive skills.

Speech Impairments Explain Unique Variance in Adaptive Behavior Skills in Young People With Down Syndrome.

Background Down syndrome (DS) is a disorder characterized by impairments in global cognitive abilities and adaptive function. In addition, individuals with DS demonstrate pronounced speech and language deficits. However, little is known about the linguistic correlates of impaired adaptive functioning in DS. Method Using the Adaptive Behavior Assessment System-Second Edition and the Children's Communication Checklist-Second Edition (CCC-2), this study investigated the unique variance in adaptive skills accounted for by speech and language impairments in individuals with DS (N = 29, M age = 13.46). Results Pearson correlations revealed that a composite of CCC-2 structural language scales, but not pragmatic language scales, was significantly correlated with the Adaptive Behavior Assessment System-Second Edition Global Adaptive Composite, Conceptual, and Practical domains. Further investigation utilizing hierarchical regression analyses identified only the Speech scale on the CCC-2 as contributing unique variance to the prediction of adaptive behavior scores in the Global Adaptive Composite, Conceptual, and Practical domains. Conclusion Speech impairments may serve as flags to identify children with DS who are at risk for adaptive behavior deficits and reinforce the need for speech-language therapies that focus on speech for these individuals. Supplemental Material https://doi.org/10.23641/asha.13231985.

Cognitive function and adaptive skills after a one-year trial of cannabidiol (CBD) in a pediatric sample with treatment-resistant epilepsy

ObjectiveCannabidiol (CBD) is a nonpsychoactive derivative of cannabis. Studies indicate that it is safe and effective in treating certain types of epilepsy. The present study examined the presence of adverse or beneficial cognitive or functional adaptive effects associated with CBD in the treatment of children, adolescents, and teenagers with treatment-resistant epilepsy (TRE) as part of an ongoing prospective, open-label safety study. MethodsParticipants (N = 38) between the age of 3 and 19 years with TRE were enrolled in an open-label study of a pharmaceutical formulation of CBD (Epidiolex®; GW Research Ltd.) as an add-on treatment. In addition to baseline physical, neurological, and laboratory testing, cognitive assessment was completed prior to initiating CBD and after one year, both using the NIH Toolbox Cognition Battery (NIHTB-CB). Many participants were unable to complete the NIHTB-CB because of the magnitude of their cognitive impairment (n = 24), and in these cases, the participant's caregiver was asked to complete the Adaptive Behavior Assessment System – Second Edition (ABAS-II) as a measure of functional adaptive skills. ResultsThere were no statistically significant changes in cognitive function, as measured by the NIHTB-CB, in those participants who were able to complete such testing, but there was a nonsignificant trend toward improvement in some cognitive domains. For participants who were unable to complete formal standardized cognitive testing because of the magnitude of their cognitive impairment, their functional adaptive skills, as measured by the ABAS-II, were unchanged after a one-year trial of CBD. SignificanceOur findings suggest that CBD, as an add-on drug for TRE in a pediatric sample, does not appear to cause adverse effects (AEs) involving cognition or adaptive function over one year of treatment.

The Development of the Social Functioning Scale for Patients with Parkinson's Disease.

Social functioning is crucial for the determinants of Parkinson's disease (PD) with dementia; however, there is no social functioning scale applicable to PD. This study aimed to develop a social functioning scale specific to PD (PDSFS) and provide a cut-off score to improve diagnosis accuracy. The items were developed through literature, interview patients, and PD expertise. After the pilot study, one hundred fifty-seven patients and 74 healthy participants were enrolled and completed the Mini-Mental State Examination, Clock Drawing Test, Activities of Daily Living, Neuropsychiatric Inventory, Adaptive Behavior Assessment System-Second Edition (ABAS-II) and part III of the Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS). The final PDSFS has 23 items. The exploratory factor analysis revealed three factors, including "Family Life, Hobbies and Self-Care", "Interpersonal Relationship and Recreational Leisure", and "Social Bond". The internal consistency coefficient was 0.883, and the test-retest reliability was 0.774, respectively. The total score of the PDSFS was significantly related to the total score of ABAS-II (r = 0.609, p < 0.001), and was not correlated with the third part of MDS-UPDRS (p = 0.736). A significant intergroup difference was found (p < 0.001), and the healthy controls had the highest PDSFS score, followed by non-demented PD and PD dementia. The optimal cut-off score for PD patients with dementia was 39 (sensitivity: 0.735; specificity: 0.857). PDSFS is a practical and psychometrically sound tool to access the social functioning of the PD population.

Sex Differences in Social Adaptive Function in Autism Spectrum Disorder and Attention-Deficit Hyperactivity Disorder.

Background: Social-communication difficulties, a hallmark of ASD, autism spectrum disorder (ASD) are often observed in attention – deficit/ hyperactivity disorder (ADHD), although are not part of its diagnostic criteria. Despite sex differences in the prevalence of ASD and ADHD, research examining how sex differences manifest in social and communication functions in these disorders remains limited, and findings are mixed. This study investigated potential sex differences with age in social adaptive function across these disorders, relative to controls. Method: One hundred fifteen youth with ASD, 172 youth with ADHD, and 63 typically developing controls (age range 7–13 years, 75% males) were recruited from the Province of Ontario Neurodevelopmental Disorder (POND) Network. Social adaptive function was assessed using the Adaptive Behavior Assessment System-Second Edition (ABAS-II). The proportions of adaptive behaviors present in each skill area were analyzed as a binomial outcome using logistic regression, controlling for age, and testing for an age-by-sex interaction. In an exploratory analysis, we examined the impact of controlling for core symptom severity on the sex effect. Results: Significant sex-by-age interactions were seen within ASD in the communication (p = 0.005), leisure (p = 0.003), and social skill areas (p < 0.0001). In all three areas, lower scores (indicating poorer function) were found in females compared to males at older ages despite females performing better at younger ages. There were significant differences in the sex-by-age interactions in the social and leisure domains between those with ASD and typically developing controls, with typically developing females showing better scores at older, compared to younger, ages. There were also significant differences in the sex-by-age interactions between ASD and ADHD on the social and leisure domains, as females with ADHD consistently scored higher on social skills than males across all ages, unlike those with ASD. Sex differences across age in the social domains for ADHD were similar to those in the typically developing group. Conclusion: Sex differences in social and communication skill areas were observed between ASD and ADHD, and typically developing controls, with females with ASD performing worse than males at older ages, despite an earlier advantage. These findings reinforce the need to take a developmental approach to understanding sex differences which may have diagnostic, prognostic, and treatment implications.

Moral reasoning and decision-making in adolescents who sustain traumatic brain injury

ABSTRACTObjective: Emerging evidence suggests that moral processes are disrupted by traumatic brain injury (TBI). The objective of this study was to explore moral reasoning (MR) and decision-making in adolescents with TBI, and to examine potential associations with global manifestations of social competence.Design: This retrospective, cross-sectional research design compared MR and decision-making in adolescents with mild TBI (n = 20), moderate-severe TBI (n = 23) and typically developing controls (n = 93).Methods: Participants completed a visual task of socio-moral reasoning (SoMoral) and the Index of Empathy for Children and Adolescents. Their parents completed questionnaires documenting their child’s behavior (Child Behavior Checklist) and adaptive functioning (Adaptive Behavior Assessment System-Second Edition).Main results: Adolescents with both mild and moderate-severe TBI displayed more immature MR than typically developing peers. Participants with TBI also provided fewer socially adapted decisions. Closer inspection revealed that this difference was apparent only in the mild TBI group. No significant group differences were observed for empathy, behavior or adaptive skills.Conclusions: Sustaining TBI appears to affect adolescents’ ability to provide mature moral justifications when faced with moral dilemmas representative of everyday social conflicts. These difficulties do not appear to be associated with behavior problems, reduced empathy, or adaptive functioning.

An Independent Investigation of the Utility of the Learning Disability Screening Questionnaire (LDSQ) within a Community Learning Disability Team.

The Learning Disability Screening Questionnaire (LDSQ) has been shown to have high sensitivity and specificity to identify those who are likely to meet intellectual disability diagnostic criteria (McKenzie, et al. ). However, there is no independent research to date to support these findings. An archival research design was used, utilizing data from diagnostic tools including the LDSQ, Wechsler Adult Intelligence assessments and Adaptive Behavior Assessment System Second Edition (ABAS-II) scores. Sensitivity and specificity values derived here were lower than those reported by (McKenzie, et al. ). Only IQ, not adaptive/social functioning, was found to be an accurate predictor of the LDSQ score. Results indicate limited validity in using(McKenzie, et al. ) proposed cut-off scores. The authors have expressed caution around using the LDSQ in isolation to identify those with an intellectual disability.

Correspondence between correctional staff and offender ratings of adaptive behavior.

Although several experts have raised concerns about using correctional officers as informants for adaptive behavior assessments, no studies have compared ratings from correctional officers to those from other informants. We compared Adaptive Behavior Assessment System-Second Edition (ABAS-II; Harrison & Oakland, 2003) scores assigned by correctional staff to those assigned by probationers (N = 56) residing in a community corrections facility. Correctional staff assigned markedly lower scores than did probationers on many ABAS-II scales (d = .59 to 1.41 for ABAS-II composite scores). Although none of the probationers qualified for a diagnosis of intellectual disability, 29% received a staff-report ABAS-II composite score that was more than 2 SDs below the normative sample mean, suggesting significant impairment. Correlations between ABAS-II and intelligence measure scores were lower than expected for both types of informants, although they were somewhat stronger for self-report. Lower staff-report scores were associated with higher levels of probationer-reported psychopathology and need for treatment. Overall, these findings highlight limitations of using correctional staff as informants for adaptive behavior assessments. (PsycINFO Database Record

ABAS-II Adaptive Profiles and Correlates in Samples of Children with HFASD or LFASD

This study was conducted to extend the research on the adaptive skills of 55 high-functioning children with autism spectrum disorder (HFASD) and 55 children with ASD and co-occurring intellectual disability (LFASD), ages 6–12, using the Adaptive Behavior Assessment System, Second Edition (ABAS-II). This study examined each group’s adaptive profile, compared adaptive functioning and cognitive ability, and assessed correlates (i.e., age, IQ) of adaptive skills. Results indicated children with HFASD demonstrated cognitive strengths and adaptive deficits, while children with LFASD demonstrated adaptive strengths in the areas of social and conceptual skills, and practical skills commensurate with estimated cognitive ability. Within the HFASD sample, age significantly, negatively correlated with adaptive social ability, while IQ was not significantly correlated with adaptive behavior. Conversely, in the LFASD sample, age did not correlate with adaptive ability, although IQ was significantly related to overall adaptive ability and all domains of the ABAS-II.

Left hemisphere structural connectivity abnormality in pediatric hydrocephalus patients following surgery

Neuroimaging research in surgically treated pediatric hydrocephalus patients remains challenging due to the artifact caused by programmable shunt. Our previous study has demonstrated significant alterations in the whole brain white matter structural connectivity based on diffusion tensor imaging (DTI) and graph theoretical analysis in children with hydrocephalus prior to surgery or in surgically treated children without programmable shunts. This study seeks to investigate the impact of brain injury on the topological features in the left hemisphere, contratelateral to the shunt placement, which will avoid the influence of shunt artifacts and makes further group comparisons feasible for children with programmable shunt valves. Three groups of children (34 in the control group, 12 in the 3-month post-surgery group, and 24 in the 12-month post-surgery group, age between 1 and 18 years) were included in the study. The structural connectivity data processing and analysis were performed based on DTI and graph theoretical analysis. Specific procedures were revised to include only left brain imaging data in normalization, parcellation, and fiber counting from DTI tractography. Our results showed that, when compared to controls, children with hydrocephalus in both the 3-month and 12-month post-surgery groups had significantly lower normalized clustering coefficient, lower small-worldness, and higher global efficiency (all p < 0.05, corrected). At a regional level, both patient groups showed significant alteration in one or more regional connectivity measures in a series of brain regions in the left hemisphere (8 and 10 regions in the 3-month post-surgery and the 12-month post-surgery group, respectively, all p < 0.05, corrected). No significant correlation was found between any of the global or regional measures and the contemporaneous neuropsychological outcomes [the General Adaptive Composite (GAC) from the Adaptive Behavior Assessment System, Second Edition (ABAS-II)]. However, one global network measure (global efficiency) and two regional network measures in the insula (local efficiency and between centrality) tested at 3-month post-surgery were found to correlate with GAC score tested at 12-month post-surgery with statistical significance (all p < 0.05, corrected). Our data showed that the structural connectivity analysis based on DTI and graph theory was sensitive in detecting both global and regional network abnormality when the analysis was conducted in the left hemisphere only. This approach provides a new avenue enabling the application of advanced neuroimaging analysis methods in quantifying brain damage in children with hydrocephalus surgically treated with programmable shunts.

Adaptive Behaviour Assessment System: Indigenous Australian Adaptation Model (ABAS: IAAM)

The study objectives were to develop, trial and evaluate a cross-cultural adaptation of the Adaptive Behavior Assessment System-Second Edition Teacher Form (ABAS-II TF) ages 5-21 for use with Indigenous Australian students ages 5-14. This study introduced a multiphase mixed-method design with semi-structured and informal interviews, school observations, and psychometric analyses of existing and new Northern Territory student data. It trained teachers to undertake psychological testing of Indigenous students. The cross-cultural adaptation was performed according to the internationally recommended methodology, using forward translation, back-translation, revision by an expert committee, and a pilot trial. The reliability was estimated through internal consistency and standard error of measurement. The validity was assessed through test content, response process, internal structure, internal consistency, age group differences, inter-correlations among adaptive domains, and correlations with other variables. A clinical validity study tested students‟ performance with and without special education needs. The results indicated good internal consistency for the adaptive domains and composite score (Social Adaptive Domain, alpha=.92; Conceptual Adaptive Domain, alpha=.94; Personal Adaptive Domain, alpha=.78; General Adaptive Composite, alpha=.94). The standard error of measurement indicated high test accuracy. Content -, construct -, and concurrent validity were measured. Concurrent validity evaluated the mean scores of students with and without special education needs, indicated significant variance (p&lt;0.001). The data showed that the cross-cultural adaptation process was successful, and the adapted instrument demonstrated satisfactory psychometric properties, making it valid and reliable to use in the Indigenous context.

Gastrostomy Tube Feeding after Neonatal Complex Cardiac Surgery Identifies the Need for Early Developmental Intervention

To compare the proportion of developmental delay in early complex cardiac surgery (CCS) survivors with and without gastrostomy tube feeding (GTF). To explore acute care predictors of GTF that might help improve care in CCS survivors. This comparison study of 2 groups within an inception cohort included 334 CCS survivors after cardiopulmonary bypass at ≤6 weeks of age (2005-2012) who did not require extracorporeal membrane oxygenation or heart transplantation. Children were assessed at 21 ± 3 months with the Bayley Scales of Infant and Toddler Development-Third Edition and the Adaptive Behavior Assessment System-Second Edition: general adaptive composite score. Delay was determined by scores >2 SD below mean. The χ(2) test compared groups. Predictors of GTF were analyzed using multiple logistic regression analysis, results expressed as OR with 95% CI. Of the survivors, 67/334 (20%) had GTF any time before the 21-month assessment. Developmental delays in children with GTF were cognitive in 16 (24%), motor in 18 (27%), language in 24 (36%) vs without GTF in 7 (3%), 8 (3%), and 32 (12%), respectively (P < .001). Gastrostomy group had almost 8 times the number of children delayed on the general adaptive composite score. Independent OR for GTF are presence of a chromosomal abnormality, OR 4.6 (95% CI 1.8, 12.0) (P = .002), single ventricle anatomy, OR 3.4 (95% CI 1.7, 6.8) (P < .001), total postoperative days of open sternum, OR 1.15 (95% CI 1.1, 1.3) (P = .031), and total number of hospital days at CCS, OR 1.03 (95% CI 1.1, 1.04) (P = .002). GTF identifies CCS survivors at risk for delay, who would benefit from early developmental intervention. The described mostly nonmodifiable predictors may guide counseling of these children's families.

Semantic Verbal Fluency Pattern, Dementia Rating Scores and Adaptive Behavior Correlate With Plasma Aβ42 Concentrations in Down Syndrome Young Adults.

Down syndrome (DS) is an intellectual disability (ID) disorder in which language and specifically, verbal fluency are strongly impaired domains; nearly all adults show neuropathology of Alzheimer’s disease (AD), including amyloid deposition by their fifth decade of life. In the general population, verbal fluency deficits are considered a strong AD predictor being the semantic verbal fluency task (SVFT) a useful tool for enhancing early diagnostic. However, there is a lack of information about the association between the semantic verbal fluency pattern (SVFP) and the biological amyloidosis markers in DS. In the current study, we used the SVFT in young adults with DS to characterize their SVFP, assessing total generated words, clustering, and switching. We then explored its association with early indicators of dementia, adaptive behavior and amyloidosis biomarkers, using the Dementia Questionnaire for Persons with Intellectual Disability (DMR), the Adaptive Behavior Assessment System-Second Edition (ABAS-II), and plasma levels of Aβ peptides (Aβ40 and Aβ42), as a potent biomarker of AD. In DS, worse performance in SVFT and poorer communication skills were associated with higher plasma Aβ42 concentrations, a higher DMR score and impaired communication skills (ABAS–II). The total word production and switching ability in SVFT were good indicators of plasma Aβ42 concentration. In conclusion, we propose the SVFT as a good screening test for early detection of dementia and amyloidosis in young adults with DS.

Fetal antiepileptic drug exposure: Adaptive and emotional/behavioral functioning at age 6 years

The Neurodevelopmental Effects of Antiepileptic Drugs (NEAD) study is a prospective observational multicenter study in the USA and UK, which enrolled pregnant women with epilepsy on antiepileptic drug (AED) monotherapy from 1999 to 2004. The study aimed to determine if differential long-term neurodevelopmental effects exist across four commonly used AEDs (carbamazepine, lamotrigine, phenytoin, and valproate). In this report, we examine fetal AED exposure effects on adaptive and emotional/behavioral functioning at 6years of age in 195 children (including three sets of twins) whose parent (in most cases, the mother) completed at least one of the rating scales. Adjusted mean scores for the four AED groups were in the low average to average range for parent ratings of adaptive functioning on the Adaptive Behavior Assessment System—Second Edition (ABAS-II) and for parent and teacher ratings of emotional/behavioral functioning on the Behavior Assessment System for Children (BASC). However, children whose mothers took valproate during pregnancy had significantly lower General Adaptive Composite scores than the lamotrigine and phenytoin groups. Further, a significant dose-related performance decline in parental ratings of adaptive functioning was seen for both valproate and phenytoin. Children whose mothers took valproate were also rated by their parents as exhibiting significantly more atypical behaviors and inattention than those in the lamotrigine and phenytoin groups. Based upon BASC parent and teacher ratings of attention span and hyperactivity, children of mothers who took valproate during their pregnancy were at a significantly greater risk for a diagnosis of ADHD. The increased likelihood of difficulty with adaptive functioning and ADHD with fetal valproate exposure should be communicated to women with epilepsy who require antiepileptic medication. Finally, additional research is needed to confirm these findings in larger prospective study samples, examine potential risks associated with other AEDs, better define the risks to the neonate that are associated with AEDs for treatment of seizures, and understand the underlying mechanisms of adverse AED effects on the immature brain.