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.

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.

Social adjustment in children diagnosed with sickle cell disease: A retrospective study.

BACKGROUND: Children with sickle cell disease (SCD) are at risk for physical, psychological, and social adjustment challenges. This study sought to investigate social adjustment and related factors in children living with SCD. METHODS: Data from 32 children (50% male, mean age = 10.32 years, SD = 3.27) were retrospectively collected from a neuropsychology clinic at a tertiary care pediatric hospital. Social adjustment was measured using the Behavior Assessment System for Children (BASC-3) parent-proxy, withdrawal subscale, and the Pediatric Quality of Life Inventory (PedsQL) Generic Module Social Functioning self- and parent-proxy subscales. Other measures captured executive functioning (i.e., Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2) Parent Form) and non-disease-related associations with social adjustment, including number of years in Canada and family functioning (i.e., PedsQL Family Impact Module). RESULTS: Sixteen percent of patients reported elevated social adjustment difficulties. Multiple linear regression found better family functioning [B = .48, t = 2.65, p = .016], and higher executive functioning [B = -.43, t = -2.39, p = .028] were related to higher scores on the PedsQL parent-proxy ratings of social adjustment [F(4,18) = 5.88, p = .003]. Male sex [B = .54, t = 3.08, p = .005], and having lived more years in Canada [B = .55, t = 2.81, p = .009], were related to higher PedsQL self-reported social adjustment [F(4,23) = 3.75, p = .017]. The model examining the BASC-3 withdrawal subscale was not statistically significant [F(4,16) = 1.63, p = .22]. IMPLICATIONS: Social adjustment in children diagnosed with SCD warrants future research to understand the influence of executive function, and non-disease-related factors, particularly focusing on sociocultural factors.

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.

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.

Social adjustment across the lifespan in survivors of pediatric acute lymphoblastic leukemia (ALL): a systematic review.

PURPOSE: The objectives of this review were to (1) summarize studies that described social adjustment in survivors of pediatric ALL across the lifespan, (2) summarize social adjustment outcomes reported across studies, and (3) examine associations between social adjustment and disease/treatment- and non-treatment-related factors. METHODS: Searched databases included EMBASE (Ovid), MEDLINE (Ovid), PsycINFO (EBSCO Information Services), and Web of Science (Thomson Reuters). Eligible studies included: (1) original research; (2) published in English; (3) a diagnosis of cancer between 0 and 21 years; (4) survivors at least 5 years from diagnosis and/or 2 years from therapy completion; and (5) quantitative assessment of social adjustment. RESULTS: The literature search yielded 3698 articles of which 43 were included in the final review. Risk of bias was assessed using domains adapted from the Cochrane risk-of-bias tool. Quality of evidence was evaluated following the Grading of Recommendations Assessment Development and Evaluation (GRADE) criteria. There was some evidence that school-aged and adolescent/young adult survivors experienced worse social adjustment compared to controls. There was some evidence suggesting cranial radiation therapy (CRT) is associated with social adjustment difficulties among young adult survivors. Inconsistent evidence was found for relapse, age at diagnosis and study, sex, and late effects in relation to social adjustment. CONCLUSION: Survivors of pediatric ALL were at higher risk of social adjustment difficulties compared to controls. However, evidence for treatment and non-treatment risk and resilience factors require stronger evidence. IMPLICATIONS FOR CANCER SURVIVORS: Information on modifiable factors that modulate social adjustment may influence targets of intervention and follow-up guidelines.

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.

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.

Health related quality of life in children with sickle cell disease: A systematic review and meta-analysis.

This review had three aims: 1) describe the measures used to assess health-related quality of life (HRQL) in pediatric patients diagnosed with sickle cell disease (SCD); 2) document the biopsychosocial factors related to HRQL in pediatric patients diagnosed with SCD; and 3) complete a meta-analysis comparing HRQL in pediatric patients diagnosed with SCD to healthy controls. Included studies were published in English, quantitatively assessed HRQL as a primary aim, in both SCD and controls, and included participants between 0 and 21 years of age. The final review included 66 articles, with a total of 8642 participants with SCD, 4 months-21 years of age, and 62,458 controls, 5-27 years of age. HRQL was predominately measured using the Pediatric Quality of Life Inventory Generic Core and Sickle Cell Disease Module. Meta-analyses revealed children with SCD had significantly worse HRQL compared to healthy controls (standardized mean difference = -0.93, 95% CI = -1.25, -0.61, p < 0.00001). Worse HRQL was associated with more severe SCD, female sex, and pain. The findings indicate that children with SCD are at risk for worse HRQL compared to their healthy peers and their HRQL may be impacted by several biopsychosocial factors. Future research is needed to examine how sociocultural factors uniquely impact this population and their overall quality of life.

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.

Cognitive outcomes of childhood primary CNS vasculitis.

OBJECTIVE: To characterize the clinical cognitive phenotypes and severity of cognitive burden according to disease subtype in children with primary central nervous system vasculitis (cPACNS). METHOD: This retrospective multicenter inflammatory brain disease database study examined the neuropsychological outcomes of 80 children (44 male; mean age = 7.89 years, SD = 4.17) consecutively diagnosed with primary CNS vasculitis between 1992 and 2016. Twenty-one children had small-vessel disease (AN_cPACNS), and 59 had large-vessel disease (including 49 nonprogressive [APNP_cPACNS] and 10 progressive [APP_cPACNS]). Neuroimaging revealed MRI abnormalities in 100% and 90% of children with large- and small-vessel vasculitis, respectively. The primary outcomes were Full Scale IQ (FSIQ) and the index scores from the Wechsler Intelligence Scale for Children-III (WISC-III, WISC-IV, and WISC-V). Analyses explored the effect of disease subtype. RESULTS: Intellectual functioning was assessed on average 2.82 years after symptom onset. Children with small-vessel CNS vasculitis had significantly lower FSIQ scores than did those with large-vessel CNS vasculitis (Ms = 81.90 vs. 94.82; p = .04). Intellectual disability (FSIQ < 70) was more frequent in children with small-vessel disease (24% vs. 5%). All groups displayed lower Working Memory and Processing Speed index scores relative to Verbal Comprehension and Perceptual Reasoning index scores. Group differences in FSIQ remained significant after controlling for the presence of seizures. CONCLUSION: Children with small-vessel CNS vasculitis are more likely to demonstrate deficits in intellectual functioning than are those with large-vessel disease, and children with both types of CNS vasculitis demonstrate relatively poor working memory and processing speed. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

The WISC-V in children and adolescents with epilepsy.

Despite its popularity in the neuropsychological evaluation of children, the utility of the Wechsler Intelligence Scale for Children - Fifth Edition (WISC-V) has not yet been investigated in children with epilepsy. Eighty clinically referred children and adolescents with epilepsy were administered the WISC-V as part of a comprehensive assessment and scores were compared to matched controls from the WISC-V standardization sample. T tests compared WISC-V indices and subtests between patients and controls and Chi-square analyses compared the rates of low scores. Correlational analyses assessed the relationships between epilepsy severity variables (e.g., age of onset, duration of epilepsy, number of antiepileptic drugs, seizure frequency). All WISC-V composites and subtests were significantly lower in patients versus controls and the rate of low scores was higher in patients than controls for all composites and subtests with the exception of Figure Weights. The Working Memory Index and Processing Speed Index were most sensitive to impairment, while the Verbal Comprehension Index and Fluid Reasoning Index were least sensitive. Of the epilepsy severity variables, age of seizure onset and number of antiepileptic drugs were strong predictors of deficits, whereas seizure frequency was the weakest predictor. Importantly, no significant differences were seen in children with right hemisphere epilepsy versus left on the five WISC-V composites, though a trend was seen towards a lower Visual-Spatial Index in those with right-sided focal seizures.

Factor structure of the CNS Vital Signs computerized cognitive battery in youth with neurological diagnoses.

Computerized cognitive batteries, such as CNS Vital Signs (CNSVS), can provide valuable information in clinical and research settings. However, psychometric properties, especially in children and adolescents, remain relatively understudied. The aim of this study was to investigate the factor structure of CNSVS in children and adolescents with neurological diagnoses. Participants with neurological diagnoses (N = 280) age 7-19 years were assessed as part of their clinical care at a tertiary hospital. All participants received the full CNSVS computerized cognitive battery, which contains seven subtests designed to measure attention, executive functioning, psychomotor speed, and memory. Principal components analyses were used to examine factor structure. Scores from CNSVS subtests loaded onto a three-component solution and accounted for 46% of the variance. The three components were deemed to best represent (1) speed, (2) memory, and (3) inhibition, with subtest scores loading differently than the original 11 primary and secondary domain scores would have suggested. Although the CNSVS program generates numerous primary and secondary domain scores, a three-component solution represents a more parsimonious approach to interpreting performance on the CNSVS in youth with neurological diagnoses. Confirmation of this factor solution in other samples is warranted.

A new kid on the block: The Memory Validity Profile (MVP) in children with neurological conditions.

Determining the validity of obtained data is an inherent part of a neuropsychological assessment. The purpose of this study was investigate the failure rate of the Memory Validity Profile (MVP) in a large clinical sample of children and adolescents with neurological diagnoses. Data were obtained from 261 consecutive patients (mean age = 12.0, SD = 3.9, range = 5-19) who were referred for a neuropsychological assessment in a tertiary care pediatric hospital and were administered the MVP. In this sample, 4.6% of youth failed the MVP. Mean administration time for the MVP was 7.4 min, although time to complete was not associated with failure rates. Failure rates were held relatively consistent at approximately 5% across age ranges, diagnoses, and psychomotor processing speed abilities. Having very low, below normal, or above normal intellectual abilities did not alter failure rate on the MVP. However, those with intellectual disability (i.e., IQ<70) had a higher fail rate at 12% on MVP Total Score, but only 6% on the MVP Visual portion. Failure rates on the MVP were associated with lower scores on memory tests. This study provides support for using the MVP in children as young as 5 years with neurological diagnoses.

Derivation of New Embedded Performance Validity Indicators for the Child and Adolescent Memory Profile (ChAMP) Objects Subtest in Youth with Mild Traumatic Brain Injury.

BACKGROUND: Development of an embedded performance validity test (PVT) is desired for visual memory tests. The goal of this study was to derive an embedded PVT for the Child and Adolescent Memory Profile (ChAMP) Objects visual memory subtest in youth with mild traumatic brain injury (MTBI). METHODS: Children and adolescents (N = 91; mean age = 14.9 years, SD = 2.2, range = 8-18) on average 25.2 weeks (SD = 15.4) post-MTBI were administered ChAMP Objects. Two stand-alone PVTs (Test of Memory Malingering and Medical Symptom Validity Test) were administered, which allowed for grouping into valid (zero failed stand-alone PVTs) and invalid (both stand-alone PVTs failed). Cutoff scores for invalid performance on ChAMP Objects and Objects Delayed were established using failure on two PVTs as the criterion. RESULTS: One in five youth (n = 19) failed both PVTs. Invalid performance was not associated with demographics or time since injury, but was significantly correlated with both ChAMP Objects (r = .53, p<.001) and Objects Delayed (r = -.63, p<.001). Area under the curve suggested adequate discrimination by Objects (.87) and excellent discrimination by Objects Delayed (.91). A cutoff scaled score of 5 or less on ChAMP Objects provided sensitivity of 58% for detecting invalid performance with 96% sensitivity. A cutoff scaled score of 5 or less on ChAMP Objects Delayed achieved sensitivity of 63% and specificity of 96%. Interpreting the two embedded PVTs simultaneously improved sensitivity to 79% with 93% specificity. CONCLUSION: This study yields promising new embedded PVTs for the ChAMP Objects subtest with strong sensitivity and specificity for detecting invalid performance in youth with MTBI.

Social adjustment in adolescent survivors of pediatric central nervous system tumors: A report from the Childhood Cancer Survivor Study.

BACKGROUND: The purpose of this study was to examine the prevalence and predictors of social difficulties in adolescent survivors of central nervous system (CNS) tumors. METHODS: Six hundred sixty-five survivors of CNS tumors (53.8% male and 51.7% treated with cranial radiation therapy [CRT]), who had a current median age of 15.0 years (range, 2.0-17.0 years) and were a median of 12.1 years (range, 8.0-17.7 years) from their diagnosis, were compared with 1376 survivors of solid tumors (50.4% male), who had a median age of 15.0 years (range, 12.0-17.0 years) and were a median of 13.2 years (range, 8.3-17.9 years) from their diagnosis, and 726 siblings (52.2% male), who had a median age of 15.0 years (range, 12.0-17.0 years). Social adjustment was measured with parent-proxy responses to the Behavior Problems Index. Latent profile analysis defined social classes. Multinomial logistic regression, adjusted for age, sex, and age at diagnosis, identified predictors of class membership. Path analyses tested mediating effects of physical limitations, sensory loss, and cognitive impairment on social outcomes. RESULTS: Caregivers reported that survivors of CNS tumors were more likely to have 0 friends (15.3%) and to interact with friends less than once per week (41.0%) in comparison with survivors of solid tumors (2.9% and 13.6%, respectively) and siblings (2.3% and 8.7%, respectively). Latent profile analysis identified 3 social classes for survivors of CNS tumors: well-adjusted (53.4%), social deficits (16.2%), and poor peer relationships (30.4%). However, 2 classes were identified for survivors of solid tumors and siblings: well-adjusted (86.2% and 91.1%, respectively) and social deficits (13.8% and 8.9%, respectively). CRT predicted class membership for CNS survivors (odds ratio [OR] for poor peer relationships, 1.16/10 Gy; 95% confidence interval [CI], 1.08-1.25; OR for social deficits 1.14/10 Gy; 95% CI, 1.04-1.25; reference, well-adjusted). Cognitive impairment mediated the association between all social outcomes and CRT (P values < .001). CONCLUSION: Almost 50% of survivors of CNS tumors experience social difficulties; the pattern is unique in comparison with solid tumor and sibling groups. Cognitive impairment is associated with increased risk, and this highlights the need for multitargeted interventions.

Long-term neuropsychological follow-up of young children with medulloblastoma treated with sequential high-dose chemotherapy and irradiation sparing approach.

High-dose chemotherapy (HDC) strategies were developed in brain tumor protocols for young children to prevent neuropsychological (NP) impairments associated with radiotherapy. However, comprehensive NP evaluations of these children treated with such strategies remain limited. We examined the long-term neurocognitive outcomes of young children (<6 years) with medulloblastoma, treated similarly, with a HDC strategy "according to" the chemotherapy regimen of the protocol CCG 99703. This retrospective study included young children less than 6 years of age at diagnosis of medulloblastoma treated from 1998 to 2011 at 7 North American institutions. Twenty-four patients who had at least one NP assessment post-treatment are the focus of the current study. Of 24 patients in this review, 15 (63%) were male and the mean age at diagnosis was 29.4 months (SD = 13.5). Posterior fossa syndrome (PFs) was reported in five patients (21%). Nine (37.5%) received radiotherapy (5 focal, 4 craniospinal). On average, children were assessed 3.5 years (SD = 1.8) post-diagnosis, and full-scale intellectual quotient (FSIQ) scores ranged from 56 to 119 ([Formula: see text]= 92; SD = 16.8). The majority of children (74%) had low-average to average NP functioning. Very young children treated with radiotherapy, who needed hearing support or with PFs had worse neurocognitive outcomes. Clinically significant deficits (<10th percentile) in at least one area of NP functioning were found in 25% of the children. NP data obtained from this sample of survivors of medulloblastoma in early childhood, all treated with sequential HDC and 1/3 with radiotherapy, describe NP functioning within average normal limits overall. However, almost 25% of children had significant deficits in specific domains.

Systematic review and meta-analysis of health-related quality of life in pediatric CNS tumor survivors.

BACKGROUND: Pediatric central nervous system (CNS) tumor survivors are at high risk for numerous late effects including decreased health-related quality of life (HRQOL). Our objective was to summarize studies describing HRQOL in pediatric CNS tumor survivors and compare HRQOL outcomes in studies that included a comparison group. PROCEDURE: EMBASE, MEDLINE, and PsychINFO were used to identify relevant articles published until August, 2016. Eligible studies reported outcomes for pediatric CNS tumor survivors diagnosed before age 21, at least 5 years from diagnosis and/or 2 years off therapy and used a standardized measure of HRQOL. All data were abstracted by two reviewers. Random-effects meta-analyses were performed using Review Manager 5.0. RESULTS: Of 1,912 unique articles identified, 74 were included in this review. Papers described 29 different HRQOL tools. Meta-analyses compared pediatric CNS tumor survivors to healthy comparisons and other pediatric cancer survivors separately. HRQOL was significantly lower for CNS (n = 797) than healthy comparisons (n = 1,397) (mean difference = -0.54, 95% confidence interval [CI] = -0.72 to -0.35, P < 0.001, I2 = 35%). HRQOL was also significantly lower for CNS (n = 244) than non-CNS survivors (n = 414) (mean difference = -0.56, 95% CI = -0.73 to -0.38, P < 0.00001, I2 = 0%). CONCLUSIONS: Pediatric CNS tumor survivors experience worse HRQOL than healthy comparisons and non-CNS cancer survivors. Future HRQOL work should be longitudinal, and/or multisite studies that examine HRQOL by diagnosis and treatment modalities.

Neurocognitive evaluation of long term survivors of atypical teratoid rhabdoid tumors (ATRT): The Canadian registry experience.

BACKGROUND: Because atypical teratoid rhabdoid tumor(ATRT) is a rare disease of infancy carrying a grim prognosis, focus on long-term outcome, especially neurocognitive remained very limited. With new era of multimodality therapy, an increasing proportion of patients are now long-term survivors. PROCEDURE: Retrospective review of neuropsychological (NP) status of survivors from the Canadian ATRT registry. RESULTS: Among 77 patients diagnosed between 1995-2012, 16(22%) were survivors. Formal NP assessments were available in eight patients. Partial information on academic achievement was available on three additional patients. There were four girls and seven boys diagnosed at a median age of 27.5 months. Seven patients underwent complete resection and three had metastatic disease. All but one received sequential high dose chemotherapy. Five patients received intrathecal chemotherapy. Three patients underwent radiation. Median age at time of formal NP assessment was 7.6 years (3.9-9.8). Full Scale Intellectual Quotient (FSIQ) ranged from less than 50-119 (mean 78). Simple expressive and receptive language appeared relatively preserved. Three recently diagnosed patients (median time assessment from diagnosis 2.6 years (1.6-4.7)) had average to high average FSIQ, academic and visual spatial skills, visual, and verbal memory. Five other patients diagnosed earlier and tested at a median time of 4.9 years (3.3-8.3) post-diagnosis had a FSIQ ranging from <50 to 71. Approximately 50% of their scores were in the impaired range. CONCLUSION: Overall this cohort appears significantly impaired at school age despite the absence of systematic radiotherapy. Larger series focusing on neurocognitive outcome are needed in the current context where treatment strategies include adjuvant radiation.

Wechsler Intelligence Scale for Children-fourth edition (WISC-IV) short-form validity: a comparison study in pediatric epilepsy.

PURPOSE: The purpose of this article was to investigate the accuracy of the WISC-IV short forms in estimating Full Scale Intelligence Quotient (FSIQ) and General Ability Index (GAI) in pediatric epilepsy. METHODS: One hundred and four children with epilepsy completed the WISC-IV as part of a neuropsychological assessment at a tertiary-level children's hospital. The clinical accuracy of eight short forms was assessed in two ways: (a) accuracy within +/- 5 index points of FSIQ and (b) the clinical classification rate according to Wechsler conventions. The sample was further subdivided into low FSIQ (≤ 80) and high FSIQ (> 80). RESULTS: All short forms were significantly correlated with FSIQ. Seven-subtest (Crawford et al. [2010] FSIQ) and 5-subtest (BdSiCdVcLn) short forms yielded the highest clinical accuracy rates (77%-89%). Overall, a 2-subtest (VcMr) short form yielded the lowest clinical classification rates for FSIQ (35%-63%). The short form yielding the most accurate estimate of GAI was VcSiMrBd (73%-84%). CONCLUSIONS: Short forms show promise as useful estimates. The 7-subtest (Crawford et al., 2010) and 5-subtest (BdSiVcLnCd) short forms yielded the most accurate estimates of FSIQ. VcSiMrBd yielded the most accurate estimate of GAI. Clinical recommendations are provided for use of short forms in pediatric epilepsy.