Factorial invariance of the Wechsler Intelligence Scale for Children, Fifth Edition, across the UK, US and Australia & New Zealand.

OBJECTIVES: This study examined the factorial invariance of the factor structure of the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) across the UK, US and Australia & New Zealand (A&NZ). The factorial equivalence of cognitive assessments should be demonstrated before assuming cross-culture generalizability and interpretations of score comparisons. METHODS: Data were obtained from the UK, US and A&NZ normative standardizations of the WISC-V. The samples consisted of 415 UK, 2200 US and 528 A&NZ children, aged 6-16. Confirmatory factor analysis was applied separately in each sample to establish the baseline model. Next, tests of factorial invariance were undertaken using the recommended hierarchical approach, firstly across the UK and A&NZ samples and then across the UK and US samples. RESULTS: The five-factor first-order scoring model was found to be excellent fit across all three samples independently. Strict factorial invariance of the WISC-V was demonstrated firstly across the UK and A&NZ and secondly the UK and US nationally representative standardization samples. Comparison of latent means found small but significant differences in female children across the UK and A&NZ samples. CONCLUSIONS: Consistent with previous research, these results demonstrate the generality of the WISC-V factor structure across the UK, US and A&NZ. Furthermore, as the WISC-V factor structure aligns with the Cattell-Horn-Carroll (CHC) model of cognitive abilities, the results add further support to the cross-cultural generalizability of the CHC model. Small but significant differences in latent factor scores found across samples support the development and use of local normative data.

Measurement Invariance of the WISC-V across a Clinical Sample of Children and Adolescents with ADHD and a Matched Control Group.

Measurement invariance of the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) 10-primary subtest battery was analyzed across a group of children and adolescents with ADHD (n = 91) and a control group (n = 91) matched by sex, age, migration background, and parental education or type of school. First, confirmatory factor analyses (CFAs) were performed to establish the model fit for the WISC-V second-order five-factor model in each group. A sufficiently good fit of the model was found for the data in both groups. Subsequently, multigroup confirmatory factor analyses (MGCFAs) were conducted to test for measurement invariance across the ADHD and control group. Results of these analyses indicated configural and metric invariance but did not support full scalar invariance. However, after relaxing equality constraints on the Vocabulary (VC), Digit Span (DS), Coding (CD), Symbol Search (SS), and Picture Span (PS) subtest intercepts as well as on the intercepts of the first-order factors Working Memory (WM) and Processing Speed (PS), partial scalar invariance could be obtained. Furthermore, model-based reliability coefficients indicated that the WISC-V provides a more precise measurement of general intelligence (e.g., represented by the Full-Scale IQ, FSIQ) than it does for cognitive subdomains (e.g., represented by the WISC-V indexes). Group comparisons revealed that the ADHD group scored significantly lower than the control group on four primary subtests, thus achieving significantly lower scores on the corresponding primary indexes and the FSIQ. Given that measurement invariance across the ADHD and the control group could not be fully confirmed for the German WISC-V, clinical interpretations based on the WISC-V primary indexes are limited and should only be made with great caution regarding the cognitive profiles of children and adolescents with ADHD.

Intellectual function and memory in children with moyamoya disease: relationship between Wechsler Intelligence Scale and Benton Visual Retention Test scores and regional cerebral blood flow.

OBJECTIVE: Intellectual function declines in about 30% of children with moyamoya disease (MMD). Memory function underpins higher order brain function, but the relationship between intellectual function and memory in pediatric MMD patients has not been well studied. This study aimed to investigate correlations between scores on the Wechsler Intelligence Scale for Children (WISC) and the Benton Visual Retention Test (BVRT), a visual memory test that can be administered to children, in children with MMD. Relationships between intellectual function or memory and regional cerebral blood flow (rCBF) have also not been well clarified in pediatric MMD patients. The authors also investigated associations between WISC or BVRT scores and rCBF in various brain regions. METHODS: WISC and BVRT scores and rCBF were assessed in 17 children with ischemic-onset MMD before revascularization. Single-photon emission CT with 123I-iodoamphetamine was used to measure rCBF. Relationships between WISC and BVRT scores were evaluated using Spearman's correlation coefficient and multivariate linear regression analysis. Cutoff values were identified for BVRT scores. Sensitivity and specificity were calculated to predict full-scale intelligence quotient (FSIQ) > 85 or ≤ 85. Associations between rCBF and WISC or BVRT scores were evaluated using linear regression analysis. RESULTS: BVRT scores were significantly correlated with FSIQ and scores on the Working Memory Index (WMI), Processing Speed Index, and Verbal Comprehension Index (VCI)/Verbal Intelligence Quotient (VIQ) of WISC. Multivariate linear regression revealed that number correct score and number of errors score of BVRT were associated with FSIQ. As cutoff values, a number correct score of 5 and a number of errors score of 8 offered the most reliable predictors of FSIQ > 85 and ≤ 85, respectively. FSIQ correlated positively with rCBF in the right and left hemispheres, right and left ganglia, right and left thalamus, right and left cerebellum, right middle cerebral artery (MCA) territory, pons, and vermis. WMI score was positively associated with rCBF in the right hemisphere, right anterior cerebral artery territory, right MCA territory, right basal ganglia, right and left thalamus, right and left cerebellum, pons, and vermis. CONCLUSIONS: BVRT score correlated well with WISC index scores, suggesting that BVRT may be helpful in screening for intellectual impairments in children with MMD. In the MCA territory, basal ganglia, thalamus, cerebellum, pons, and vermis, rCBF associated well with WISC index scores, suggesting that reduced rCBF in relevant brain regions may influence intellectual function.

Gender Differences in the Wechsler Intelligence Scale for Children in a Large Group of Italian Children with Attention Deficit Hyperactivity Disorder.

Despite being repeatedly investigated in children with typical development, research on gender differences in intellectual abilities in specific groups of children, including children with attention deficit hyperactivity disorder (ADHD), has been scarce. In this paper, we evaluated the performance of a large group of Italian children with ADHD using the WISC-IV. We aimed at investigating the presence of gender differences using a multi-group confirmatory factor analysis approach. Results showed that the WISC is largely gender-invariant. However, some tasks present non-invariant patterns (block design and coding). Differences at the latent level also showed some differences (favoring boys) in the verbal comprehension index. Conversely, differences at the latent level were not found in the full-scale IQ or in the other main indices. These results have theoretical and practical implications.

WISC-V Measurement Invariance According to Sex and Age: Advancing the Understanding of Intergroup Differences in Cognitive Performance.

This study sought to verify whether the constructs measured on the WISC-V are equivalent according to sex and age group in Chilean students to substantiate intergroup comparisons. For this, the measurement invariance of two variants of the five-factor intelligence model was explored with the ten primary subtests (hierarchical and oblique) using multigroup confirmatory factor analysis. Seven hundred and forty participants between 6 and 16 years of age from the Chilean standardization sample were assessed. The results show complete invariance according to sex, but incomplete according to the age group. The implications of these findings in both the professional area of psychology and future research are discussed.

Cross-National Generalizability of WISC-V and CHC Broad Ability Constructs across France, Spain, and the US.

The Cattell-Horn-Carroll (CHC) model is based on psychometric cognitive ability research and is the most empirically supported model of cognitive ability constructs. This study is one in a series of cross-national comparisons investigating the equivalence and generalizability of psychological constructs which align with the CHC model. Previous research exploring the cross-cultural generalizability of cognitive ability measures concluded that the factor analytic models of cognitive abilities generalize across cultures and are compatible with well-established CHC constructs. The equivalence of the psychological constructs, as measured by the Wechsler Intelligence Scale for Children-Fifth Edition (WISC-V), has been established across English-speaking samples. However, few studies have explored the equivalence of psychological constructs across non-English speaking, nationally representative samples. This study explored the equivalence of the WISC-V five-factor model across standardization samples from France, Spain, and the US. The five-factor scoring model demonstrated excellent fit across the three samples independently. Factorial invariance was investigated and the results demonstrated strict factorial invariance across France, Spain, and the US. The results provide further support for the generalizability of CHC constructs across Western cultural populations that speak different languages and support the continued use and development of the CHC model as a common nomenclature and blueprint for cognitive ability researchers and test developers. Suggestions for future research on the CHC model of intelligence are discussed.

The Efficacy and Cognitive Impact of Perampanel Monotherapy in Patients with Self-Limited Epilepsy with Centrotemporal Spikes: A Retrospective Analysis.

Objective: The third generation of antiepileptic medication (ASM) perampanel (PER), is mostly used as an add-on treatment for refractory epilepsy patients, and rarely used as a monotherapy. This study aims to observe the efficacy and assess the cognitive effects of PER monotherapy in patients with self-limited epilepsy with centrotemporal spikes (SeLECTS). Patients and Methods: Through screening, 86 patients who were first diagnosed with SeLECTS and treated with PER monotherapy were included in this study. All patients were followed up at least 12 months, and Evaluated the efficacy and safety of PER by observing the seizures of patients. At the same time, we used the P300 event-related potential (ERP) component and Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV) to evaluate the cognitive changes in children before and after treatment with PER. Results: Ten percent of the children experienced adverse effects, such as dizziness, gait instability, and irritability. The drug retention rate at the last follow-up was 98.83%. Further more, the P300 ERP component and WISC-IV tests were performed no significant difference before and 12 months after PER monotherapy in SeLECTS children. Conclusion: The third-generation of ASM PER monotherapy had a clear effect in children with SeLECTS. A small dose of PER can control seizures well and has no obvious effect on cognitive development.

Network Analysis of ADHD Symptoms and Cognitive Profiles in Children.

Purpose: Although many studies have reported the cognitive profiles in attention-deficit/hyperactivity disorder (ADHD), the interactions between ADHD symptoms and the patients' cognitive profiles have not been carefully examined through the network analysis. Here, in this study, we systematically analyzed the ADHD patents' symptoms and cognitive profiles, and identified a set of interactions between ADHD symptoms and cognitive domains using the network approach. Patients and Methods: A total of 146 children with ADHD, 6 to 15 years of age, were included in the study. All participants were assessed by the Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV) test. The patients' ADHD symptoms were evaluated by the Vanderbilt ADHD parent and teacher rating scales. GraphPad Prism 9.1.1 software was used for descriptive statistics and R 4.2.2 was used for network model construction. Results: The ADHD children in our sample showed lower scores for full scale intelligence quotient (FSIQ), verbal comprehension index (VCI), processing speed index (PSI) and working memory index (WMI). Among all the ADHD core symptoms and comorbid symptoms, the academic ability, inattention symptoms and mood disorder showed direct interaction with the cognitive domains of WISC-IV. In addition, oppositional defiant of the ADHD comorbid symptoms, and perceptual reasoning of the cognitive domains exhibited the highest strength centrality in the ADHD-Cognition network based on parent ratings. Classroom behaviors of the ADHD functional impairment, and verbal comprehension of the cognitive domains exhibited the highest strength centrality in the network based on teacher ratings. Conclusion: We highlighted the importance of considering the interactions between the ADHD symptoms and cognitive properties when designing the intervention plans for the ADHD children.

Examine sex differences in autism spectrum disorder in school-aged children and adolescents with fluent language.

There are noteworthy sex disparities in the prevalence of autism spectrum disorders (ASD), while findings regarding the sex differences in core symptoms are inconsistent. There are few relevant studies on sex differences in mainland China. This study was dedicated to a deeper understanding of the impact of sex differences on the clinical presentation of ASD with fluent language. We retrospectively studied 301 children with ASD (58 females) and utilized raw scores from the ADI-R and ADOS and the intelligence quotient (IQ) to measure symptomatology. Based on the Full-Scale IQ (FS-IQ), a binary split of average, above-average IQ (high-IQ), and below-average IQ (low IQ) occurs at 85. Across the entire sample, males and females are comparable in the FS-IQ, while males scored higher in the Perceptual Reasoning Index (PRI) (F = 7.812, p = 0.006). ADI-R did not find any statistically significant sex differences in the diagnostic cutoff score satisfaction or the raw domain scores. While a significant effect of sex on ADOS social affect domain scores was found in the total sample [λ = 0.970, partial η2 = 0.030, F (3,295) = 3.019, p = 0.030]. Tests of between-subjects effects revealed that males scored higher than females mainly in the ADOS reciprocal social interaction subcategory (partial η2 = 0.022, F = 6.563, p = 0.011). Stratified analysis revealed that the effect of sex on ADOS reciprocal social interaction subcategory scores only significant in the low-IQ children with ASD (partial η2 = 0.092, F = 10.088, p = 0.002). In general, overall cognitive functioning is similar across males and females with ASD, while males have a higher perceptual reasoning ability. Females with ASD are more likely to have comorbid intellectual impairment than males, and they could require additional intervention support. Autistic children with low IQs are more likely to exhibit sex differences in their core symptoms than children with high IQs. Intelligence plays a key role in sex-based differences in the core symptoms of ASD.

Vitamin D status in pregnancy and childhood associates with intelligence quotient at age 7 years: An Odense child cohort study.

OBJECTIVES: Animal studies indicate a key role for vitamin D in brain development and function, but observational studies in humans only suggests a borderline positive association between prenatal vitamin D exposure and cognitive development in the offspring. Knowledge gaps include insights in exposure time window and differences by sex for the association. We aimed to investigate the association between blood concentrations of serum 25-hydroxyvitamin D measured at four different time points and intelligence quotient score at the age of 7 years, including analyses spilt by child sex. METHODS: In Odense child cohort, we included 1404 mother-child pairs with serum 25-hydroxyvitamin D data from early pregnancy to age 7 years. Full-scale intelligence quotient was assessed with Wechsler Intelligence Scale for Children - fifth edition. Associations were adjusted for maternal education, pre-pregnancy body mass index, gestational age, sex and head circumference. Subanalyses stratified by sex were performed. RESULTS: The median (interquartile range) serum 25-hydroxyvitamin D in cord was 45.88 (31.15-61.08) nmol/L; early pregnancy, 66.45 (51.29-78.74); late pregnancy, 79.13 (59.69-97.31); 7 years, 66.29 (53.45-80.23) nmol/L. The mean (standard deviation) full-scale intelligence quotient was 99.44 (11.98). In adjusted analyses, cord serum 25-hydroxyvitamin D < 50 nmol/L was associated with 2.2 points lower full-scale intelligence quotient compared to the reference (50-75 nmol/L) in boys, ß = -2.2; 95% confidence interval = [-4.3, -0.1], p = 0.039. The same association with full-scale intelligence quotient was found for early pregnancy serum 25-hydroxyvitamin D, ß = -2.5 [-4.6, -0.3], p = 0.025, primarily driven by an association in boys, ß = -4.0 [-7.2, -0.8], p = 0.015; and for serum 25-hydroxyvitamin D at 7 years in girls, ß = -3.0 [-6.0, -0.1], p = 0.042. CONCLUSION: In this cohort, serum 25-hydroxyvitamin D < 50 nmol/L in both early gestation and cord blood in boys and current serum 25-hydroxyvitamin D < 50 nmol/L in girls were independent risk factors for two to four points lower full-scale intelligence quotient at the age of 7 years. Vulnerability to hypovitaminosis D, especially in pregnancy, may relate to child sex.

Peripapillary retinal nerve fibre layer thickness and macular ganglion cell layer volume in association with motor and cognitive outcomes in 11-year-old children born very preterm.

PURPOSE: The purpose of the study was to study the association between retinal parameters and motor and cognitive outcomes in children born very preterm. METHODS: This study is part of a prospective cohort study of very preterm infants (birth weight ≤ 1500 grams/gestational age < 32 weeks). At 11 years of age, the ophthalmological assessment included a retinal optical coherence tomography (OCT) examination of the peripapillary retinal nerve fibre layer (PRNFL) and the macular ganglion cell layer (GCL). The motor performance was assessed with the Movement Assessment Battery for Children-Second Edition (Movement ABC-2), and the cognitive outcome with the Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV). RESULTS: A total of 141 children were included. The mean (SD) average PRNFL was 95 µm (10.2 µm). The mean (SD) macular GCL volume was 0.34 mm3 (0.03 mm3 ). Higher PRNFL thickness associated with higher percentiles for total scores in the motor assessment (b = 0.5, 95% CI 0.1-0.8, p = 0.01) and higher macular GCL volume with higher scores in the cognitive assessment (b = 1.4, 95% CI 0.5-2.3, p = 0.002), also when adjusted for gender, birth weight z-score (birth weight in relation to gestational age) and major brain pathology at term. CONCLUSION: The associations between higher average PRNFL thickness and better motor performance as well as higher macular GCL volume and better cognitive performance refer to more generalized changes in the brain of 11-year-old children born very preterm. Retinal OCT examinations might provide a deeper insight than mere eyesight in long-term neurodevelopmental follow-up of children born very preterm.

Prenatal and early childhood predictors of intelligence quotient (IQ) in 7-year-old Danish children from the Odense Child Cohort.

BACKGROUND: Cognitive development measured as intelligence quotient can predict socioeconomic markers in adulthood. It is therefore of interest to determine predictors of childhood intelligence quotient. AIM: To assess intelligence quotient scores based on standardised Danish age-appropriate scores and to evaluate potential predictors of intelligence quotient. MATERIALS: At 7 years of age children in the Odense Child Cohort completed an abbreviated version of the Wechsler intelligence scale for children 5th edition consisting of four subtests (vocabulary, similarities, block design and matrix reasoning) from which the full scale intelligence quotient and verbal comprehension index were estimated. Potential predictors from pregnancy through childhood were collected from questionnaires, birth records and clinical examinations. METHODS: Intelligence quotient scores were investigated through descriptive statistics and linear regression models. RESULTS: The mean full scale intelligence quotient among 1375 children was 99.1 (95% confidence interval 98.5; 99.8) points. Higher full scale intelligence quotient scores were observed in girls 100.8 (95% confidence interval 100.0; 101.8) compared to boys 97.6 (96.7; 98.4), and in children of mothers with high and intermediate education 101.7 (100.4; 103.1) and 99.6 (98.7; 100.5), respectively, compared to low education 96.1 (94.9; 97.3). In linear regression analyses, longer maternal education and child sex (girls) remained strong predictors of intelligence quotient at age 7 years. In addition, paternal education, child head circumference and longer duration of breastfeeding were associated with higher intelligence quotient, whereas maternal overweight and obesity before pregnancy was associated with lower intelligence quotient. CONCLUSIONS: Mean intelligence quotient scores were comparable to the standardised mean intelligence quotient of 100 point of Danish peers. It is important to follow up these children to determine which predictors persist into adulthood.

Effect of Mahjong on children's intelligence quotient.

This study investigated the effect of Mahjong, which is a table game played by three or four players and involves intellectual activity, on the intelligence quotient (IQ) of children. The participants were children between the age of 6 and 15 years, and their IQ was assessed immediately after enrolling in children's Mahjong classes and 1 year after the enrollment using the Wechsler Intelligence Scale for Children Fourth Edition (WISC-IV). Twenty children were included in the analysis. Their mean age at the time of the initial evaluation was 9 years and 6 months. In addition, we conducted a 1-year post-examination. The change in the IQ of this group was compared to that of a historical control group with a similar age range and test-retest interval. The mean overall full-scale IQ of the 20 children during the initial and post-1-year examinations was 106.05 and 113.75, respectively, and showed a statistically significant increase (p < 0.01). Based on the subscale index, the verbal comprehension index (VCI) and processing speed index (PSI) scores both showed a statistically significant increase from 100.6 to 106.75 and from 108.05 to 119.05 (p < 0.01), respectively. The PSI of the children included in the analysis showed a statistically significant increase compared to the historical control group. This study suggests that children who participate in Mahjong classes during their childhood have increased PSI scores of WISC-IV.

A comparative analysis of children born with low birthweight and attention deficit hyperactivity disorder.

BACKGROUND: We aimed to compare the profile of the Wechsler Intelligence Scale for Children Fourth Edition (WISC-IV) between Japanese schoolchildren born very preterm (VP) and with very low birthweight (VLBW) and those with attention deficit hyperactivity disorder (ADHD), and to identify the specific neurocognitive characteristics of VLBW/VP children. METHODS: The VLBW/VP group in the present study included 50 (19 male, 31 female) first- to third-grade elementary school children born between January 2008 and February 2013 at Osaka Medical and Pharmaceutical University Hospital and Saiseikai Suita Hospital with birthweights <1,500 g and <32 gestational weeks. The ADHD group included 18 (13 male, 5 female) first- to third-grade elementary school children who visited Osaka Medical and Pharmaceutical University Hospital between January 2019 and October 2021. Full-scale intelligence quotient scores, four indices, and 12 subtests of the WISC-IV were calculated for all participants and compared between the VLBW/VP and ADHD groups. We assessed whether the patients' clinical history was associated with a low score on the cancellation task in the VLBW/VP group. RESULTS: The WISC-IV profiles showed similar between-group patterns, and the VLBW/VP group had lower cancellation task scores than the ADHD group. CONCLUSIONS: This is the first study to compare WISC-IV profiles between VLBW/VP children and those with ADHD. Further investigation is needed on the association between academic performance and the score of the cancellation task, and the neural mechanism of low performance for cancellation tasks in VLBW/VP children.

IQ Was Not Improved by Post-Discharge Fortification of Breastmilk in Very Preterm Infants.

(1) Very preterm infants are at increased risk of cognitive deficits, motor impairments, and behavioural problems. Studies have tied insufficient nutrition and growth to an increased risk of neurodevelopmental impairment; (2) Methods: Follow-up study on cognitive and neuropsychological development at 6 years corrected age (CA) in 214 very preterm infants, including 141 breastfed infants randomised to mother's own milk (MOM) with (F-MOM) or without (U-MOM) fortification and 73 infants fed a preterm formula (PF-group), from shortly before discharge to 4 months CA. Infants with serious congenital anomalies or major neonatal morbidities were excluded prior to intervention. The Wechsler Intelligence Scale for Children IV was used for cognitive testing, and the children's parents completed the Five to Fifteen Questionnaire (FTF); (3) Results: Post-discharge fortification of MOM did not improve either full-scale intelligence quotient (FSIQ) with a median of 104 vs. 105.5 (p = 0.29), subdomain scores, or any domain score on the FTF questionnaire. Compared to the PF group, the MOM group had significantly better verbal comprehension score with a median of 110 vs. 106 (p = 0.03) and significantly better motor skills scores on the FTF questionnaire (p = 0.01); (4) Conclusions: The study supports breastfeeding without fortification as post-discharge nutrition in very preterm infants, and it seems superior to preterm formula.

The effects of obstructive sleep apnea-hypopnea syndrome (OSAHS) on learn and memory function of 6-12 years old children.

BACKGROUND: Obstructive sleep apnea-hypopnea syndrome (OSAHS) is a sleep disorder causing cognitive impairments. AIMS: We use the auditory verbal learning test (AVLT), clock drawing test (CDT), Wechsler intelligence scale for children (WISC) and Montreal cognitive assessment (MoCA) to evaluate the memory and spatial impairments of OSHAS in 6-12 years old children patients with different severity. MATERIAL AND METHODS: A total of 137 children of snoring were enrolled following the inclusion criteria of this study. According to the apnea-hypopnea indices (AHI), they were divided into three groups. The AVLT, CDT, WISC and MoCA tests were executed by physicians. The self-rating depression scale (SDS) test was performed for depression screening. RESULTS: Compared with the children in the primary snoring group, the other two groups had higher body mass index (BMI), longer periods of snoring and older age. The AHI, oxygen desaturation index (ODI) and 90% oxygen saturation (TS90%) showed increasing trends whereas the lowest blood oxygen saturation (LSaO2) showed a decreasing trend. Besides, compared with the primary snoring group, the two groups had lower immediate recall scores in AVLT. CONCLUSION: AVLT had clinical values for evaluation of impaired memory function in OSAHS children, suggesting a correlation between cognitive impairments and nocturnal hypoxia.

Early clinical signs and treatment of Menkes disease.

Menkes disease (MD) is an X-linked recessive disorder caused by mutations in ATP7A. Patients with MD exhibit severe neurological and connective tissue disorders due to copper deficiency and typically die before 3 years of age. Early treatment with copper injections during the neonatal period, before the occurrence of neurological symptoms, can alleviate neurological disturbances to some degree. We investigated whether early symptoms can help in the early diagnosis of MD. Abnormal hair growth, prolonged jaundice, and feeding difficulties were observed during the neonatal period in 20 of 69, 16 of 67, and 3 of 18 patients, respectively. Only three patients visited a physician during the neonatal period; MD diagnosis was not made at that point. The mean age at diagnosis was 8.7 months. Seven patients, who were diagnosed in the prenatal stage or soon after birth, as they had a family history of MD, received early treatment. No diagnosis was made based on early symptoms, highlighting the difficulty in diagnosing MD based on symptoms observed during the neonatal period. Patients who received early treatment lived longer than their elderly relatives with MD. Three patients could walk and did not have seizures. Therefore, effective newborn screening for MD should be prioritized.

A prospective behavioral and imaging study exploring the impact on long-term memory of radiotherapy delivered for a brain tumor in childhood and adolescence.

BACKGROUND: Posterior fossa tumors represent two thirds of brain tumors in children. Although progress in treatment has improved survival rates over the past few years, long-term memory impairments in survivors are frequent and have an impact on academic achievement. The hippocampi, cerebellum and cerebellar-cortical networks play a role in several memory systems. They are affected not only by the location of the tumor itself and its surgical removal, but also by the supratentorial effects of complementary treatments, particularly radiotherapy. The IMPALA study will investigate the impact of irradiation doses on brain structures involved in memory, especially the hippocampi and cerebellum. METHODS/DESIGN: In this single-center prospective behavioral and neuro-imaging study, 90 participants will be enrolled in three groups. The first two groups will include patients who underwent surgery for a posterior fossa brain tumor in childhood, who are considered to be cured, and who completed treatment at least 5 years earlier, either with radiotherapy (aggressive brain tumor; Group 1) or without (low-grade brain tumor; Group 2). Group 3 will include control participants matched with Group 1 for age, sex, and handedness. All participants will perform an extensive battery of neuropsychological tests, including an assessment of the main memory systems, and undergo multimodal 3 T MRI. The irradiation dose to the different brain structures involved in memory will be collected from the initial radiotherapy dosimetry. DISCUSSION: This study will provide long-term neuropsychological data about four different memory systems (working memory, episodic memory, semantic memory, and procedural memory) and the cognitive functions (attention, language, executive functions) that can interfere with them, in order to better characterize memory deficits among the survivors of brain tumors. We will investigate the correlations between neuropsychological and neuroimaging data on the structural (3DT1), microstructural (DTI), functional (rs-fMRI), vascular (ASL) and metabolic (spectroscopy) impact of the tumor and irradiation dose. This study will thus inform the setting of dose constraints to spare regions linked to the development of cognitive and memory functions. TRIAL REGISTRATION: ClinicalTrials.gov: NCT04324450, registered March 27, 2020, updated January 25th, 2021. Retrospectively registered, https://www.clinicaltrials.gov/ct2/show/NCT04324450.

Correlation between the Bayley-III at 3 years and the Wechsler Intelligence Scale for Children, Fourth Edition, at 6 years.

BACKGROUND: Although several studies have investigated the association between Bayley-III results in infancy and future intellectual development, conclusions remain unclear. We used the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) at 3 years of age and the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV) at 6 years of age to assess the neurodevelopment of very low birthweight infants. METHODS: We investigated the correlation between Bayley-III's cognitive, language, and motor scores and the WISC-IV's Full-Scale Intelligence Quotient (FSIQ). We also determined the optimal cut-off value of Bayley-III to enter the normal development zone (FSIQ ≥ 85). RESULTS: We found a strong correlation between the Bayley-III and the FSIQ. Optimal cut-off scores of the Bayley-III to enter the normal range on the WISC-IV were 95 for the cognitive scale, 89 for the language scale, and 91 for the motor development scale. CONCLUSIONS: Although Bayley-III scores strongly correlated with the WISC-IV FSIQ, the lower normal limit of 85 on the Bayley-III suggests a potential overestimation of development in children who were VLBW infants.

Intellectual development of patients with biliary atresia who underwent living donor liver transplantation in infancy.

BACKGROUND: The impact of pediatric liver transplantation on intellectual development has yet to be determined. We investigated the intellectual outcomes of school-aged patients after living donor liver transplantation for biliary atresia in infancy. METHODS: The Wechsler Intelligence Scale for Children-fourth edition test was administered to 20 patients who survived [Formula: see text] 5 years after living donor liver transplantation. Borderline full scale intelligence quotient was defined as ≤ 85. Pre-, peri-, and postoperative data were compared between patients with > 85 and ≤ 85 to identify predictive factors of borderline performance. RESULTS: The one-sample t test demonstrated that the mean full scale intelligence quotient of patients after transplantation for biliary atresia was significantly lower than that of the general population (91.8 vs. 100.0, p = 0.026) and 7 (35%) were classified as intellectual borderline functioning. Multivariable logistic regression models were unable to identify any factors predictive of full scale intelligence quotients of ≤ 85. CONCLUSION: This is the first study to indicate that the mean full scale intelligence quotient among school-aged patients who underwent living donor liver transplantation for biliary atresia in infancy is significantly lower than that of the general population.