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Over the past 15 years, there have been significant advances in the treatment of acute and chronic medical consequences of stroke in childhood. Given high rates of survival in pediatric stroke, practitioners are tasked with treating the ongoing motor and neuropsychological sequelae in patients over the course of their development. This article provides a review of the current literature on neuropsychological outcomes in pediatric stroke, including intelligence, academics, language, visual-spatial skills, attention, executive functions, memory, and psychosocial function. Recent developments in functional neuroimaging are discussed, with a particular focus on language outcomes. We further review the current research on cognitive and behavioral rehabilitation and introduce intervention models in pediatric stroke. In the final section, we discuss future directions for clinical practice and research in pediatric stroke.

Background Given the expanding evidence of clinico-radiological differences between moyamoya disease (MMD) and moyamoya syndrome (MMS), we compared the clinical and radiographic features of childhood MMD and MMS to identify predictors of ischemic event recurrence. Methods We reviewed a pediatric moyamoya cohort followed between 2003 and 2019. Clinical and radiographic characteristics at diagnosis and follow-up were abstracted. Comparisons between MMD and MMS as well as between MMD and two MMS subgroups (neurofibromatosis [MMS-NF1] and sickle cell disease [MMS-SCD]) were performed. Results A total of 111 patients were identified. Patients with MMD presented commonly with transient ischemic attacks (TIAs) (35 % MMD versus 13% MMS-NF1 versus 9.5% MMS-SCD; P = 0.047). Symptomatic stroke presentation (MMD 37% versus MMS-NF1 4% versus 33%; P = 0.0147) and bilateral disease at diagnosis (MMD 73% versus MMS-NF1 22 % versus MMS-SCD 67%; P = 0.0002) were uncommon in MMS-NF1. TIA recurrence was common in MMD (hazard ratio 2.86; P = 0.001). The ivy sign was absent on neuroimaging in a majority of patients with MMS-SCD (MMD 67% versus MMS-NF1 52% versus MMS-SCD 9.5%; P = 0.0002). Predictors of poor motor outcome included early age at diagnosis (odds ratio [OR] 8.45; P = 0.0014), symptomatic stroke presentation (OR 6.6; P = 0.019), and advanced Suzuki stage (OR 3.59; P = 0.019). Conclusions Moyamoya exhibits different phenotypes based on underlying etiologies. Frequent TIAs is a common phenotype of MMD and symptomatic stroke presentation a common feature of MMD and MMS-SCD, whereas unilateral disease and low infarct burden are common in MMS-NF1. In addition, absence of ivy sign is a common phenotype in MMS-SCD.

Understanding how pediatric neuropsychological evaluations support families and the child's medical team is an important component of ensuring evidence-based care. For the first time within a Canadian context, we investigated the impact of neuropsychological assessments on parent knowledge, advocacy, and stress and the role of socioeconomic factors in parents' perceptions of the assessment. Responses from referring clinicians were also examined. As part of a hospital quality improvement project, 91 parents of children between the ages of 3 and 17 years (M = 8y7m; SD = 4y1m) and 45 clinician consumers (clinical staff who use neuropsychological services) completed one of two online questionnaires in English: Parent Overall Assessment of Supports and Testing, or Clinician Overall Assessment of Supports and Testing. Most parents indicated the neuropsychological evaluation promoted understanding of their child's cognitive profile and improved their ability to support their child at home and at school or in the community. Families characterized as being at higher social risk indicated that the evaluation led to more changes in how they approached their child at home than families with lower social risk status. Referring clinicians indicated neuropsychological reports were effective in communicating findings to them and patients/families. The most valuable sections of the report according to referring clinicians included the diagnosis/impression and recommendations sections. Parents and referral providers reported many benefits from the neuropsychological evaluation but also identified areas for service delivery improvement. Parent perceptions varied based on family and socio environmental factors, offering important targets for future research and clinical consideration.

Aim Although many children who experience ischemic stroke come from bilingual backgrounds, it is unclear whether bilingual exposure affects poststroke development. Our research evaluates bilingual and monolingual exposure on linguistic/cognitive development poststroke across 3 stroke-onset groups. Method: An institutional stroke registry and medical charts were used to gather data on 237 children across 3 stroke-onset groups: neonatal

The current study examined the utility of the Behavior Rating Inventory of Executive Function-Preschool Version (BRIEF-P) in capturing emerging deficits in executive function in preschool children with a history of perinatal stroke. Parents and teachers of 55 clinically referred preschool children (3-5 years of age) provided ratings using the BRIEF-P. Both parent (

The current study examined reported patterns of utilization and barriers to early and school-age interventions, as well as directions for future care, among families of children with congenital or ...

Aim To assess long-term cognitive function in children after cerebral sinovenous thrombosis (CSVT). Method Children with CSVT, who had neuropsychological testing for intellectual ability, executive function, attention, language, or behavior, were included in a prospective observational study. Outcomes were compared with normative means using one-sample t-tests. Predictors of abnormal function were examined using logistic regression. Results Fifty children with CSVT were included (median age at diagnosis 2y 10mo, interquartile range 7d-6y 10mo; 35 males, 15 females). The median follow-up time was 4 years 2 months (interquartile range 2y 8mo-6y 4mo). Compared with normative means, children with CSVT had lower mean (± standard deviation) full-scale IQ, working memory, and processing speed scores (93.3±16, p=0.01; 93.6±16, p=0.04; 93.7±15.3, p=0.02 respectively). They also had lower scores in executive function, attention, and language domains. Refractory seizure at presentation was associated with a trend in behavioral problems (odds ratio [OR] 6.3, 95% confidence interval [CI] 0.9-46, p=0.07). Females were less likely to experience processing speed difficulties (OR 0.22, 95% CI 0.04-1.3, p=0.09). Incomplete recanalization was associated with a greater risk of abnormal verbal comprehension (OR 5.3, 95% CI 0.93-30.5, p=0.059). Interpretation Children with CSVT as a group performed below age expectations on standardized neuropsychological tests, although there was variability across individuals and cognitive domains. Larger studies are needed to evaluate predictors of cognitive deficits in children with CSVT.

The pediatric stroke outcome measure (PSOM) is a standardized, disease-specific outcome measure. We aimed to validate the overall classification of neurological deficit severity using PSOM.We identified 367 neonates/children with arterial ischemic stroke (AIS) (Derivation Cohort). We analyzed the PSOM subscales (scored as 0 [no deficit], 0.5 [minimal/mild deficit; normal function], 1 [moderate deficit; slowing function], or 2 [severe deficit; missing function]) to derive severity levels using latent class analysis (LCA). We validated a severity classification scheme (PSOM-SCS) in: (a) children who had Pediatric Evaluation of Disability Inventory (PEDI; n = 63) and/or the Pediatric Quality-of-Life Inventory (PedsQL; n = 97) scored; and (b) an external cohort (AIS; n = 102) with concurrently scored modified Rankin Scale (mRS), King's Outcome Scale for Childhood Head-Injury (KOSCHI) and PSOM.Within the Derivation Cohort, LCA identified three severity levels: "normal/mild," "moderate," and "severe" (83.7%, 13.3%, and 3%, respectively). We developed severity classification based on PSOM subscale scores: "normal/mild"-normal function in all domains or slowing in one domain, "moderate"-slowing in ≥2 domains or missing function in one domain, and "severe"-missing function in ≥2 domains or slowing in ≥1 plus missing in one domain. PEDI and PedsQL both differed significantly across the severity groups. PSOM-SCS displayed high concordance with mRS (agreement coefficient [AC2] = 0.88) and KOSCHI (AC2 = 0.79).The PSOM-SCS constitutes a valid tool for classifying overall neurological severity emphasizing function and encompassing the full range of severity in pediatric stroke.Arithmetic summing of the PSOM subscales scores to assess severity classification is inadequate.The prior severity classification using PSOM overestimates poor outcomes.Three distinct severity profiles using PSOM subscales are identified.The PSOM-SCS is in moderate to excellent agreement with other disability measures.PSOM-SCS offers a valid tool for classifying the overall neurological deficit severity.

Pediatric arterial ischemic stroke involving the basal ganglia and/or thalamus is one of the most common causes of dystonia in children. Dystonia is a movement disorder in which excessive, involuntary muscle contractions result in twisting or repetitive movements, and abnormal posturing. The basal ganglia have been implicated in mood functioning and difficulties in these domains have been noted in adults with dystonia, yet little is currently known about these outcomes in children with dystonia following stroke. The objective of this study was to explore mental health outcomes in children with basal ganglia stroke and to determine whether children with post-stroke dystonia experience additional mental health difficulties. We also examined associations between mental health, cognitive, and motor outcomes. Participants were 75 children with stroke involving the basal ganglia and/or thalamus (dystonia n = 24, no dystonia n = 51). Results supported the presence of greater levels of anxiety and depression symptoms in children with post-stroke dystonia after stroke relative to those with similar patterns of stroke, but no dystonia. There were no significant associations between motor, cognitive, and mental health outcomes in children with post-stroke dystonia aside from depression and behavioral regulation. Motor and cognitive outcome were significantly associated in the stroke only group. These findings suggest maladaptive reorganization after stroke may contribute to motor, cognitive, and mental health outcomes in children with post-stroke dystonia, and that these outcomes are independent from one another.

Background: Dystonia is one of the most common movement disorders after pediatric basal ganglia stroke, causing significant disability. It presents several months post stroke suggesting a role of maladaptive neuroplasticity in its manifestation. Children with dystonia show additional difficulties in intelligence (IQ), academics, and cognitive inhibition, beyond those associated with stroke. Objective: To examine structural connectivity in children with post-stroke dystonia and its relationship with cognitive outcomes. Method: A prospective study of children with unilateral basal ganglia stroke and dystonia and healthy controls. Diffusion tensor imaging was used to assess white matter integrity in the cingulum and superior longitudinal fasciculus (SLF) using fractional anisotropy (FA) and mean diffusivity (MD). Participants completed measures of IQ and cognitive inhibition and flexibility. Results: Six children with basal ganglia stroke and dystonia (6 female; Median age= 15.98, IQR= 4.32) and 6 neurotypical controls (3 male; Median age= 18.01, IQR= 5.13) were included. Participants were age-matched within 1-3 years. Hemispheres were matched by dominance such that if the stroke was in the dominant hemisphere the control’s dominant hemisphere was matched to the ipsilateral side and vice versa. Mann Whitney-U tests showed children with dystonia had poorer performance on measures of IQ (U= 0, p=. 002, η 2 = .69) and inhibition (U= 4.5, p= .026, η 2 = .39). FA was lower in the ipsilateral cingulum (U= 5, p= .041, η 2 = .36) and MD was higher in the ipsilateral SLF (U= 32, p= .026, η 2 = .42) in the dystonia participants. Significant associations (p< .05) were found between ipsilateral cingulum FA and IQ (r= .56), inhibition (r= .76), and flexibility (r= .60); contralateral cingulum FA and inhibition (p= .59); ipsilateral SLF FA and inhibition (r= .51) and flexibility (r= .58); and ipsilateral SLF MD and inhibition (r= -.54) and flexibility (r= -.55). Associations between the contralateral cingulum FA and IQ (r= .47, p= .060) and flexibility (r= .50, p= .051) trended to significance. Conclusion: Findings support the hypothesis that post-stroke dystonia reflects maladaptive reorganization after injury that is associated with motor and cognitive outcome.

Introduction: Approximately 2/3 of children face neurological deficits following arterial ischemic stroke (AIS). In neurotypical development, bilingualism is thought to confer certain linguistic and cognitive advantages. However, research on whether this may affect development in bilingual pediatric stroke patients is scarce. Objectives: To evaluate the effects of bilingual vs. monolingual exposure on language and cognitive outcomes in children following arterial ischemic stroke across two studies: (1) large-scale group analysis using the Pediatric Stroke Outcome Measure (PSOM); (2) exploratory case studies using neuropsychological measures. Methods: An institutional stroke registry and chart review were used to gather patient and stroke data, including the Pediatric Stroke Outcome Measure (PSOM) and standardized neuropsychological measures performed at several timepoints post-stroke in children with AIS. In Study 1, growth curve modeling based on PSOM subscales, predicted by age at stroke groups, language groups and their interactions was used. In Study 2, comparative case studies were conducted with a monolingual-bilingual pair, aged 7 and 8 at stroke onset. Results: In Study 1, 237 patients were identified (44%, bilingual patients). Patients were stratified based on age at stroke onset i.e. neonatal ( Conclusion: Overall, we found no negative consequences of a bilingual environment on post-stroke outcomes. Additionally, it is possible that bilingualism has a faciliatory effect on expressive language development post-stroke. Further research should evaluate linguistic advantage effects prospectively in bilingual children with stroke and a control group.

AIM Many children who experience ischemic stroke come from bilingual backgrounds. Whether bilingual exposure affects post-stroke development is unclear. Our research evaluates the effects of bilingual vs. monolingual exposure on linguistic/cognitive development post-stroke. METHOD An institutional stroke registry and medical charts were used to gather clinical variable and outcome measure performance data on 237 children across 3 stroke-onset groups: neonatal (0-28 days), first-year (>28 days to 12 months) and childhood (13 months to 18 years). We used the Pediatric Stroke Outcome Measure (PSOM) administered at several timepoints post-stroke, to evaluate cognitive and linguistic development. RESULTS While no main effect by language exposure group was found, bilingual children with stroke onset between 1 and 12 months of age, had better post-stroke performance on the language expression subscale, compared to monolinguals within 10 years after stroke onset. INTERPRETATION Overall, no clear advantages nor detrimental effects of bilingualism were found on children9s post-stroke development of language and cognition. Our study suggests that a bilingual environment facilitates some aspects of language development - if the stroke occurs early in life. WHAT THIS PAPER ADDS Bilingual exposure post-stroke in pediatric patients does not result in worse linguistic and/or cognitive development. Bilingual children with stroke onset between 1-12 months had better expressive language post-stroke. Bilingualism may be associated with protective factors when expressive language development is considered.

Pediatric stroke is associated with a range of maladaptive cognitive and behavioral outcomes that often require targeted intervention. Despite increasing research on neuropsychological outcomes over the past decade, evidence for effective therapies and interventions for the most commonly reported cognitive and behavioral challenges is still limited. The most widely prescribed interventions address more overt deficits in sensorimotor and speech/language functions, yet interventions for higher-order cognitive, linguistic and behavioral deficits are notably less defined. Moreover, concepts of rehabilitation in adult stroke cannot be easily translated directly to pediatric populations because the effect of stroke and recovery in the developing brain takes a very different course than in the mature brain. In pediatric stroke, neuropsychological deficits often emerge gradually over time necessitating a long-term approach to intervention. Furthermore, family and school context often play a much larger role. The goal of this review is to describe cognitive and behavioral interventions for perinatal and childhood stroke, as motor rehabilitation is covered elsewhere in this issue. We also discuss cognitive aspects of current rehabilitative therapies and technology. Acknowledging the current limited state of stroke-specific rehabilitation research in children, findings from pediatric acquired brain injury intervention and use of transdiagnostic approaches lend important insights. Because there is limited support for single domain (cognitive) trainings and translation of research rehabilitation programs to clinical practice can be challenging, the value of holistic multidisciplinary approaches to improve everyday function in children and adolescents following stroke is emphasized.

Understanding how pediatric neuropsychological evaluations support families and the child's medical team is an important component of ensuring evidence-based care. For the first time within a Canadian context, we investigated the impact of neuropsychological assessments on parent knowledge, advocacy, and stress and the role of socioeconomic factors in parents' perceptions of the assessment. Responses from referring clinicians were also examined. As part of a hospital quality improvement project, 91 parents of children between the ages of 3 and 17 years (

The current longitudinal study examined academic outcomes of children diagnosed with secondary attention deficit-hyperactivity disorder (S-ADHD) following stroke in comparison to children with stroke-only and children with developmental ADHD (D-ADHD), and explored potential predictors of progress in these groups. We followed 55 children (n = 17 S-ADHD, n = 18 stroke-only, and n = 20 D-ADHD) over approximately four years. Children with S-ADHD and D-ADHD were more likely to have a comorbid learning disability, but children with S-ADHD were more likely to have declines in their reading scores over time. No individual or neurological factors accounted for declines. Math scores were equally likely to decline across all youth.

Objectives:The current study used a mixed-method design to qualitatively examine parents’ definitions of resilience and factors they believed optimized their child’s early outcome following neonatal brain injury. This was followed by quantitative analyses of early developmental and mental health outcomes and their relation to salient biopsychosocial factors.Methods:Participants were parents of children diagnosed with neonatal brain injury due to stroke or hypoxic-ischemic encephalopathy (N=51; age range of children 18 months to 8 years). The Parent Experiences Questionnaire (PEQ) was used to qualitatively analyze parents’ open-ended responses about their child’s early experiences and outcome. The Child Behavior Checklist (CBCL) and Scales of Independent Behaviour Early Developmental Form (SIB-ED) parent ratings were used to measure child resilience from a quantitative perspective, identifying “at-risk” and “resilient” children using standard cutoffs. “Resilient” and “at-risk” children were compared on biopsychosocial variables using univariatettests and chi-square analyses.Results:Parents provided five unique definitions of their child’s positive outcomes, and many children demonstrated resilience based on parent perspectives and quantitative definitions. Supporting factors included close medical follow-up, early intervention, and intrinsic factors within the child and parent. Group comparisons of “resilient” and “at-risk” children highlighted the importance of parent mental health across these early developmental and mental health outcomes.Conclusions:Many children were described as resilient during the early years by parents using qualitative and quantitative approaches. Findings highlighted the importance of parent well-being in promoting optimal early outcomes. (JINS, 2019,25, 390–402.)

Introduction: Pediatric arterial ischemic stroke (AIS) is a serious cause of lifelong neurological and cognitive disabilities. Although they are at increased risk of obstructive sleep apnea (OSA), children with AIS may have abnormal sleep indices even in the absence of OSA diagnosis. This can adversely affect their blood vessels health (impaired cerebrovascular reactivity, CVR) and can lead to neurocognitive impairments. Therefore, we aimed to investigate the association of cerebral blood vessels health with polysomnographic measures and cognitive function in children with AIS. Hypothesis: Quantitative measure of negative CVR, i.e. fractional negativity (fneg) and negative CVR correlate significantly with abnormal overnight oxygen and carbon dioxide levels in children with AIS, even in the absence of OSA. Methods: We conducted a single-centre cross-sectional study in children with AIS aged between 8 and 18 years old and who were not at risk of OSA (i.e., pediatric sleep questionnaire score (1) Non-invasive Blood Oxygen Level Dependent magnetic resonance imaging to study CVR. (2) An overnight polysomnography (3) Comprehensive evaluation of intellectual abilities, attention and executive function. Results: Ten children with AIS (40% males; median age: 14.3 years) were included after a median of 8.8 years following stroke onset. None of the children was diagnosed with OSA. We found significant correlations between fneg and minimum oxygen saturation during REM sleep (r=0.81, r=0.68 and r=0.76 in grey matter, white matter and whole brain, respectively; p Conclusion: Impaired CVR in children with AIS correlates with abnormal sleep indices even in the absence of OSA diagnosis. This can potentially result in cognitive difficulties, the majority of which remain not fully explained by stroke-related factors.

Although pediatric stroke is associated with higher survival rates compared with adult stroke, a substantial body of evidence indicates significant neuropsychologic morbidity in pediatric stroke survivors. Neuroplasticity does not guarantee good outcome in children. The general trends observed in the literature are reviewed as is the profile observed in common causes of pediatric stroke: congenital heart disease, moyamoya disease, and sickle cell disease. The neuropsychologic profile of pediatric stroke patients is heterogeneous due to the multiplicity of associated causes. Stroke in early infancy and large strokes are associated with cognitive impairment while more limited disorders, such as phasic deficit, are observed in childhood stroke. Executive dysfunction is common in pediatric stroke, but social interaction skills are usually preserved. Congenital heart disease and sickle cell disease are associated with global neuropsychologic dysfunction while cognition is usually preserved in moyamoya. Executive dysregulation is instead more frequently reported in this population. Further study of maladaptive processes after pediatric stroke will allow identification of predictors of functional and neuropsychologic outcomes and permit personalization of care.

Parents of children with neonatal stroke offer critical insight into potential avenues to direct neuropsychological care from the time of diagnosis through the early years. The aims of this study were to (1) describe what parents remember about early prognostic discussions with the medical team and (2) to examine the relationships between initial prognoses and the child's current neurodevelopmental status and parents' mental health.

Background: Cerebral arteriopathy (CA) is a major risk factor for childhood arterial ischemic stroke and is associated with significant cognitive impairments. Disease-related characteristics partially explain the variability observed in specific cognitive outcomes. Adult studies demonstrate an association between stroke risk factors such as increased body mass index (BMI) and blood pressure (BP) and cognitive function, suggesting a more diffuse mechanism of brain injury. We aimed to evaluate the association between BMI and BP with cognitive function in children with CA. Methods: We conducted a cross-sectional study in children with CA and no stroke, aged 5-18 years. Children were evaluated using Wechsler Intelligence Scale for Children (WISC), Adaptive Behaviour Assessment System (ABAS), Beery-Buktenica Developmental Test (BEERY) and Behaviour Assessment System for Children (BASC). Children with BMI≥85 th percentile were classified as overweight and those with systolic or diastolic BP≥90 th percentile were classified as prehypertensive/hypertensive (Pre-HTN). Results: Of 88 children with CA, 38 were included (63% female; median age:11.3 years). Pre-HTN and overweight were present in 26% and 29%, respectively. Significant negative correlations were found between BMI and working memory (R 2 =-0.5, p=0.01) and perceptual reasoning (R 2 =-0.4, p=0.04) of the WISC. Beery-perception scores correlated significantly with diastolic BP (R 2 =-0.5, p=0.02) and BMI (R 2 =-0.7, p=0.01). The social composite score of the ABAS correlated significantly with the BMI (R 2 =-0.7, p=0.04). Overweight children had significantly lower BEERY-motor scores (53.5±30.4 vs 84.8±17.8, p=0.03) and higher BASC-externalizing problems (58±11.4 vs 47.9±8.4, p=0.02) compared to children with normal weight. Hypertensive children displayed significantly lower BEERY-visual perception scores (63.5±26.2 vs 92.2±12.5, p=0.01). Conclusion: Increased BMI and BP may contribute to cognitive impairments observed in children with CA. We suggest that a more diffuse mechanism of brain injury is responsible for the specific cognitive impairments associated with increased BMI and BP. Larger controlled studies involving structural and perfusion imaging are needed.

An important cause of acquired brain injury in children, pediatric ischemic stroke can cause sequelae across a wide range of cognitive domains, including verbal reasoning and processing speed. As a result, survivors are especially vulnerable to academic difficulties and face unique challenges compared to their peers. Despite this knowledge, pediatric stroke remains an understudied neurological condition, and its impact on school functioning poorly understood. The present clinical study addressed academic outcome in this population using a multifaceted approach. Patients were recruited for participation from the Children's Stroke Program at the Hospital for Sick Children in Toronto, Canada. A battery of standardized neuropsychological tests was administered, and additional data was collected through parent-rated measures and review of recent academic report cards. Compared to peers, youth with stroke exhibited deficits in processing speed, verbal reasoning, and core academic skills spanning reading, reading comprehension, writing, and math. Lesion laterality did not affect cognitive and academic scores. Youth with a combined cortical-subcortical lesion scored lower on a test of nonverbal reasoning compared to youth with either cortical or subcortical lesions. Compared to healthy peers, the pediatric stroke group was more likely to report requiring extra help at school, accommodations, individual education plans, and assistive technology. They were also more likely to be diagnosed with a learning disability, but not Attention Deficit/Hyperactivity Disorder. Nonverbal reasoning skills, school grades, and school-related quality of life were comparable between groups. The present study contributed to a more nuanced understanding of the impact of pediatric stroke on academic outcome.

Stroke and other cerebrovascular conditions are typically thought of as adult diseases. In fact, the incidence of ischemic stroke peaks twice throughout the lifetime—first in the perinatal period and later in older adulthood (Cardenas et al., Child’s Nervous System 27(9):1375–1390, 2011). Historically, what was referred to as “congenital hemiplegia” is now recognized to be ischemic stroke in the majority of cases (Kirton et al., Neurology 86(18):1659–1667, 2016). Modern advances in neuroimaging and medicine have allowed for more accurate diagnosis and treatment of congenital heart disease, stroke, and other vascular conditions in childhood, including sickle cell disease and moyamoya disease (Dlamini and Kirkham, Current Opinion in Pediatrics 21(6):751–761, 2009). Consequently, the importance of screening for subclinical strokes in these populations is now recognized as viable and efficacious. As a result, there is also a growing interest in studying neuropsychological outcomes following vascular injury and disease in infancy, childhood, and adolescence. As for other medical conditions, etiology and outcomes may be vastly different in children than in adults with the same vascular diagnoses due, in part, to the strong influence of dynamic developmental factors and a child’s evolving brain maturity. Despite the widely held belief that increased plasticity insulates the developing brain from the effects of injury and disease, there is considerable evidence that early onset neurovascular conditions are associated with significant neurological and neuropsychological morbidity (Dennis et al., Neuroscience and Biobehavioral Reviews 37:2760, 2013; Fuentes et al., Child Neuropsychology 22:37–41, 2014). This chapter summarizes the literature on congenital heart disease, ischemic and hemorrhagic stroke, sickle cell disease, moyamoya disease, and other vasculopathies in neonates, children, and adolescents. We also highlight important developmental considerations that are unique to pediatric populations in general, and to pediatric vascular conditions specifically.

Although pediatric stroke is associated with higher survival rates compared with adult stroke, a substantial body of evidence indicates significant neuropsychologic morbidity in pediatric stroke survivors. Neuroplasticity does not guarantee good outcome in children. The general trends observed in the literature are reviewed as is the profile observed in common causes of pediatric stroke: congenital heart disease, moyamoya disease, and sickle cell disease. The neuropsychologic profile of pediatric stroke patients is heterogeneous due to the multiplicity of associated causes. Stroke in early infancy and large strokes are associated with cognitive impairment while more limited disorders, such as phasic deficit, are observed in childhood stroke. Executive dysfunction is common in pediatric stroke, but social interaction skills are usually preserved. Congenital heart disease and sickle cell disease are associated with global neuropsychologic dysfunction while cognition is usually preserved in moyamoya. Executive dysregulation is instead more frequently reported in this population. Further study of maladaptive processes after pediatric stroke will allow identification of predictors of functional and neuropsychologic outcomes and permit personalization of care.

Current research suggests that pediatric stroke is associated with a reduction in intellectual functioning. However, less is known about academic achievement and the contribution of specific executive functions to math and literacy in this population. The current study investigates behavioral ratings of executive functioning and their relationship to math and spelling performance in children with a history of unilateral arterial ischemic stroke.Thirty-two pediatric patients with stroke (MRelative to controls, stroke participants demonstrated significantly poorer functioning in math, spelling, metacognition, and behavioral-regulation. Pencil and paper arithmetic was particularly challenging for the stroke group, with 40% of patients reaching levels of clinical impairment. Hierarchical regression in stroke participants further revealed that metacognition was a robust predictor of academic deficits. Stroke occurring in later childhood and affecting cortical and subcortical brain regions also presented as potential clinical risk factors.Children with stroke were especially vulnerable to math achievement deficits. Metacognition made a substantial contribution to academic achievement abilities among stroke patients, and results underscore the importance of early metacognitive skills in the completion of schoolwork. Results also emphasize that pediatric stroke patients are a heterogeneous group with regard to functioning and that there is value in examining standard score distributions of clinical participant samples.

Dystonia is a movement disorder that involves excessive, involuntary muscle contractions resulting in repetitive movements and/or abnormal posturing. One common cause of unilateral dystonia in childhood is ischemic stroke involving the basal ganglia and/or thalamus. Virtually nothing is known about neuropsychological outcomes in children who have dystonia following basal ganglia stroke. The present study explored whether or not children with secondary dystonia experience additional cognitive challenges when compared to children with similar patterns of brain injury, but no dystonia. We examined intellectual function, academics, and several aspects of executive function in children with unilateral basal ganglia stroke during childhood, comparing those with dystonia and those without. Although groups did not differ in terms of lesion size, we found significantly lower performance on measures of verbal and nonverbal reasoning, inhibitory control, and academic ability in children with secondary dyston...

This cross-sectional retrospective clinical research study examines a large group of children followed within a pediatric stroke program and a developmental attention-deficit/hyperactivity disorder (ADHD) clinic at the Hospital for Sick Children, between May 2004 and June 2016. All children with a history of stroke who participated in a neuropsychological assessment between the ages of 4 and 18 years were considered for inclusion. From a sample of 275 participants with a history of stroke, 36 children (13.1%) received a diagnosis of secondary ADHD. Children with secondary ADHD were younger at the time of stroke and more likely to be identified as having a presumed perinatal stroke and persistent seizures than children without secondary ADHD diagnoses. There were no differences in pattern of lesion, size, or laterality between children who developed secondary ADHD and those who did not. Children with secondary ADHD had the lowest scores across all cognitive and academic measures compared to children with stroke-only and developmental ADHD. Findings highlight the added risk of receiving a diagnosis of secondary ADHD following pediatric stroke. Implications for future research and directed intervention are discussed.

The majority of pediatric neuropsychological stroke research has focused on perinatal stroke outcomes given its relative frequency. Meanwhile, childhood-onset stroke is under-represented in the literature, resulting in limited knowledge about its neurocognitive sequelae. This retrospective study examined cognitive outcomes in children and youth (n = 27) with childhood arterial ischemic stroke (stroke occurring between 29 days and 18 years of life) isolated to the cortical region. Intellectual, academic, language, visual-perception, visual-motor integration, fine motor coordination, and executive function scores were examined relative to normative means. Results indicate that although these children are doing well in terms of general intellectual ability, they demonstrate lower scores on tasks of processing speed and fine motor coordination. Exploratory analysis also revealed that of the personal and neurologic factors examined, age at stroke was positively correlated with perceptual reasoning and fine motor control, age at assessment was negatively correlated with math calculation abilities, and maternal education was positively correlated with working memory and parent-reported behavioral regulation and impulse inhibition abilities. While neurologic variables were not predictive of cognitive neuropsychological outcomes, those with significant poorer performance had higher rates of medium/large, right-sided lesions with frontal lobe involvement. Our results highlight the overall resilience of the injured developing brain but also the vulnerability of specific cognitive skills within this unique population.

The basal ganglia are important for movement and executive function, but its contribution to language is less understood. This study explored language outcomes associated with childhood basal ganglia stroke. A detailed language coding scheme, which examined expressive and receptive language, verbal fluency, narrative discourse, pragmatic/applied language, and academics, was developed from qualitative and quantitative data acquired from neuropsychological testing and reports. Overall intellectual functioning and verbal comprehension was in the average range. Twelve participants had psychological diagnoses, including Learning Disorder. No one had a Language Disorder diagnosis. Among the 18 children who did not receive a diagnosis, many exhibited language issues in the mild to severe range according to our coding scheme. These children had higher-order language difficulties in verbal fluency, narrative, and pragmatic language rather than overt expressive difficulties noted in Diagnostic and Statistical Manual (DSM) diagnostic criteria. There was an association between infarct size and ESL/immersion education, math performance, and presence of a psychological diagnosis. Psychological diagnosis was also associated with literacy skills. The results highlight that language issues following basal ganglia stroke may not be fully captured by standardized neuropsychological tests and psychological diagnoses. Findings reinforce the need to integrate quantitative and qualitative findings when examining language functioning.

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).

Introduction: The balance of plasticity versus vulnerability to acute ischemic injury in the immature brain across development remains controversial. We aimed to investigate the relationship between age at stroke and lesion location and volume with long-term outcomes following arterial ischaemic stroke (AIS). Hypothesis: Age at stroke onset interacts with lesion location and volume to predict long-term neurological outcomes. Methods: In a single-centre prospective study, children aged term birth to 18 years with acute symptomatic AIS from 1992 to 2016 who underwent neuroimaging (CT/MRI) within 14 days of stroke onset were studied. Long term outcomes were assessed with the Pediatric Stroke Outcome Measure (PSOM). Outcomes were defined as normal (score 0-0.5 on 1-5 PSOM subscales), otherwise abnormal. AIS lesions were classified based on major (anterior, middle, posterior, other) cerebral arteries and branch sub-territories (e.g. MCA-lateral lenticulostriate). For each vascular territory, the proportion of infarcted brain was estimated as ‘small’ (≤50% territory) or ‘large’ (>50% territory). Factors predictive of long term outcomes were evaluated using logistic regression models. Results: Among 285 children, median age at stroke onset was 22 months (IQR: 0.03-101), 41% were females. PSOM scoring at median 9 years (IQR: 4-15) post-stroke was normal in 61%. Abnormal outcome was associated with age 1-4 years (OR=2.2, 95% CI: 1.02-4.9), ‘large’ proportion infarcts involving cortex within any major artery territory (OR=6.1, 95% CI: 2.6-14.4), and subcortical infarcts: OR= 2.7 (95% CI: 1.4-5.4) for small proportion infarcts; OR=8.3 (95% CI: 4.2-16.6) for large proportion infarcts in either medial or lateral lenticulostriate arteries; OR=15.8 (95% CI: 5-50.3) for large volume lesions in both lenticulostriate territories. In multivariate logistic regression, age, cortical and subcortical infarcts predicted long-term neurologic outcomes. Conclusions: In addition to age at stroke onset, infarct location including volume plays a key role in predicting long-term neurological outcomes in children. While the relationship between age and neurological outcomes seems to be bimodal, a linear effect of lesion volume on stroke outcomes is evident.

Introduction: Sleep-related breathing disorders (SRBDs) in childhood are associated with adverse outcomes including developmental and behavioral disorders. The association of SRBDs with pediatric stroke is poorly understood. We aimed to assess the risk of SRBDs in children with stroke or non-stroke high risk arteriopathies (NSA) and to evaluate their association with cognitive and behavioral function. Hypothesis: Children with stroke or NSA have a higher rate of SRBD and associated poor cognitive and behavioral function. Methods: We conducted a cross-sectional study of children with arterial ischemic stroke (AIS), cerebral sinovenous thrombosis (CSVT) and NSA such as moyamoya. All children completed the Pediatric Sleep Questionnaire (PSQ). High risk for SRBDs was defined as a PSQ score ≥0.33. Children completed the Adaptive Behavior Assessment System (ABAS-2) parent and teacher forms and/or The Wechsler Intelligence Scale for Children (WISC-V) within 1 year of completing the PSQ. Results: Overall, 213 were included (54% males, median age: 9 years [IQR 5-14]). Forty-nine children (23%) were at high risk of SRBDs. The risk of SRBDs did not vary with age, gender or stroke subtype: AIS (25%), CSVT (20%), other stroke subtypes (20%) and NSA (24%) (p= 0.8). Children with SRBD had higher mean BMI z-scores (0.9±1.5 vs 0.4±1.3, p=0.06). Twenty-four and 15 children underwent ABAS-2 parent and teacher assessment, respectively. The global adaptive composite scores of both forms significantly correlated with the total PSQ scores (r s =-0.6, ps =-0.63, p=0.01 for the teacher form). Correlation coefficients remained statistically significant upon controlling for BMI. In 19 children who completed the WISC-V, those at risk of SRBDs (n=7) had significantly lower Full Scale IQ (70±24.7 vs 91±17, p=0.03), processing speed (55±44 vs 96±19, p=0.02) and working memory (77±23 vs 98±15, p=0.02) compared to children at no risk. Cognitive and behavioral function did not differ across stroke subtypes. Conclusions: Pediatric stroke or NSA is commonly associated with SRBDs which in turn is linked to significant decline in cognitive and behavioral function. Further studies are required to understand the underlying pathological mechanisms.

Introduction: Neonatal arterial ischemic stroke (NAIS) is the most common form of childhood stroke. Unlike adults with stroke, children with left middle cerebral artery (MCA) NAIS seldom become aphasic, although adolescence often reveals the emergence of higher-order language deficits. Functional language networks may be predictive of later-emerging language outcomes, and can aid to identify at-risk children with NAIS. Methods: Five neurotypical children (2F; 5 RH; mean 12.7 ± 2.6 years), and five children with unilateral-MCA NAIS (3F; 5 RH; mean 11.3 ± 2.0 years) listened to semantically correct and incorrect sentences while magnetoencephalography (MEG) was recorded. Task-related functional connectivity was calculated using the phase lag index (PLI) across regions of interest. The relationship between the functional brain networks and language skill was examined. Results: Neurotypical children showed increased global functional connectivity for semantically correct sentences 1.8 to 2.0 seconds from stimulus onset in the theta band (4-7 Hz; p p p Conclusions: These results suggest reorganization of expected unilateral and frontal language networks towards a bilateral and temporal distribution following stroke, with less reliance on traditional language nodes. Such reorganization may underlie the language ability trajectory of children with neonatal MCA stroke. Reorganization of functional brain networks may be used as a predictive marker for language development following neonatal stroke, which can ultimately guide precision medicine and improve long-term outcomes.

To assess the prevalence of obstructive sleep apnea syndrome (OSAS) in children with arterial ischemic stroke (AIS) and to evaluate its association with neuropsychological outcomes.We conducted a cross-sectional study of sleep health and neuropsychological outcome in children with AIS. A consecutive cohort of children attending a stroke clinic were assessed using a standardized pediatric sleep questionnaire (PSQ) and standardized measures of pediatric stroke outcome and intellectual, executive and adaptive function. High risk for OSAS was defined as PSQ score ≥0.33.Overall, 102 children were included (55% males, median age: 9 years [interquartile-range [IQR]: 6-14]). The prevalence of OSAS in children with AIS was significantly higher compared to published normative prevalence rate (25.5% vs 5%, p 0.001). Children with OSAS were more likely to have infarcts affecting both the anterior and posterior circulation (37.5% vs 9.5%, p = 0.021). In addition, children with OSAS had significantly higher median Pediatric Stroke Outcome Measure (PSOM) scores (2 [IQR: 0-2] vs 1 [IQR: 1-3.5], p = 0.01) and were more likely to be prescribed concomitant medications affecting sleep architecture (50% vs 22.4%, p = 0.007). OSAS was associated with significantly lower scores on intellectual, memory, cognitive, behavioral, attention, executive and adaptive function scales. The association between PSQ and intellectual ability and working memory remained statistically significant upon controlling for potential confounding factors including stroke related characteristics (neurologic impairment and arterial territory).The prevalence of OSAS in children with AIS compared to healthy controls is significantly elevated and is associated with poor neuropsychological outcomes. We highlight the importance of regular screening for OSAS - a modifiable risk factor - in children with AIS. The specific risk factors for OSAS and the potential benefits of therapeutic interventions in this patient population warrant further investigation.

There is a dearth of research examining working memory (WM) following pediatric arterial ischemic stroke (AIS). This study assesses the WM patterns of 32 children, aged 6 to 14 years, with a history of unilateral AIS and 32 controls using a paradigm based on Baddeley and Hitch's multi-component WM model. The results indicate compromised WM in children with AIS relative to controls and parent reports confirm higher rates of dysfunction. Supplementary analyses of impairment confirm higher rates in children with AIS, ranging from 31.25% to 38.70% on performance-based measures and 50.00% on parent reports, compared to 0.00% to 21.88% on performance-based measures in controls and 15.63% on parent reports. Continual follow-up is recommended given that a subset of children with stroke appear to be at risk for WM impairment. Moreover, the subtle nature of WM challenges experienced by many children who have experienced a stroke increases the likelihood that WM impairment could go undetected. The long-term trajectories of WM in the pediatric stroke population remains unknown and future studies are needed to track changes in WM functioning over time.

Childhood arterial ischemic stroke often involves basal ganglia and thalamus but little is known about neuropsychological outcomes in this group. We examined intellectual ability, academics, attention, executive function, and psychological diagnoses in children and adolescents (6–20 years of age) with childhood stroke involving the basal ganglia (n = 32) or thalamus (n = 12). Intellectual ability was age-appropriate but working memory was significantly lower than expected. Compared to the normative mean, the stroke group exhibited significantly weaker performance in reading comprehension, math fluency, attention, and greater challenges with executive function. Children with basal ganglia stroke had weaker working memory and were more likely to receive diagnoses of Attention Deficit Hyperactivity Disorder and Anxiety Disorder than those with thalamic stroke. Lesion size was most important in predicting working memory ability, whereas age at stroke and age at test were important in predicting academ...

This study aimed to create a specific questionnaire (Parent Experiences Questionnaire) about early experiences, service involvement, and needs of children and parents following neonatal brain injury that could be used to inform clinical care and needed interventions.A mixed-method design was utilized, engaging in both qualitative and quantitative methods across three phases. Phase 1 employed participatory design involving 12 parent and clinician participants in semi-structured interviews to address main topics, item importance, and overall impressions of the questionnaire. In phase 2, the questionnaire was piloted by 32 parents. Post hoc revisions added four questions to address current parent and child therapeutic needs in phase 3.The final questionnaire yielded 24 items addressing topics of early communication between parent and clinicians, early intervention services, efficacy, and barriers in optimizing the child's development and parents' experience. The questionnaire was reviewed positively by a group of parents in phase 2 and demonstrated good acceptance, online feasibility, stability, and association with current parental mental health and child development.This investigation offers a valuable new questionnaire to inform clinical care regarding discussions with parents about neonatal brain injury, evaluate the perceived efficacy of early intervention services, and guide relevant future intervention efforts.

We report the neuropsychological profile of a 6-year-old girl with Wiskott-Aldrich syndrome, a rare X-linked immunodeficiency disorder associated with thrombocytopenia, eczema, recurrent infections, and malignancy. Wiskott-Aldrich syndrome occurs almost exclusively in males and is extremely rare in females, with no known research focused on cognitive and academic functioning in this population. Our patient was referred due to concerns about her memory and academic functioning. She had a history of progressive thrombocytopenia and hematopoietic stem cell transplantation at age 15 months. Standardized measures of intellectual ability, language, visual-spatial and visual-motor skills, attention, memory, and academic achievement were administered. The results showed average to above-average performance in multiple areas of cognitive and academic functioning, with weaknesses in phonological awareness and rapid naming. The advent of hematopoietic stem cell transplantation has led to considerable improvement in the long-term prognosis of children with Wiskott-Aldrich syndrome. Although the impact of this syndrome and related conditions on neurocognitive development is presently unknown, this case highlights both the importance of considering base rates for commonly occurring conditions and the significant role neuropsychology can play in identifying cognitive strengths and weaknesses in the context of the developing brain.

BACKGROUND AND PURPOSE: There is a critical need for a reliable and clinically feasible imaging technique that can enable prognostication and selection for revascularization surgery in children with Moyamoya disease. Blood oxygen level–dependent MR imaging assessment of cerebrovascular reactivity, using voluntary breath-hold hypercapnic challenge, is one such simple technique. However, its repeatability and reliability in children with Moyamoya disease are unknown. The current study sought to address this limitation. MATERIALS AND METHODS: Children with Moyamoya disease underwent dual breath-hold hypercapnic challenge blood oxygen level–dependent MR imaging of cerebrovascular reactivity in the same MR imaging session. Within-day, within-subject repeatability of cerebrovascular reactivity estimates, derived from the blood oxygen level–dependent signal, was computed. Estimates were associated with demographics and intellectual function. Interrater reliability of a qualitative and clinically applicable scoring scheme was assessed. RESULTS: Twenty children (11 males; 12.1 ± 3.3 years) with 30 MR imaging sessions (60 MR imaging scans) were included. Repeatability was “good” on the basis of the intraclass correlation coefficient (0.70 ± 0.19). Agreement of qualitative scores was “substantial” (κ = 0.711), and intrarater reliability of scores was “almost perfect” (κ = 0.83 and 1). Younger participants exhibited lower repeatability ( P = .027). Repeatability was not associated with cognitive function ( P > .05). However, abnormal cerebrovascular reactivity was associated with slower processing speed ( P = .015). CONCLUSIONS: Breath-hold hypercapnic challenge blood oxygen level–dependent MR imaging is a repeatable technique for the assessment of cerebrovascular reactivity in children with Moyamoya disease and is reliably interpretable for use in clinical practice. Standardization of such protocols will allow further research into its application for the assessment of ischemic risk in childhood cerebrovascular disease.

Background: In this case series, functional magnetic resonance imaging was used to examine brain networks that mediate different aspects of language function in 4 young adults (17-22 years) with a history of left middle cerebral artery (MCA) stroke in childhood (40 years of age). Although it is widely believed that altered lateralization patterns are more likely to occur following early brain injuries compared with later brain injuries, the presumed plasticity of the young brain has been challenged in recent years, particularly in the domain of language. Methods: We explored this issue by contrasting the brain activation patterns of individuals with childhood left MCA stroke and adult left MCA stroke while performing two language tasks: verb generation and picture-word matching. Importantly, both groups showed significant recovery of language function, based on standard clinical indicators. Results: Controls showed left lateralized activation for both tasks, although much more pronounced for verb generation. Adult stroke patients also showed left lateralization for both tasks, though somewhat weaker than controls. Childhood stroke patients exhibited significantly weaker lateralization than the adult group for verb generation, but there was no significant group difference for picture-word matching. Conclusions: These preliminary findings suggest that successful reorganization of language function is more likely to involve bilateral recruitment following left MCA stroke in childhood than in adulthood. Of importance, although childhood stroke patients had primarily subcortical lesions, there were substantial alterations in cortical activation patterns.

Our understanding of cognitive and behavioral outcomes of perinatal and childhood stroke is rapidly evolving. A current understanding of cognitive outcomes following pediatric stroke can inform prognosis and direct interventions and our understanding of plasticity in the developing brain. However, our understanding of these outcomes has been hampered by the notable heterogeneity that exists amongst the pediatric stroke population, as the influences of various demographic, cognitive, neurological, etiological, and psychosocial variables preclude broad generalizations about outcomes in any one cognitive domain. We therefore aimed to conduct a detailed overview of the published literature regarding the effects of age at stroke, time since stroke, sex, etiology, lesion characteristics (i.e., location, laterality, volume), neurologic impairment, and seizures on cognitive outcomes following pediatric stroke. A key theme arising from this review is the importance of interactive effects among variables on cognitive outcomes following pediatric stroke. Interactions particularly of note include the following: (a) age at Stroke x Lesion Location; (b) Lesion Characteristics (i.e., volume, location) x Neurologic Impairment; (c) Lesion Volume x Time Since Stroke; (d) Sex x Lesion Laterality; and (e) Seizures x Time Since Stroke. Further, it appears that these relationships do not always apply uniformly across cognitive domains but, rather, are contingent upon the cognitive ability in question. Implications for future research directions are discussed.

Background Hypercapnic-challenge blood oxygen level–dependent magnetic resonance imaging cerebrovascular reactivity (CVR), measures the regional perfusion response to altered carbon dioxide. CVR correlates with the tissue-level microvascular dysfunction and ischemic risk. Among children with arterial ischemic stroke, transient cerebral arteriopathy (TCA) is a frequent, nonprogressive unilateral intracranial arteriopathy, which typically results in basal ganglia infarction and chronic cerebral artery stenosis. Therefore TCA provides a model for studying the consequences of chronic nonprogressive stenosis using CVR and intellectual outcome. We hypothesized that children with TCA and chronic nonprogressive intracranial artery stenosis have impaired CVR distal to the stenosis and associated cognitive impairment. Methods We studied children with a prior diagnosis of TCA as defined by infarction limited to the basal ganglia, internal capsule, or both; and significant (greater than 50% diameter) residual stenosis of the supraclinoid internal carotid artery, its proximal branches or both. All children had CVR, intellectual function, and infarct volumes quantified. Results We performed CVR studies in five children at mean 8.96 years (3.33 to 14.58 years) poststroke. Impaired CVR was limited to the infarct zone and adjacent white matter in most children. Intellectual function was broadly average in all but one subject. Conclusions In children with typical TCA, ipsilateral cortical CVR and intellectual function seem to be preserved despite persistent arterial stenosis in the majority. These findings suggest that chronic revascularization strategies in these children may not be indicated and require further exploration in a larger cohort of children.

There is a dearth of research examining working memory (WM) following pediatric arterial ischemic stroke (AIS). This study assesses the WM patterns of 32 children, aged 6 to 14 years, with a history of unilateral AIS and 32 controls using a paradigm based on Baddeley and Hitch's multi-component WM model. The results indicate compromised WM in children with AIS relative to controls and parent reports confirm higher rates of dysfunction. Supplementary analyses of impairment confirm higher rates in children with AIS, ranging from 31.25% to 38.70% on performance-based measures and 50.00% on parent reports, compared to 0.00% to 21.88% on performance-based measures in controls and 15.63% on parent reports. Continual follow-up is recommended given that a subset of children with stroke appear to be at risk for WM impairment. Moreover, the subtle nature of WM challenges experienced by many children who have experienced a stroke increases the likelihood that WM impairment could go undetected. The long-term trajectories of WM in the pediatric stroke population remains unknown and future studies are needed to track changes in WM functioning over time.

Aim Moyamoya vasculopathy is characterized by progressive stenosis of the major arteries of the Circle of Willis, resulting in compromised cerebral blood flow and increased risk of stroke. The objectives of the current study were to examine intellectual and executive functioning of children with moyamoya and to evaluate the impact of moyamoya type, stroke (clinical or silent), vasculopathy laterality, and disease duration on neurocognitive abilities. Method Thirty pediatric participants (mean age 10y 10mo, SD 4y; 18 females, 12 males) completed age-appropriate Wechsler Intelligence Scales before any therapeutic revascularization procedures. Reports of executive function were obtained from parents and teachers using the Behavior Rating Index of Executive Function. Results Children with moyamoya scored significantly lower than the test standardization samples on all indices of intelligence and ratings of executive functioning (p