Assessment of MR blood-oxygen-level-dependent (BOLD) cerebrovascular reactivity under general anesthesia in children with moyamoya.

BACKGROUND: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve. However, non-compliant or younger children require general anesthesia to achieve BOLD-CVR imaging. OBJECTIVE: To determine the same-day repeatability of BOLD-CVR imaging under general anesthesia in children with moyamoya. MATERIALS AND METHODS: Twenty-eight examination pairs were included (mean patient age = 7.3 ± 4.0 years). Positive and negatively reacting voxels were averaged over signals and counted over brain tissue and vascular territory. The intraclass correlation coefficient (ICC), Wilcoxon signed-rank test, and Bland-Altman plots were used to assess the variability between the scans. RESULTS: There was excellent-to-good (≥ 0.59) within-day repeatability in 18 out of 28 paired studies (64.3%). Wilcoxon signed-rank tests demonstrated no significant difference in the grey and white matter CVR estimates, between repeat scans (all p-values > 0.05). Bland-Altman plots of differences in mean magnitude of positive and negative and fractional positive and negative CVR estimates illustrated a reasonable degree of agreement between repeat scans and no systematic bias. CONCLUSION: BOLD-CVR imaging provides repeatable assessment of cerebrovascular reserve in children with moyamoya imaged under general anesthesia.

Tele-neuropsychological Assessment of Children and Young People: A Systematic Review.

The coronavirus pandemic identified a clinical need for pediatric tele-neuropsychology (TeleNP) assessment. However, due to limited research, clinicians have had little information to develop, adapt, or select reliable pediatric assessments for TeleNP. This preliminary systematic review aimed to examine the feasibility of pediatric TeleNP assessment alongside (1) patient/family acceptability, (2) reliability, and (3) the quality of the literature. Between May 2021 and November 2022, manual searches of PubMed, PsycINFO, and Google Scholar were conducted using terms related to "pediatric" and "tele-neuropsychology." After extracting relevant papers with samples aged 0-22 years, predefined exclusion criteria were applied. Quality assessment was completed using the AXIS appraisal tool (91% rater-agreement). Twenty-one studies were included in the review, with reported qualitative and quantitative data on the feasibility, reliability, and acceptability extracted. Across included studies, TeleNP was completed via telephone/video conference with participants either at home, in a local setting accompanied by an assistant, or in a different room but in the same building as the assessor. Pediatric TeleNP was generally reported to be feasible (e.g., minimal behavioral differences) and acceptable (e.g., positive feedback). Nineteen studies conducted some statistical analyses to assess reliability. Most observed no significant difference between in-person and TeleNP for most cognitive domains (i.e., IQ), with a minority finding variable reliability for some tests (e.g., attention, speech, visuo-spatial). Limited reporting of sex-assigned birth, racialized identity, and ethnicity reduced the quality and generalizability of the literature. To aid clinical interpretations, studies should assess underexamined cognitive domains (e.g., processing speed) with larger, more inclusive samples. Supplementary Information: The online version contains supplementary material available at 10.1007/s40817-023-00144-6.

Health-related quality of life and the burden of prolonged seizures in noninstitutionalized children with epilepsy.

OBJECTIVE: This study aimed to provide information on the burden of illness and health-related quality of life (HRQoL) in children with epilepsy who experience prolonged acute convulsive seizures (PACS) in the community setting, and to investigate factors that may predict poor HRQoL in this population. METHODS: Noninstitutionalized children (aged 3-16 years) who had experienced at least one PACS within the past year and had currently prescribed PACS rescue medication were enrolled in a cross-sectional study in Germany, Italy, Spain, and the United Kingdom (Practices in Emergency and Rescue medication For Epilepsy managed with Community-administered Therapy 3 [PERFECT-3]). Clinicians, parents/guardians, and patients completed web-based questionnaires regarding clinical characteristics, PACS frequency, and day-to-day impairment. Patients' HRQoL was rated by clinicians, parents/guardians, and patients themselves using the 5-dimension EuroQol questionnaire (EQ-5D) and summarized as a utility score. Potential predictors of poor HRQoL were tested in individual univariate generalized linear models and a global multivariable model. RESULTS: Enrolled children (N = 286) had experienced 1-400 PACS (median: 4) in the past year. Clinicians reported that 216/281 patients (76.9%) had learning disabilities of varying severity. Mean EQ-5D utility scores rated by clinicians (n = 279), parents (n = 277), and patients (n = 85) were 0.52 (standard deviation: 0.41), 0.51 (0.39), and 0.74 (0.29), respectively. Increasing PACS frequency, increasing severity of learning disability, and specialist school attendance were significantly associated with decreasing EQ-5D utility score. In the multivariable model, having learning disabilities was the best predictor of poor HRQoL. SIGNIFICANCE: Health-related quality of life was very poor in many children with epilepsy whose PACS were managed with rescue medication in the community, with learning disability being the most powerful predictor of patients' HRQoL. Mean EQ-5D utility scores were lower (worse) than published values for many other chronic disorders, indicating that optimal treatment should involve helping children and their families to manage learning disabilities and day-to-day impairments, in addition to preventing seizures.

The Role of Family Functioning in the Development of Executive Functions in Preschool Children with Sickle Cell Anemia.

Executive functions are compromised in children with sickle cell anemia. There is limited research on the development of executive functions in preschool children with sickle cell anemia and the factors that contribute to executive dysfunction. We looked at the relation between biomedical and environmental factors, including family functioning and socioeconomic status, and executive functions in 22 preschool children with sickle cell anemia. We found that family functioning was the strongest predictor of executive outcomes in young children with sickle cell anemia with no evidence for an influence of disease severity at this early stage.

Executive performance on the preschool executive task assessment in children with sickle cell anemia and matched controls.

Executive deficits are commonly reported in children with sickle cell anemia. Earlier identification of executive deficits would give more scope for intervention, but this cognitive domain has not been routinely investigated due to a lack of age-appropriate tasks normed for preschool children. In particular, information relating to patient performance on an executive task that reflects an everyday activity in the classroom could provide important insight and practical recommendations for the classroom teacher at this key developmental juncture as they enter the academic domain. The performance of 22 children with sickle cell anemia was compared to 24 matched control children on the Preschool Executive Task Assessment. Findings reveal that children with sickle cell anemia are performing poorer than their matched peers on this multi-step assessment. In particular, children with sickle cell anemia required more structured support to shift focus after a completed step, as reflected by poorer scores in the quantitative Sequencing and Completion domains. They also required more support to stay on task, as seen by poorer ratings in the qualitative Distractibility domain. Abbreviations:PETA: Preschool Executive Task Assessment; SCA: Sickle Cell Anemia; EF: Executive Functioning.

Assessment of Executive Functions in Preschool Children With Sickle Cell Anemia.

OBJECTIVES: Children with sickle cell anemia (SCA) are commonly reported to experience executive dysfunction. However, the development of executive function (EF) in preschool-age children without stroke in this patient population has not been investigated so it is unclear when and how these deficits emerge. METHODS: This case-control study examines the feasibility of assessing the early development of executive functioning in 22 preschool children years with SCA in the domains of processing speed, working memory, attention, inhibitory control, and cognitive flexibility, as well as everyday function, in comparison to matched control children. RESULTS: A pattern of potential deficits in early emerging executive skills was observed in the domains of inhibitory control and cognitive flexibility. Parents reported no differences for everyday EF and no significant differences were observed for working memory and processing speed. CONCLUSIONS: Results suggest that deficits in everyday executive difficulties, working memory, and processing speed, as commonly reported for older children with SCA, may not yet have emerged at this early developmental stage, despite specific deficits in cognitive flexibility and inhibitory control on behavioral measures. The feasibility of using available executive measures with preschool age children to characterize the development of early EF skills is discussed. (JINS, 2018, 24, 949-954).

Task utility and norms for the Preschool Executive Task Assessment (PETA).

Earlier identification of executive deficits in preschool children using an ecological approach would give more scope for intervention. The Preschool Executive Task Assessment (PETA) was developed to resemble an everyday age-appropriate task in order to examine the self-direction and integration of executive functions during a multistep task. It was designed so that performance can be evaluated in a microanalytic way and so individualized feedback and support can be easily communicated. The utility of the PETA was assessed with 166 three-to five-year olds. Results showed improved performance with increasing age and verbal intellectual quotient as well as good task reliability and utility. Evidence for influence of socioeconomic status, gender, and use of self-talk was also observed. Clinical applications and future directions of this novel measure are discussed.

A general model to calculate the spin-lattice (T1) relaxation time of blood, accounting for haematocrit, oxygen saturation and magnetic field strength.

Many MRI techniques require prior knowledge of the T1-relaxation time of blood (T1bl). An assumed/fixed value is often used; however, T1bl is sensitive to magnetic field (B0), haematocrit (Hct), and oxygen saturation (Y). We aimed to combine data from previous in vitro measurements into a mathematical model, to estimate T1bl as a function of B0, Hct, and Y. The model was shown to predict T1bl from in vivo studies with a good accuracy (± 87 ms). This model allows for improved estimation of T1bl between 1.5-7.0 T while accounting for variations in Hct and Y, leading to improved accuracy of MRI-derived perfusion measurements.