T. Andrew Zabel

Parent and self-report ratings of executive function in adolescents with myelomeningocele and hydrocephalus.

Parent and self-report ratings were obtained for 28 adolescents with myelomeningocele and congenital hydrocephalus using the Behavior Assessment System for Children (BASC; Reynolds & Kamphaus, 1998a, 1998b) and the Behavior Rating Inventory of Executive Function (BRIEF; Gioia, Isquith, Guy, & Kenworthy, 2000; Guy, Gioia, & Isquith, 1998). Parents rated their children as having significantly more problems, compared to published norms, on the BRIEF but not the BASC. Adolescents rated themselves as having significantly more problems, compared to parent ratings, on scales comprising the BRIEF Behavioral Regulation Index; but not on common scales of the BASC. Parents also reported more problems on the BRIEF Global Executive Composite than on all three primary BASC indices, and more problems on the BRIEF Metacognition Index than on the BRIEF Behavioral Regulation Index. We conclude that the BRIEF captures salient executive dysfunction among individuals with myelomeningocele and hydrocephalus, and may be useful in identifying needs for intervention that might not be identified by broad-band behavior rating scales alone.

Factor Structure of a Sluggish Cognitive Tempo Scale in Clinically-Referred Children

Abstract“Sluggish cognitive tempo” (SCT) is a construct hypothesized to describe a constellation of behaviors that includes daydreaming, lethargy, drowsiness, difficulty sustaining attention, and underactivity. Although the construct has been inconsistently defined, measures of SCT have shown associations with symptoms of attention-deficit/hyperactivity disorder (ADHD), particularly inattention. Thus, better characterization of SCT symptoms may help to better predict specific areas of functional difficulty in children with ADHD. The present study examined psychometric characteristics of a recently developed 14-item scale of SCT (Penny et al., Psychological Assessment 21:380–389, 2009), completed by teachers on children referred for outpatient neuropsychological assessment. Exploratory factor analysis identified three factors in the clinical sample: Sleepy/Sluggish, Slow/Daydreamy, and Low Initiation/Persistence. Additionally, SCT symptoms, especially those loading on the Sleepy/Sluggish and Slow/Daydreamy factors, correlated more strongly with inattentive than with hyperactive/impulsive symptoms, while Low Initiation/Persistence symptoms added significant unique variance (over and above symptoms of inattention) to the predictions of impairment in academic progress.

Correlates of Depressive and Anxiety Symptoms in Young Adults with Spina Bifida

Objective Based on social ecological theory, this study was designed to examine the unique relationships between multi-level ecological factors and psychological symptoms in young adults with spina bifida (SB). Method A sample of 61 individuals with SB, 18–25 years of age, completed standardized self-report measures of attitude toward SB, satisfaction with family functioning, Chronic Care Model (CCM) services, and depressive and anxiety symptoms. A chart review yielded SB clinical data. Results High rates of depressive and anxiety symptoms were found. Hierarchical regression analysis identified the proximal individual (attitude toward SB) and family (satisfaction with family functioning) factors as more strongly related to depressive symptoms than the distal healthcare system factor (CCM services). Self-reported pain was the only ecological factor associated with anxiety symptoms. Conclusions Study findings provide a potential foundation for multi-factor screening of young adults with SB at risk for psychological symptoms.

Aspirin Use in Sturge-Weber Syndrome: Side Effects and Clinical Outcomes

Sturge-Weber syndrome is a neurocutaneous disorder with skin, eye, and brain involvement. Prior series suggest about 50% of patients have seizures/neurodeterioration. Low-dose (3-5 mg/kg/d) aspirin use in this population is controversial. This study further addresses the side effects and outcomes of low-dose aspirin usage in Sturge-Weber syndrome. Fifty-eight subjects on aspirin with brain involvement were analyzed in a retrospective chart review. Charts were evaluated for brain involvement, age at first seizure, and side effects. Subjects’ clinical stability was compared using neurologic scores. The majority of subjects had neurologic scores reflecting reasonable seizure control (91%), none or mild hemiparesis (57%), no vision impairment (71%), and none or mild cognitive impairment (80%). Forty-nine reported no significant side effects, and 9 reported either allergic reaction or minimal to significant bleeding on aspirin. This cohort’s clinical experience adds significant support for low-dose aspirin use to optimize neurodevelopmental outcome in Sturge-Weber syndrome with minimal side effects.

Survey of Aspirin Use in Sturge-Weber Syndrome

Sturge-Weber syndrome is a rare congenital disorder. Seizures, stroke-like episodes, glaucoma, headache, and developmental delay are frequently associated features. An Internet-based questionnaire was designed to assess the frequency of use, effectiveness, and safety of aspirin treatment in Sturge-Weber syndrome. Thirty-four of 98 subjects who completed the survey reported having used aspirin. The mean number of reported stroke-like episodes was reduced from 1.1 to 0.3 per month in the year after starting aspirin (n = 26, p = .014). The median number of seizures was significantly reduced from 3 to 1 episodes per month (n = 21, p = .002). Thirty-nine percent of subjects reported a history of complications (predominantly increased bruising or gum/nose bleeding) while on aspirin; however, none reported discontinuing aspirin because of side effects. Our study showed a significant relative reduction in both self-reported seizure frequency and stroke-like episodes after starting aspirin. It also suggests that low-dose aspirin can be safely used in these patients.

AGE-RELATED DIFFERENCES IN EXECUTIVE FUNCTION AMONG CHILDREN WITH SPINA BIFIDA/HYDROCEPHALUS BASED ON PARENT BEHAVIOR RATINGS

Previous research has suggested that adolescents with myelomeningocele and shunted hydrocephalus (MMH) have difficulties with aspects of executive functioning and, in turn, with functional independence. There is little research, however, examining patterns of executive functioning across adolescence in this population. The goal of this cross-sectional study was to examine parent ratings of executive function in children with MMH and in typically developing peers across late childhood and adolescence. Parents of 36 individuals with MMH and 35 typically developing peers, ages 10 to 18 years, completed the Behavior Rating Inventory of Executive Function (BRIEF). The BRIEF is organized into eight scales and two primary indices—Metacognition (MCI) and Behavioral Regulation (BRI). As a whole, the children with MMH had significantly higher BRIEF T-scores, as well as a higher prevalence of clinically significant T-scores across subscales, particularly those representing cognitive control. Effects of group, age, and age-by-group interactions on the mean raw scores of the MCI and BRI were examined using regression analyses. There were significant group effects (p <. 05) for both the BRI and MCI, with the controls having significantly lower mean ratings than the MMH group. There was also a significant contribution of age-by-group interaction on the BRI (p <. 05). Although mean raw scores on the BRI for the MMH group remained stable across ages, mean raw scores in the control group decreased as age increased. Thus, healthy children have age-related improvements in executive control behaviors across adolescence, particularly behavioral control, while children with MMH demonstrate no age-related improvements in parent reported executive behaviors across adolescence. Therefore, children with MMH may continue to require targeted interventions and modifications to address executive dysfunction into young adulthood in order to promote functional independence.

Interrelationships of sex, level of lesion, and transition outcomes among young adults with myelomeningocele

Aim  To advance understanding of the interrelationships of sex, level of lesion (LOL), self‐management, community integration (employment, independent living), and quality of life (QOL) in young adults with myelomeningocele.

Reliability concerns in the repeated computerized assessment of attention in children.

Assessment of attentional processes via computerized assessment is frequently used to quantify intra-individual cognitive improvement or decline in response to treatment. However, assessment of intra-individual change is highly dependent on sufficient test reliability. We examined the test–retest reliability of selected variables from one popular computerized continuous performance test (CPT)—i.e., the Conners’ CPT – Second Edition (CPT-II). Participants were 39 healthy children (20 girls) ages 6–18 without intellectual impairment (mean PPVT-III SS = 102.6), LD, or psychiatric disorders (DICA-IV). Test–retest reliability over the 3–8 month interval (mean = 6 months) was acceptable (Intraclass Correlations [ICC] =.82 to.92) on comparison measures (Beery Test of Visual Perception, WISC-IV Block Design, PPVT-III). In contrast, test–retest reliability was only modest for CPT-II raw scores (ICCs ranging from.62 to.82) and T-scores (ICCs ranging from.33 to.65) for variables of interest (Omissions, Commissions, Variability, Hit Reaction Time, and Attentiveness). Using test–retest reliability information published in the CPT-II manual, 90% confidence intervals based on reliable change index (RCI) methodology were constructed to examine the significance of test–retest difference/change scores. Of the participants in this sample of typically developing youth, 30% generated intra-individual changes in T-scores on the Omissions and Attentiveness variables that exceeded the 90% confidence intervals and qualified as “statistically rare” changes in score. These results suggest a considerable degree of normal variability in CPT-II test scores over extended test–retest intervals, and suggest a need for caution when interpreting test score changes in neurologically unstable clinical populations.

Serial Neuropsychological Assessment and Evidence of Shunt Malfunction in Spina Bifida: A Longitudinal Case Study

Myelomeningocele is often accompanied by hydrocephalus (MMH), making it a potentially unstable neurological condition requiring shunt placement and possible revisions. Serial neuropsychological assessment is an important tool in monitoring children with MMH, as cognitive changes can indicate shunt malfunction and hydrocephalus. We present the case of a girl with MMH who had five neuropsychological assessments (ages 5, 7, 11, 12, and 14). Despite a lack of overt neurological symptoms or report of behavioral decline, testing at age 11 revealed decline in multiple neurobehavioral domains, and imaging at that time showed increased hydrocephalus, requiring shunt revision. Subsequent neuropsychological assessment conducted after a 2-year period of medical stability showed improvement and/or a return to baseline levels in some skill areas (i.e., working memory, verbal memory, visuomotor integration, and sustained attention), yet more lasting impairments in others (i.e., Verbal IQ, processing speed, organization, and response inhibition). These lasting cognitive deficits potentially impact independent completion of complex medical self-care tasks. This pattern of recovery highlights vulnerability of brain systems supporting executive functions in children with hydrocephalus and shunt failure.

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.