[Formula: see text]Intellectual and adaptive functioning in Sturge-Weber Syndrome.

The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome (SWS). A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. Thirty-two percent of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or nonimpaired adaptive and intellectual functioning and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.

To ID or not to ID? Changes in classification rates of intellectual disability using DSM-5.

The Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition (DSM-5) diagnostic criteria for intellectual disability (ID) include a change to the definition of adaptive impairment. New criteria require impairment in one adaptive domain rather than two or more skill areas. The authors examined the diagnostic implications of using a popular adaptive skill inventory, the Adaptive Behavior Assessment System-Second Edition, with 884 clinically referred children (ages 6-16). One hundred sixty-six children met DSM-IV-TR criteria for ID; significantly fewer (n  =  151, p  =  .001) met ID criteria under DSM-5 (9% decrease). Implementation of DSM-5 criteria for ID may substantively change the rate of ID diagnosis. These findings highlight the need for a combination of psychometric assessment and clinical judgment when implementing the adaptive deficits component of the DSM-5 criteria for ID diagnosis.

Classification of intellectual disability using the Wechsler Intelligence Scale for Children: Full Scale IQ or General Abilities Index?

AIM: We examined the implications of using the Full Scale IQ (FSIQ) versus the General Abilities Index (GAI) for determination of intellectual disability using the Wechsler Intelligence Scales for Children, fourth edition (WISC-IV). METHOD: Children referred for neuropsychological assessment (543 males, 290 females; mean age 10y 5mo, SD 2y 9mo, range 6-16y) were administered the WISC-IV and the Adaptive Behavior Assessment System, second edition (ABAS-II). RESULTS: GAI and FSIQ were highly correlated; however, fewer children were identified as having intellectual disability using GAI (n=159) than when using FSIQ (n=196). Although the 44 children classified as having intellectual disability based upon FSIQ (but not GAI) had significantly higher adaptive functioning scores than those meeting intellectual disability criteria based upon both FSIQ and GAI, mean adaptive scores still fell within the impaired range. FSIQ and GAI were comparable in predicting impairments in adaptive functioning. INTERPRETATION: Using GAI rather than FSIQ in intellectual disability diagnostic decision-making resulted in fewer individuals being diagnosed with intellectual disability; however, the mean GAI of the disqualified individuals was at the upper end of criteria for intellectual impairment (standard score 75), and these individuals remained adaptively impaired. As GAI and FSIQ were similarly predictive of overall adaptive functioning, the use of GAI for intellectual disability diagnostic decision-making may be of limited value.

More than intelligence: distinct cognitive/behavioral clusters linked to adaptive dysfunction in children.

Impairments in adaptive functioning are frequently associated with intellectual disability (ID); however, adaptive dysfunction can be seen in many individuals with a variety of neurological conditions without ID. The extent to which other variables may be associated with adaptive dysfunction is unclear. In a mixed clinical sample of children (n = 348) consecutively referred for neuropsychological evaluation, the majority were rated as showing weak adaptive skills (ABAS-II, >1 SD below the mean; 71%), with a substantial proportion evidencing frank impairment (>2 SD below the mean, 45%). We examined patterns of scores on measures of intelligence (WISC-IV) and behavioral/affective dysregulation (BRIEF and BASC-2). Using hierarchical cluster analysis, a four cluster model yielded the most appropriate fit and adaptive functioning was subsequently examined across clusters. As expected, adaptive functioning was most intact in the cluster characterized by average IQ and minimal behavioral dysregulation. Other clusters were marked by adaptive dysfunction and distinguished by sub-average intellectual functioning and varying behavioral/emotional dysregulation. In contrast to traditional views associating low IQ with adaptive dysfunction, adaptive impairment was comparable between the cluster characterized by low intelligence and the cluster with average intelligence but significant behavioral dysregulation. These data suggest that adaptive functioning should be considered across various cognitive/behavioral conditions.

Sensitivity of the BASC-2 Adaptive Skills Composite in detecting adaptive impairment in a clinically referred sample of children and adolescents.

The Behavior Assessment Scale for Children (BASC-2) is widely used to assess for internalizing/externalizing behavior problems; however, the role of its adaptive scales is not well established. This study examined the sensitivity of the parent version of the BASC-2 Adaptive Skills Composite (ASC) in identifying children with impairments on a more comprehensive measure of adaptive functioning, the Adaptive Behavior Assessment System (ABAS-II). Participants were referred for neuropsychological assessment (N = 1884, M age = 10.96 years, SD = 3.50, 65% male). The ASC, rather than individual BASC-2 adaptive scales, showed the strongest correlations with the ABAS-II General Adaptive Composite (GAC) and domains. Receiver operating characteristic analysis showed that the BASC-2 ASC discriminated children with impaired adaptive functioning on the ABAS-II (≥ -2 SD) from those without impairment (area under curve range = .86[social domain] to .78[practical domain]); however, sensitivity was inadequate at the typical -2 SD cut-off (48%). A -1 SD cut-off on the BASC-2 (T-score ≤ 40) was more appropriate for predicting impairment on the ABAS-II (89%). Among children with impaired IQ, the ABAS-II GAC was impaired in 69% compared to only 36% on the ASC. Findings have implications for the diagnosis of Intellectual Disability and for quantifying the functional impact of other diagnoses, particularly with the release of DSM-5.

Active seizures are associated with reduced adaptive functioning in children with epilepsy.

Children with epilepsy are at risk of suboptimal adaptive functioning. Research has not yet established how specific seizure and treatment variables may affect adaptive functioning, which would allow clinicians to better identify at-risk children. This study sought to determine the seizure and treatment variables predictive of adaptive functioning. Forty-six children with epilepsy participated in this study. Using multiple regression, active seizures (one or more seizures in the prior year) significantly predicted scores on the General Adaptive Composite of the Adaptive Behavior Assessment System-II. The active seizures variable uniquely explained 19% of the variance in adaptive functioning, with children with active seizures demonstrating significantly poorer adaptive functioning. The number of current AEDs, past AEDs, seizure types, age at seizure onset, and temporal lobectomy were not significant predictors. Post hoc analyses that divided the active seizures group according to seizure frequency in the prior month did not find significant differences in adaptive functioning. The results of this study suggest that children with seizures that are not fully controlled are at greater risk of suboptimal adaptive functioning.

Parent report of attention problems predicts later adaptive functioning in children with brain tumors.

Children with brain tumors are at risk for psychological and behavioral difficulties. This study examined the ability of parent report of attention problems, withdrawal, anxiety, and depression, as well as IQ, to predict later adaptive functioning in 42 children treated for brain tumors. Age at diagnosis, SES, gender, and scores on the Neurological Predictor Scale (NPS) also were examined as predictors. Parent report of attention problems, SES, and NPS were significant predictors of later adaptive functioning across domains. This finding highlights the ability of parent report of attention problems to predict later adaptive functioning in children treated for brain tumors.

Cognitive predictors of adaptive functioning vary according to pediatric brain tumor location.

This archival study sought to determine if the relationship between cognitive and adaptive abilities varied according to brain tumor location. Participants were 36 children treated for brain tumors. The best cognitive predictors of adaptive functioning were hypothesized to be attention span within the cerebellar group and verbal memory within the third ventricle group. Auditory attention span significantly predicted communication skills for the cerebellar group, whereas verbal memory significantly predicted socialization skills for the third ventricle group. These findings suggest that cognitive predictors vary according to tumor location, and highlight the need for more research examining adaptive functioning and its correlates.

Attention mediates radiation's impact on daily living skills in children treated for brain tumors.

BACKGROUND: Radiotherapy is associated with an increased survival rate in children with brain tumors, but also with cognitive decline. This study examined the time-dependent effects of radiation treatment on adaptive functioning in children with brain tumors. The potentially mediating effects of attention span (Trial 1 of the Rey Auditory Verbal Learning Test [RAVLT]) assessed within 7 years of diagnosis were explored. PROCEDURE: Twenty-two children treated with cranial radiation for third ventricle or cerebellar tumors were included in this archival study. The mean age at diagnosis was 7.62 years (SD = 4.78) and 10.16 years (SD = 3.83) at evaluation. We examined the extent to which auditory attention span was able to mediate the relationship between time elapsed since the initiation of radiation treatment (M = 2.43 years; SD = 2.37) and adaptive functioning (Vineland Adaptive Behavior Scales [VABS]). RESULTS: Attention span was found to mediate the relationship between time since the initiation of radiation and daily living skills. These findings were shown to be specific to attention and not a reflection of generalized neuropsychological decline, nor were they a result of increasing time since diagnosis in and of itself. CONCLUSIONS: The results of this study suggest that time since radiation may directly decrease attention and poor attention in turn may be associated with lower adaptive functioning on tasks of daily living.