Patterns of WISC-V Performance in Children with Congenital Heart Disease.

Congenital heart disease (CHD) is one of the most common congenital birth defects. As surgical and interventional techniques have improved, the mortality has been greatly reduced and the focus has shifted to quality of life and long-term outcomes. The impact of CHD on development and cognition is becoming increasingly recognized. However, more research is needed to understand how children with CHD perform across various cognitive and intellectual domains. This study explored the performance of children with CHD on the newest version of the Wechsler Intelligence Scale for Children compared to normative controls. Children with CHD performed more poorly than normal controls across all indices and most subtests with large effect sizes. Additionally, we explored the patterns of impairment across indices and subtests, as well as the relationships between heard disease variables and WISC-V performance. Block design, Digit Span, and Similarities were the most commonly impaired scores in children with CHD, while Symbol Search, Picture Span, Figure Weights, and Vocabulary were least likely to be impaired.

Neuropsychological, behavioral, and quality-of-life outcomes in children and adolescents with sickle cell disease treated with nonmyeloablative matched sibling donor hematopoietic cell transplantation: A case series.

BACKGROUND/OBJECTIVES: Despite advances in the treatment of sickle cell disease (SCD), cerebrovascular and cognitive insults can have lifelong consequences. Hematopoietic cell transplantation (HCT) is an established curative therapy, and recent studies have demonstrated efficacy with reduced toxicity nonmyeloablative (NMA) regimens, but little is known about neuropsychological outcomes. The objective of this study was to describe neuropsychological, behavioral, and quality-of-life outcomes with medical correlates in children with SCD who received an NMA matched sibling donor (MSD) HCT. DESIGN/METHODS: Retrospective cohort analysis of nine recipients with hemoglobin SS SCD who underwent MSD HCT using the National Institutes of Health (NIH) NMA protocol. RESULTS: Mean full-scale intellectual functioning (FSIQ) was average pre-HCT (FSIQ = 92.1, SD 9.0; n = 8) and 2 years post-HCT (mean FSIQ = 96.6; SD 11.1; N = 9). Neuropsychological functioning was largely average across all cognitive domains, and no pre/post-HCT differences were found to be statistically significant given the small sample size. However, effect sizes revealed moderate improvements in processing speed (Cohen's d = .72) and verbal memory (Cohen's d = .60) post-HCT, and declines in measures of attention (Cohen's d = -.54) and fine motor speed and dexterity (Cohen's d = -.94). Parents endorsed better quality of life (Cohen's d = .91), less impact of SCD on their family, and less worry about their child's future (Cohen's d = 1.44). CONCLUSION: Neuropsychological functioning in a sample of children and adolescents treated uniformly with NMA MSD HCT remained stable or improved in most cognitive domains, and improvements in quality of life and family functioning were observed.

Fine motor impairment in children with epilepsy: Relations with seizure severity and lateralizing value.

Motor skill deficits are common in epilepsy. The Grooved Pegboard Test (GPT) is the most commonly used fine motor task and is included in the NIH Common Data Elements Battery for the assessment of epilepsy. However, there are limited data on its utility in children and adolescents. The present study investigated the effectiveness of this task in children and adolescents with epilepsy clinically referred for neuropsychological evaluation in a tertiary medical center. Two hundred and two children and adolescents (ages 6-16, 104 males, 98 females) completed the GPT. Base rates of impairment were calculated, correlational analyses determined relations with clinical variables, and ANOVAs and t-tests assessed for differences by seizure type, gender, and lateralized deficits in those with lateralized focal epilepsy. The GPT was sensitive to fine motor impairment in these children and adolescents, with over 60% having impaired performances. Further, performance was significantly correlated with IQ, age of epilepsy onset, number of medications, and seizure frequency. At the group level, those with lateralized focal epilepsy did not show significant differences between left and right hands, though the GPT correctly lateralized 63% of those with large between-hand performance disparities (i.e., one standard deviation or greater). In sum, the GPT is sensitive to fine motor deficits in pediatric epilepsy and is related to known epilepsy severity factors. However, the ability of the task to lateralize epilepsy onset is not robust.

Emotional functioning in pediatric epilepsy: Evidence of greater externalizing behavior with left hemisphere onset.

Though it is wellknown that psychiatric concerns are common in children with epilepsy, factors predicting such problems are not well understood. The present investigation studied rates of parent-reported psychological concerns in clinically referred children with epilepsy. Further, it investigated differences in psychological distress across epilepsy subtypes (i.e., focal, generalized, mixed), relationships with epilepsy severity variables, gender, and lateralization of seizure foci. The parents of 170 children and adolescents (ages 6-18 years, 78 girls, 92 boys) completed the Child Behavior Checklist (CBCL). Scale elevation frequencies (T-scores ≥ 65) were calculated and Chi square analyses examined rates of elevations between epilepsy groups. Internalizing problems (32.4%) were more common than externalizing problems (17.1%) for the sample, with attention problems being the most common concern across all epilepsy types (48.8%). While there were no significant relationships between epilepsy severity variables and CBCL broadband scales, the Total Problems scale was inversely related to intellectual functioning (r = -0.174, p = 0.023). Rates of anxiety and depression did not differ across epilepsy subtypes and no gender differences were found. Those with left-sided epilepsy had higher rates of externalizing problems (33.2%) than those with right (14.0%; χ2[1, 88] = 4.55, p = 0.03), with rule-breaking behaviors (15.4%) being more common in left-hemisphere epilepsy (15.4% versus 2.3%; χ2[1,88] = 4.66, p = 0.03). In summary, while no significant differences were found across epilepsy groups, the current study adds to the literature regarding lateralization effects and mood/behavior, with more externalizing problems in those with left hemisphere epilepsy.

Memory in children with epilepsy: Utility of the WRAML-2 in generalized and focal epilepsy syndromes.

The material-specific model for memory impairment predicts that verbal memory deficits are seen with left temporal seizures, and visual memory deficits are seen with right temporal seizures (Henkin et al., 2005). In pediatric epilepsy, seizure pathology has not always yielded the expected material-specific memory profiles. This study used the Wide Range Assessment of Memory and Learning-Second Edition (WRAML-2) to assess memory functioning among pediatric patients with epilepsy. The WRAML-2 was administered to 180 youth with epilepsy during their neuropsychological evaluations. Memory and recognition scores correlated significantly with epilepsy severity variables. There were no significant differences in verbal and visual memory and recognition index scores among patients with generalized epilepsy or among those with lateralized or localized electroencephalography (EEG) patterns and lesions on imaging. However, clinically meaningful verbal versus visual discrepancy scores were significantly related to lateralized abnormalities on EEG and magnetic resonance imaging (MRI) results. Most patients with right hemisphere pathology showed the expected material-specific visual memory deficits, while fewer than 15% of the left hemisphere cases showed the expected verbal memory deficits. Over one-third of those with identified left-sided pathology showed clinically significant deficits in visual memory. Findings are incongruent with the material-specific memory model and reflect the fact that early developmental neurological insults can lead to functional reorganization/crowding effects in children with left hemisphere epilepsy. On exploratory analyses, there were no significant differences in discrepancy scores among participants with left, right, and bilateral languages on Wada and functional MRI (fMRI). However, those with right and bilateral language dominance were more likely to show discrepancies that were incongruent with the material-specific model.

Measuring executive functioning with the Multidimensional Everyday Memory Ratings for Youth (MEMRY): concurrent validity with the BRIEF-2 in a large neurological and medical sample.

Executive skills are critical cognitive skills for everyday functioning in children; accurate measurement using validated tools is thus important. The purpose of this study was to examine concurrent validity between the MEMRY Executive/Working Memory scale and the BRIEF2. Participants included a large pediatric clinical sample who completed parent (n = 567), teacher (n = 148), and self-report (n = 88) scales. All correlations were significant between the MEMRY Executive/Working Memory and the BRIEF2 Global Executive Composite, Cognitive Regulation Index, and Working Memory scale (all r's > .80). Classification agreement metrics ranged from fair to excellent. This study provides evidence of strong concurrent validity of the MEMRY Executive/Working Memory scale as a brief, useful tool for assessing executive functioning using parent, teacher, and self-report versions.

Parent ratings on the MEMRY questionnaire predict children's academic performance.

Given the high rates of learning challenges in children with medical conditions, efficient and reliable screening methods are crucial. This study examined whether parent report of daily learning and memory predicts academic achievement in youth. Parents of 213 youth (aged 6-18) with varied medical diagnoses completed the Multidimensional Everyday Memory Ratings for Youth (MEMRY), and youth completed subtests from the Wechsler Individual Achievement Test-Third Edition (WIAT-III) as part of a comprehensive assessment. All scales of the MEMRY (Learning, Daily Memory, Executive/Working Memory) correlated significantly with WIAT-III Spelling, Word Reading, and Numerical Operations, while only the MEMRY Learning subscale correlated significantly with WIAT-III Pseudoword Decoding. Regression analyses indicated that MEMRY Learning significantly predicted WIAT-III Word Reading and Spelling, while both the MEMRY Learning and MEMRY Daily Memory scales significantly predicted WIAT-III Numerical Operations. When Full Scale IQ was entered into the models first, the MEMRY Learning subscale accounted for an additional 4% of variance in WIAT-III Word Reading and 7% of variance in WIAT-III Spelling, but did not account for additional variance in WIAT-III Numerical Operations or Pseudoword Decoding. Analyses in a subset of children with broadly normal intellectual functioning demonstrated very similar results, with even higher variance in academic testing accounted for by the MEMRY. In sum, the MEMRY questionnaire may serve as an efficient screen to identify children at risk for reading, spelling, and math deficits.

New Child and Adolescent Memory Profile Embedded Performance Validity Test.

OBJECTIVE: It is essential to interpret performance validity tests (PVTs) that are well-established and have strong psychometrics. This study evaluated the Child and Adolescent Memory Profile (ChAMP) Validity Indicator (VI) using a pediatric sample with traumatic brain injury (TBI). METHOD: A cross-sectional sample of N = 110 youth (mean age = 15.1 years, standard deviation [SD] = 2.4 range = 8-18) on average 32.7 weeks (SD = 40.9) post TBI (71.8% mild/concussion; 3.6% complicated mild; 24.6% moderate-to-severe) were administered the ChAMP and two stand-alone PVTs. Criterion for valid performance was scores above cutoffs on both PVTs; criterion for invalid performance was scores below cutoffs on both PVTs. Classification statistics were used to evaluate the existing ChAMP VI and establish a new VI cutoff score if needed. RESULTS: There were no significant differences in demographics or time since injury between those deemed valid (n = 96) or invalid (n = 14), but all ChAMP scores were significantly lower in those deemed invalid. The original ChAMP VI cutoff score was highly specific (no false positives) but also highly insensitive (sensitivity [SN] = .07, specificity [SP] = 1.0). Based on area under the curve (AUC) analysis (0.94), a new cutoff score was established using the sum of scaled scores (VI-SS). A ChAMP VI-SS score of 32 or lower achieved strong SN (86%) and SP (92%). Using a 15% base rate, positive predictive value was 64% and negative predictive value was 97%. CONCLUSIONS: The originally proposed ChAMP VI has insufficient SN in pediatric TBI. However, this study yields a promising new ChAMP VI-SS, with classification metrics that exceed any other current embedded PVT in pediatrics.

Culturally Informed Neuropsychological Evaluations in Pediatric Epilepsy: Evidence-Based Practice Considerations.

OBJECTIVE: Epilepsy is one of the most common reasons for referral for a pediatric neuropsychological evaluation due its high prevalence in childhood and our well-established clinical role in tertiary care settings. Emerging evidence indicates that racial and ethnic minority populations experience increased epilepsy burden compared with White peers. Although there has been heightened recognition in our specialty regarding the dire need for culturally and linguistically responsive evaluations, the scientific evidence to support effective neuropsychological service delivery for bi/multilingual and bi/multicultural youth with epilepsy is comparatively scant and of poor quality. As a result, significant patient and clinical challenges exist, particularly in high stakes presurgical pediatric epilepsy evaluations of bi/multilingual and bi/multicultural children. METHOD: Given that Spanish is the most common language spoken in the United States after English, this paper will focus on Spanish and English measures, but will provide evidence-based practice considerations that can inform practices with other non-English speaking communities. Cultural and linguistic factors that affect clinical decision-making regarding test selection, test interpretation, and feedback with families are highlighted. RESULTS: We offer a review of neuropsychological profiles associated with pediatric epilepsy as well as a flexible, multimodal approach for the assessment of linguistically and culturally diverse children with epilepsy based on empirical evidence and the clinical experiences of pediatric neuropsychologists from diverse backgrounds who work with children with epilepsy. CONCLUSION: Limitations to this approach are discussed, including the lack of available measures and resources for culturally and linguistically diverse pediatric populations. A case illustration highlights a culturally informed assessment approach.

Brief memory assessment in children: can the ChAMP Screening Index detect memory impairment?

Abbreviated memory batteries play a role in some clinical and research assessments, but their validity and accuracy need to be well supported. The purpose of this study was to examine the accuracy of the ChAMP Screening Index for detecting memory impairment. The sample included N = 804 youths (ages 5-21 years) with medical and neurological diagnoses who were presented for a clinical neuropsychological assessment. All completed the full Child and Adolescent Memory Profile and had valid data. The ChAMP Screening Index contains the first two subtests of the battery (Lists and Objects) and takes about 10 min to administer (full ChAMP is about 35 min). Analyses to examine the accuracy of the ChAMP Screening Index with both the Total Memory Index and Delayed Memory Index included Intraclass correlations, Cohen's Kappa coefficients, receiver operating characteristics, and classification metrics (e.g., sensitivity, specificity, positive predictive values [PPV], and negative predictive values [NPV]). Very strong correlations, minimal mean difference scores, substantial agreement on kappa coefficients, and outstanding receiver operating characteristics all support the Screening Index accuracy. A cutoff score on the Screening Index of 70 provides a good balance between a high PPV (.91) and a high NPV (.96) for the Total Memory Index. When detecting impairment on the Delayed Memory Index, a Screening Index cutoff score of 65 provides a balance between a high PPV (.92) and a high NPV (.94). This study supports the accuracy, validity, and utility of the 10-min ChAMP Screening Index in those clinical and research situations where a brief evaluation of memory is desired.

Detecting epilepsy-related cognitive problems in clinically referred children with epilepsy: is the WISC-IV a useful tool?

PURPOSE: The Wechsler Intelligence Scale for Children - Fourth Edition is the most widely used intelligence quotient (IQ) test in use today. However, despite numerous studies on IQ in childhood epilepsy, data exist almost exclusively from prior editions of the test, and no studies to date provide information on the sensitivity of specific WISC-IV scores (full-scale IQ [FSIQ], index, and subtest scores) to epilepsy-related cognitive impairments. The goal of this study was to determine the relative sensitivity of WISC-IV index and subscale scores in detecting cognitive problems in a group of clinically referred children with epilepsy compared to matched controls, and to define the relationship among WISC-IV scales, demographic factors, and epilepsy-related variables. METHODS: WISC-IV data for children with epilepsy and high seizure burden were obtained from the Alberta Children's Hospital (ACH) and the New York University Comprehensive Epilepsy Center (NYU), two tertiary care medical centers for pediatric epilepsy. All children were clinically referred and received a standard assessment including WISC-IV. Matched controls were obtained from the WISC-IV Canadian and American standardization samples. KEY FINDINGS: WISC-IV scores from 212 children were included: 106 children with epilepsy (46 girls, 60 boys; mean age 11.0 years, standard deviation [SD] 3.1; parental education 14.5 years, SD 2.8), and 106 controls matched for age, gender, ethnicity, and parental education. Of the children with epilepsy, 44 had a clearly lateralized focus on electroencephalography (EEG) involving either the right or left hemisphere (26 left, 18 right). FSIQ for the epilepsy group was significantly lower than for controls, and 36.8% of children had IQs compatible with intellectual disability (FSIQ < 70), versus <1% of controls. In children with epilepsy, Working Memory and Processing Speed Index scores were lower than those for Verbal Comprehension and Perceptual Reasoning (p < 0.01). At the subtest level, scores for children with epilepsy were highest on visual and verbal subtests measuring reasoning skills such as Matrix Reasoning, and lowest on Coding (mean 5.93, SD 3.6). In terms of percentage of children on each subtest with low scores (i.e., scores below 2 SDs from the expected normative mean of 10), the Coding subtest identified the most children (28.3%) with low scores, and the Similarities subtest identified the fewest (16%). Later age at onset and shorter epilepsy duration were both correlated with higher WISC-IV FSIQ and index scores (r correlation coefficient values ranging from 0.36 to 0.44, p < 0.0001), and number of current and previous antiepileptic drug trials were both inversely correlated with FSIQ and index scores (r -0.27 to -0.47, all p-values < 0.01). Neither the FSIQ nor the index scores were significantly related to seizure frequency. A similar pattern was found for subtest scores. No differences in FSIQ, index scores, or subtest scores were found between children with left- and right-hemisphere seizure foci, or between those with positive or negative magnetic resonance imaging (MRI) findings. SIGNIFICANCE: The WISC-IV is sensitive to epilepsy-related cognitive problems in clinically referred children with high seizure burden, particularly problems relating to expressive verbal, working memory, and processing speed difficulties. Compared to healthy children, these children have a very high rate of cognitive difficulties as assessed by the WISC-IV. The usefulness of the WISC-IV in detecting cognitive deficits in children with milder forms of epilepsy remains to be determined.

Neuropsychological profiles of two patients with differing SCN8A-pathogenic variants.

The impact of gene-related early infancy onset epilepsies in cognitive development can be potentially devastating. Here we report two cases of SCN8A-related epilepsy that highlight the neuropsychological heterogeneity seen with differing de-novo pathogenic variants. Case 1 is a 6-year-old right-handed girl who presented with SCN8A-developmental and epileptic encephalopathy (SCN8A-DEE) and a missense pathogenic variant (c.802A > C), not previously documented in the literature. Her history includes speech and motor delay, with focal motor seizures starting at 4-months. Early EEG showed bilateral centroparietal epileptiform discharges. She shows motor and language delays and prominent motor tics. Testing documented Intellectual Disability (ID) (Mild) with widespread neuropsychological deficits (i.e., academics, attention/executive functions, memory, visual-spatial skills, fine motor, language). Case 2 is an 8-year-old right-handed girl who presented with SCN8A-related epilepsy with c.5630A > G pathogenic variant with seizure onset at 5-months. Her initial EEG showed right occipital spikes. She shows low average intellect and average academics, but evaluation documented attention deficits, fine motor delays, and behavioral issues in addition to tics; she was diagnosed with Attention-Deficit/Hyperactivity Disorder, Oppositional Defiant Disorder, Obsessive Compulsive Disorder, and Tourette's. These cases expand limited knowledge regarding neuropsychological functioning of children with SCN8A-related epilepsy with unique de-novo pathogenic variants. While SCN8A-DEE is clearly associated with ID, other pathogenic variants may show better preserved intellect, despite other neuropsychological and behavioral concerns.

Quality of life following epilepsy surgery for children with tuberous sclerosis complex.

Parents of children with tuberous sclerosis complex who underwent multistage resections for treatment of refractory seizures were offered a telephone questionnaire regarding quality of life (QOL) of child and family since surgery. Of 53 families, 39 responded. Age at epilepsy onset was birth to 3 months. Average duration of epilepsy before the first surgery was 5.1 years, and average age at surgery was 5.8. The average follow-up was 3.9. Seventy-seven percent had a >90% reduction in disabling seizures. In all outcome categories, 46-85% had at least a moderate improvement in QOL. There was a significant correlation between QOL variables and Engel outcome class. Despite the potential burden posed by the aggressive surgical approach, including multiple surgeries and long hospitalization periods, 94% of parents would choose the same course once again. We conclude that aggressive surgical treatment of tuberous sclerosis complex-related refractory seizures is associated with significant control of epilepsy as well as improved QOL for the patient and family.

Cognitive and behavioral functioning in Coffin-Siris syndrome and epilepsy: a case presentation.

The authors characterized the cognitive, adaptive, and behavioral sequelae of Coffin-Siris (CS) syndrome and epilepsy in a 7.5-year-old child. Little is known about the early neurobehavioral presentation of CS. Clinical features consistent with this genetic anomaly include underdeveloped tips and nails of the fifth fingers, extended infranasal depression, and craniofacial abnormalities. MRI findings often reveal callosal agenesis. The authors conducted a neuropsychological evaluation and obtained parental ratings of behavioral and adaptive functioning. Attentional abilities were limited. As assessed by the Mullen Scales of Early Learning, receptive language abilities (age equivalent [AE]: 3-3) were relatively stronger than expressive skills (AE: 1-4). Adaptive functioning was low across all domains (Vineland Adaptive Behavior Composite AE: 1-9). On the Behavior Assessment for Children (BASC-2), social skills dysfunction, stereotyped and self-stimulatory behaviors, restricted interests, ritualistic play, and inappropriate object usage were noted. No significant mood disturbances were endorsed. Study findings indicate a diffuse pattern of neurobehavioral deficits in a child with CS and epilepsy. Further clinical assessment and research should include multidimensional assessment techniques, including evaluation of adaptive behavior, in an effort to capture the full range developmental sequelae in children with CS.

The Utility of the Repeatable Battery of Neuropsychological Status in Patients with Temporal and Non-temporal Lobe Epilepsy.

OBJECTIVE: The Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) is a brief neuropsychological battery that has been validated in the assessment of dementia and other clinical populations. The current study examines the utility of the RBANS in patients with epilepsy. METHODS: Ninety-eight patients with epilepsy completed the RBANS as part of a more comprehensive neuropsychological evaluation. Performance on the RBANS was evaluated for patients with a diagnosis of temporal lobe epilepsy (TLE; n = 51) and other epilepsy patients (non-TLE, n = 47) in comparison to published norms. Multivariate analysis of variance compared group performances on RBANS indices. Rates of impairment were also compared across groups using cutoff scores of ≤1.0 and ≤1.5 standard deviations below the normative mean. Exploratory hierarchical regressions were used to examine the relations between epilepsy severity factors (i.e., age of onset, disease duration, and number of antiepileptic drugs [AEDs]) and RBANS performance. RESULTS: TLE and non-TLE patients performed below the normative sample across all RBANS indices. Those with TLE performed worse than non-TLE patients on the Immediate and Delayed Memory indices and exhibited higher rates of general cognitive impairment. Number of AEDs was the only epilepsy severity factor that significantly predicted RBANS total performance, accounting for 14% of the variance. CONCLUSIONS: These findings suggest that the RBANS has utility in evaluating cognition in patients with epilepsy and can differentiate TLE and non-TLE patients. Additionally, number of AEDs appears to be associated with global cognitive performance in adults with epilepsy.

The clinical utility of the Social Responsiveness Scale and Social Communication Questionnaire in tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is often associated with epilepsy, mental retardation, and autism spectrum disorders (ASDs). Thus, screening for ASDs is important when evaluating these individuals. We examined the utility of the Social Responsiveness Scale (SRS) and Social Communication Questionnaire (SCQ), two measures for screening for ASDs, in a TSC population. Twenty-one children were evaluated, with 52.4% classified as having ASDs on the SRS and 42.9% classified as such on the SCQ. Number of antiepileptic drugs significantly correlated with SRS Total score, as did level of intellectual functioning. Evidence for convergent validity was obtained between the SRS and SCQ Total scores (r=0.605). Moreover, all SRS subscales correlated with SCQ Total score (r>0.400). All SCQ subscales except for Communication correlated with SRS total. Overall, the results demonstrate that these questionnaires appear to be effective screens for ASDs in a TSC population and are measuring similar constructs.

The use of the MSVT in children and adolescents with epilepsy.

Pediatric neuropsychologists are increasingly recognizing the importance of performance validity testing during evaluations. The use of such measures to detect insufficient effort is of particular importance in pediatric epilepsy evaluations, where test results are often used to guide surgical decisions and failure to detect poor task engagement can result in postsurgical cognitive decline. The present investigation assesses the utility of the Medical Symptom Validity Test (MSVT) in 104 clinically referred children and adolescents with epilepsy. Though the overall failure rate was 15.4% of the total group, children with 2nd grade or higher reading skills (a requirement of the task) passed at a very high rate (96.6%). Of the three failures, two were unequivocally deemed true positives, while the third failed due to extreme somnolence during testing. Notably, for those with ≥2nd grade reading levels, MSVT validity indices were unrelated to patient age, intellectual functioning, or age of epilepsy onset, while modest relations were seen with specific memory measures, number of epilepsy medications, and seizure frequency. Despite these associations, however, this did not result in more failures in this population of children and adolescents with substantial neurologic involvement, as pass rates exceeded 92% for those with intellectual disability, high seizure frequency, high medication burden, and even prior surgical resection of critical memory structures.

A review of performance and symptom validity testing with pediatric populations.

Growing recognition and concerns of non-credible performance in pediatric populations have led clinicians to investigate the utility of performance and symptom validity tests (PVT/SVTs) among children and adolescents. Yet current research has indicated that a minority of clinicians routinely utilize a free-standing PVT in pediatric neuropsychological evaluations. The current article investigates the rationale for using PVT/SVTs, and the impact that failure of such exams have on other neurocognitive tests. A review of common adult PVTs and their appropriateness for use with specific pediatric clinical populations is presented, as well as empirical evidence for evaluating embedded validity indicators. The limited literature on SVTs with youth is also reviewed and provides additional insight into symptom exaggeration. There are various reasons children would provide noncredible performance, many of which are different from adults. A review of how the clinician should handle this behavior in pediatric evaluations is provided and what patient populations may present with a higher base rate of failure. Finally, various approaches are offered on how to explain these results to children and their caregivers.

Fatigue and quality of life in pediatric multiple sclerosis.

Fatigue and quality of life are significant concerns in adult multiple sclerosis (MS) but little is known about these factors in pediatric MS. The present investigation evaluates fatigue and quality of life in 51 pediatric MS patients to determine the rate of fatigue and reduced quality of life and assesses the relations between these variables and clinical factors. Fatigue and quality of life were assessed by self- and parent-report via the PedsQL Multidimensional Fatigue Scale and the PedsQL Quality of Life Scale. One-sample t-tests determined if scores were below published data for healthy individuals. Moreover, scores falling one standard deviation from norms were considered mildly affected, with severe difficulties being defined as scores falling two or more standard deviations from norms. Associations between self- and parent-reported difficulties and clinical factors were examined via Pearson correlation analyses. In comparison with healthy samples, pediatric MS patients reported greater difficulties with respect to fatigue, sleep, cognition, physical limitations, and academics. In addition to significant difficulties on these factors, parents reported problems with respect to emotional functioning, and tended to report greater fatigue, sleep, and cognitive difficulties than were self-reported. Expanded Disability Status Scale score was the only neurologic variable significantly related to fatigue or quality of life scores. Fatigue was significantly correlated with reports of sleep difficulties, cognitive problems, and quality of life variables. These findings suggest that fatigue and poorer quality of life is a clear concern in pediatric MS, and is related to overall physical disability.

Negative affect predicts subsequent cognitive change in multiple sclerosis.

Baseline predictors of cognitive change were explored in a sample of persons with multiple sclerosis (MS). Potential predictors included demographic features, baseline clinical characteristics, and psychological state. Participants were 38 individuals diagnosed with either relapsing remitting or secondary progressive MS who did not meet criteria for a current major depressive episode. Subjects were tested at baseline and approximately 1 year in an ongoing longitudinal study of cognition in MS. Participants completed neuropsychological tests sensitive to impairment in MS. They also completed self-report measures of depression, anxiety, fatigue, apathy, and positive and negative affect. Baseline measures of negative affect (e.g., depressed mood, state anxiety, and negative affective state) consistently predicted cognitive change over the course of the study. Higher baseline levels of negative affect were associated with greater relative declines in cognitive performance. This longitudinal relation occurred in the absence of a cross-sectional relation between negative affect and overall cognition. High baseline negative affect particularly predicted a relative decline in episodic memory for newly learned verbal and visuospatial information. The negative affect measures were unique in their predictive value among all the baseline measures assessed. (JINS, 2009, 15, 53-61.).