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Objective: The brain arteriovenous malformation (BAVM) nidus compactness score (CS), determined on angiography, predicts BAVM recurrence after surgical resection among children with sporadic BAVMs. We measured the angiographic CS for BAVMs among children with hereditary hemorrhagic telangiectasia (HHT) to determine CS characteristics in this population. Methods: A pediatric interventional neuroradiologist reviewed angiograms to determine the CS of BAVMs in children with HHT recruited to the BVMC. CS is based on overall nidus and perinidal anomalous vessel compactness. CS categories included 1 = diffuse nidus, 2 = intermediate nidus, and 3 = compact nidus. Results: Forty-eight of 78 children (61.5%) with HHT and brain vascular malformations had a conventional angiogram; 47 (97.9%) angiograms were available. Fifty-four BAVMs were identified in 40 of these 47 children (85.1%). Of 54 BAVMs in children with HHT, CS was 1 in 7 (13%), 2 in 29 (53.7%), and 3 in 18 BAVMs (33.3%) compared with CS of 1 in six (26.1%), 2 in 15 (65.2%), and 3 in 2 BAVMs (8.7%) among 23 previously reported children with sporadic BAVMs, p = 0.045 (Fisher's exact). Seven children with HHT had intracranial hemorrhage: 4 had CS = 3, 1 had CS = 2, and 2 had CS = 1. Conclusions: A range of CSs exists across HHT BAVMs, suggesting it may be an angiographic measure of interest for future studies of BAVM recurrence and hemorrhage risk. Children with HHT may have more compact niduses compared to children with sporadic BAVMs. Additional research should determine whether CS affects hemorrhage risk or post-surgical recurrence risk in HHT-associated BAVMs, which could be used to direct BAVM treatment. [ABSTRACT FROM AUTHOR]

Approximately 10% of people with hereditary hemorrhagic telangiectasia (HHT) have brain vascular malformations (VMs). Few reports describe de novo brain VM formation. International HHT Guidelines recommend initial brain VM screening upon HHT diagnosis in children but do not address rescreening. We aimed to confirm whether brain VMs can form de novo in patients with HHT. The Brain Vascular Malformation Consortium HHT project is a 17-center longitudinal study enrolling patients since 2010. We analyzed the database for de novo VMs defined as those detected (1) on follow-up neuroimaging in a patient without previous brain VMs or (2) in a location distinct from previously identified brain VMs and reported those in whom a de novo VM could be confirmed on central neuroimaging review. Of 1909 patients enrolled, 409 (21%) had brain VMs. Seven patients were recorded as having de novo brain VMs, and imaging was available for central review in four. We confirmed that three (0.7% of individuals with brain VMs) had de novo brain VMs (two capillary malformations, one brain arteriovenous malformation) with intervals of six, nine, and 13 years from initial imaging. Two with de novo brain VMs were <18 years. The fourth patient, a child, did not have a de novo brain VM but had a radiologically confirmed increase in size of an existing brain arteriovenous malformation. Brain VMs can, albeit rarely, form de novo in patients with HHT. Given the potential risk of hemorrhage from brain VMs, regular rescreening in patients with HHT may be warranted. [ABSTRACT FROM AUTHOR]

Following a pediatric stroke, outcome measures selected for monitoring functional recovery and development vary widely. We sought to develop a toolkit of outcome measures that are currently available to clinicians, possess strong psychometric properties, and are feasible for use within clinical settings. A multidisciplinary group of clinicians and scientists from the International Pediatric Stroke Organization comprehensively reviewed the quality of measures in multiple domains described in pediatric stroke populations including global performance, motor and cognitive function, language, quality of life, and behavior and adaptive functioning. The quality of each measure was evaluated using guidelines focused on responsiveness and sensitivity, reliability, validity, feasibility, and predictive utility. A total of 48 outcome measures were included and were rated by experts based on the available evidence within the literature supporting the strengths of their psychometric properties and practical use. Only three measures were found to be validated for use in pediatric stroke: the Pediatric Stroke Outcome Measure, the Pediatric Stroke Recurrence and Recovery Questionnaire, and the Pediatric Stroke Quality of Life Measure. However, multiple additional measures were deemed to have good psychometric properties and acceptable utility for assessing pediatric stroke outcomes. Strengths and weaknesses of commonly used measures including feasibility are highlighted to guide evidence-based and practicable outcome measure selection. Improving the coherence of outcome assessment will facilitate comparison of studies and enhance research and clinical care in children with stroke. Further work is urgently needed to close the gap and validate measures across all clinically significant domains in the pediatric stroke population. [ABSTRACT FROM AUTHOR]

Objective: Severe complications of severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) include arterial ischemic stroke (AIS) in adults and multisystem inflammatory syndrome in children. Whether stroke is a frequent complication of pediatric SARS‐CoV‐2 is unknown. This study aimed to determine the proportion of pediatric SARS‐CoV‐2 cases with ischemic stroke and the proportion of incident pediatric strokes with SARS‐CoV‐2 in the first 3 months of the pandemic in an international cohort. Methods: We surveyed 61 international sites with pediatric stroke expertise. Survey questions included: numbers of hospitalized pediatric (≤ 18 years) patients with SARS‐CoV‐2; numbers of incident neonatal and childhood ischemic strokes; frequency of SARS‐CoV‐2 testing for pediatric patients with stroke; and numbers of stroke cases positive for SARS‐CoV‐2 from March 1 to May 31, 2020. Results: Of 42 centers with SARS‐CoV‐2 hospitalization numbers, 8 of 971 (0.82%) pediatric patients with SARS‐CoV‐2 had ischemic strokes. Proportions of stroke cases positive for SARS‐CoV‐2 from March to May 2020 were: 1 of 108 with neonatal AIS (0.9%), 0 of 33 with neonatal cerebral sinovenous thrombosis (CSVT; 0%), 6 of 166 with childhood AIS (3.6%), and 1 of 54 with childhood CSVT (1.9%). However, only 30.5% of neonates and 60% of children with strokes were tested for SARS‐CoV‐2. Therefore, these proportions represent 2.9, 0, 6.1, and 3.0% of stroke cases tested for SARS‐CoV‐2. Seven of 8 patients with SARS‐CoV‐2 had additional established stroke risk factors. Interpretation: As in adults, pediatric stroke is an infrequent complication of SARS‐CoV‐2, and SARS‐CoV‐2 was detected in only 4.6% of pediatric patients with ischemic stroke tested for the virus. However, < 50% of strokes were tested. To understand the role of SARS‐CoV‐2 in pediatric stroke better, SARS‐CoV‐2 testing should be considered in pediatric patients with stroke as the pandemic continues. ANN NEUROL 2021;89:657–665 [ABSTRACT FROM AUTHOR]

Background: We determined the frequency of cerebrovascular malformations in a pediatric cohort with hereditary hemorrhagic telangiectasia.Methods: Retrospective cohort study of 54 children diagnosed with hereditary hemorrhagic telangiectasia at a tertiary care center. All neuroimaging was reviewed to assess for number and types of cerebrovascular malformations and for intracerebral hemorrhage and arterial ischemic stroke. Clinical charts were reviewed for clinical manifestations, genetic mutation, and clinically evident intracerebral hemorrhages and arterial ischemic strokes.Results: Among 54 children with hereditary hemorrhagic telangiectasia with a median age of 3.5 years (interquartile range 0.4 to 7.9 years) at diagnosis, neuroimaging was performed in 52 (96.3%) at a median age of 5.2 years (interquartile range 1.8 to 9 years). Fourteen of 52 imaged children (26.9%) had cerebrovascular malformations. Cerebrovascular malformations included arteriovenous malformations, arteriovenous fistulas, vein of Galen malformations, and developmental venous anomalies. Six of the 14 children with cerebrovascular malformations (42.9%) had multiple malformations. Three children developed new cerebral arteriovenous malformations over time. Six children (11.1%) had clinically evident intracerebral hemorrhage, arterial ischemic stroke, or transient ischemic attack. The three children with intracerebral hemorrhage presented at young ages (4.3 to 7.7 years).Conclusions: More than a quarter of children with hereditary hemorrhagic telangiectasia who were imaged had cerebrovascular malformations, and overt stroke occurred in more than 10%. Intracerebral hemorrhages can occur in pediatric hereditary hemorrhagic telangiectasia patients at young ages, and new cerebral arteriovenous malformations may develop over time. Early screening with neuroimaging including neurovascular imaging as well as repeat neuroimaging may be warranted in children with hereditary hemorrhagic telangiectasia. [ABSTRACT FROM AUTHOR]

Subdural hemorrhages (SDHs) in the pediatric population are associated with a high mortality and morbidity and may present in the context of abusive head trauma. Diagnostic investigations for such cases often include evaluation for rare genetic and metabolic disorders that can have associated SDH. Sotos syndrome is an overgrowth syndrome associated with macrocephaly and increased subarachnoid spaces and rarely with neurovascular complications. Here, we report two cases of Sotos syndrome, one with SDH during infancy who underwent repeated evaluation for suspected child abuse prior to the Sotos syndrome diagnosis and the other with enlarged extra-axial cerebrospinal fluid spaces, demonstrating a possible mechanism for SDH development in this setting. These cases suggest that some individuals with Sotos syndrome may be at elevated risk of developing SDH in infancy and that Sotos syndrome should be on the differential diagnosis during a medical genetics evaluation in cases of unexplained SDH, especially in the setting of macrocephaly. [ABSTRACT FROM AUTHOR]

Background: Perihematomal edema (PHE) expansion rate may predict functional outcome following spontaneous intracerebral hemorrhage (ICH). We hypothesized that the effect of PHE expansion rate on outcome is greater for deep versus lobar ICH.Methods: Subjects (n = 115) were retrospectively identified from a prospective ICH cohort enrolled from 2000 to 2013. Inclusion criteria were age ≥ 18 years, spontaneous supratentorial ICH, and known onset time. Exclusion criteria were primary intraventricular hemorrhage (IVH), trauma, subsequent surgery, or warfarin-related ICH. ICH and PHE volumes were measured from CT scans and used to calculate expansion rates. Logistic regression assessed the association between PHE expansion rates and 90-day mortality or poor functional outcome (modified Rankin Scale > 2). Odds ratios are per 0.04 mL/h.Results: PHE expansion rate from baseline to 24 h (PHE24) was associated with mortality for deep (p = 0.03, OR 1.13[1.02-1.26]) and lobar ICH (p = 0.02, OR 1.03[1.00-1.06]) in unadjusted regression and in models adjusted for age (deep p = 0.02, OR 1.15[1.02-1.28]; lobar p = 0.03, OR 1.03[1.00-1.06]), Glasgow Coma Scale (deep p = 0.03, OR 1.13[1.01-1.27]; lobar p = 0.02, OR 1.03[1.01-1.06]), or time to baseline CT (deep p = 0.046, OR 1.12[1.00-1.25]; lobar p = 0.047, OR 1.03[1.00-1.06]). PHE expansion rate from baseline to 72 h (PHE72) was associated with mRS > 2 for deep ICH in models that were unadjusted (p = 0.02, OR 4.04[1.25-13.04]) or adjusted for ICH volume (p = 0.02, OR 4.3[1.25-14.98]), age (p = 0.03, OR 5.4[1.21-24.11]), GCS (p = 0.02, OR 4.19[1.2-14.55]), or time to first CT (p = 0.03, OR 4.02[1.19-13.56]).Conclusions: PHE72 was associated with poor functional outcomes after deep ICH, whereas PHE24 was associated with mortality for deep and lobar ICH. [ABSTRACT FROM AUTHOR]

Hemorrhagic transformation can complicate both arterial ischemic stroke and cerebral sinus venous thrombosis. Risk factors for hemorrhagic transformation after adult arterial ischemic stroke include larger infarct volume, cardioembolic stroke, and anticoagulation in the acute period. Large hemorrhagic transformation in adults is associated with poor outcome. Therefore hemorrhagic transformation is used as a safety end point for most arterial ischemic stroke acute treatment and secondary prevention trials. Up to 30% of children with arterial ischemic stroke have hemorrhagic transformation, most of which are petechial. As in adults, large infarct size is the greatest predictor of hemorrhagic transformation, but in children, acute anticoagulation is not a clear predictor of hemorrhage. As use of acute endovascular interventions for arterial ischemic stroke has expanded in adults, these therapies have also been used in some teenagers and even younger children. More information, including safety data with end points like hemorrhagic transformation, is needed in the pediatric population. In adults with cerebral sinus venous thrombosis, including those with hemorrhagic transformation, acute anticoagulation is associated with better outcomes and is the standard of care. Some hemorrhagic transformation may be evident at baseline in over half of children and neonates with cerebral sinus venous thrombosis. Anticoagulation-associated hemorrhage in pediatric cerebral sinus venous thrombosis occurs in about 10% of children but is not clearly related to outcome, whereas lack of anticoagulation may be associated with clot propagation and worse outcomes. This review provides background on hemorrhagic transformation of ischemic stroke in adults and summarizes literature regarding hemorrhagic transformation of pediatric arterial ischemic stroke and cerebral sinus venous thrombosis, with a focus on implications for acute treatment and outcome. [ABSTRACT FROM AUTHOR]

Background: Patients with large territory infarction are at high risk of cerebral edema and neurological deterioration, including death. Preclinical studies have shown that a continuous infusion of glyburide blocks edema formation and improves outcome. We hypothesize that treatment with RP-1127 (Glyburide for Injection) reduces formation of brain edema in patients after large anterior circulation infarction.Methods: GAMES-RP is a prospective, randomized, double-blind, multicenter trial designed to evaluate RP-1127 in patients at high risk for the development of malignant cerebral edema. The study population consisted of subjects with a clinical diagnosis of acute severe anterior circulation ischemic stroke with a baseline diffusion-weighted image lesion between 82 and 300 cm(3) who are 18-80 years of age. The target time from symptom onset to start of study infusion was ≤10 h. Subjects were randomized to RP-1127 (glyburide for injection) or placebo and treated with a continuous infusion for 72 h.Results: The primary efficacy outcome was a composite of the modified Rankin Scale and the incidence of decompressive craniectomy, assessed at 90 days. Safety outcomes were the frequency and severity of adverse events, with a focus on cardiac- and glucose-related serious adverse events.Conclusions: GAMES-RP was designed to provide critical information regarding glyburide for injection in patients with large hemispheric stroke and will inform the design of future studies. [ABSTRACT FROM AUTHOR]

Background: The purpose of this article is to aid practitioners in choosing appropriate neuroimaging for children who present with symptoms that could be caused by stroke.Methods: The Writing Group members participated in one or more pediatric stroke neuroimaging symposiums hosted by the Stroke Imaging Laboratory for Children housed at the Hospital for Sick Children in Toronto, Ontario, Canada. Through collaboration, literature review, and discussion among child neurologists with expertise diagnosing and treating childhood stroke and pediatric neuroradiologists and neuroradiologists with expertise in pediatric neurovascular disease, suggested imaging protocols are presented for children with suspected stroke syndromes including arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke.Results: This article presents information about the epidemiology and classification of childhood stroke with definitions based on the National Institutes of Health Common Data Elements. The role of imaging for the diagnosis of childhood stroke is examined in depth, with separate sections for arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke. Abbreviated neuroimaging protocols for rapid diagnosis are discussed. The Writing Group provides suggestions for optimal neuroimaging investigation of various stroke types in the acute setting and suggestions for follow-up neuroimaging. Advanced sequences such as diffusion tensor imaging, perfusion imaging, and vessel wall imaging are also discussed.Conclusions: This article provides protocols for the imaging of children who present with suspected stroke. [ABSTRACT FROM AUTHOR]

Objectives: Intracerebral hemorrhage is a devastating disorder with no current treatment. Whether perihematomal edema is an independent predictor of neurologic outcome is controversial. We sought to determine whether perihematomal edema expansion rate predicts outcome after intracerebral hemorrhage.Design: Retrospective cohort study.Setting: Tertiary medical center.Patients: One hundred thirty-nine consecutive supratentorial spontaneous intracerebral hemorrhage patients 18 years or older admitted between 2000 and 2013.Interventions: None.Measurements and Main Results: Intracerebral hemorrhage, intraventricular hemorrhage, and perihematomal edema volumes were measured from CT scans obtained at presentation, 24-hours, and 72-hours postintracerebral hemorrhage. Perihematomal edema expansion rate was the difference between initial and follow-up perihematomal edema volumes divided by the time interval. Logistic regression was performed to evaluate the relationship between 1) perihematomal edema expansion rate at 24 hours and 90-day mortality and 2) perihematomal edema expansion rate at 24 hours and 90-day modified Rankin Scale score. Perihematomal edema expansion rate between admission and 24-hours postintracerebral hemorrhage was a significant predictor of 90-day mortality (odds ratio, 2.97; 95% CI, 1.48-5.99; p = 0.002). This association persisted after adjusting for all components of the intracerebral hemorrhage score (odds ratio, 2.21; 95% CI, 1.05-4.64; p = 0.04). Similarly, higher 24-hour perihematomal edema expansion rate was associated with poorer modified Rankin Scale score in an ordinal shift analysis (odds ratio, 2.40; 95% CI, 1.37-4.21; p = 0.002). The association persisted after adjustment for all intracerebral hemorrhage score components (odds ratio, 2.07; 95% CI, 1.12-3.83; p = 0.02).Conclusions: Faster perihematomal edema expansion rate 24-hours postintracerebral hemorrhage is associated with worse outcome. Perihematomal edema may represent an attractive translational target for secondary injury after intracerebral hemorrhage. [ABSTRACT FROM AUTHOR]

Background: Recently a single-center study suggested that hypertension after stroke in children was a risk factor for mortality. Our goal was to assess the association between hypertension and outcome after arterial ischemic stroke in children from a large national sample.Methods: Using the Healthcare Cost and Utilization Project Kids' Inpatient Database, children (1-18 years) with a primary diagnosis of ischemic stroke (International Classification of Diseases, Ninth Revision [ICD-9] codes 433-437.1) who also had a diagnosis of elevated blood pressure (ICD-9 code 796.2) or hypertension (ICD-9 codes 401 and 405) from 2003, 2006, and 2009 were identified. Clinical characteristics, discharge outcomes, and length of stay were assessed. Multivariable logistic regression was used to assess the relationship between hypertension and in-hospital mortality or discharge outcomes.Results: Of 2590 children admitted with arterial ischemic stroke, 156 (6%) also had a diagnosis of hypertension. Ten percent of children with hypertension also had renal failure. Among patients with arterial ischemic stroke, hypertension was associated with increased mortality (7.4% vs. 2.8%; P = 0.01) and increased length of stay (mean 11 ± 17 vs. 7 ± 12 days; P = 0.004) compared with those without hypertension. After adjusting for age, sex, intubation, presence of a fluid and electrolyte disorder, and renal failure, children with hypertension had an increased odds of in-hospital death (odds ratio 1.2, 95% confidence interval [1.1-3.3, P = 0.04]).Conclusion: Hypertension was associated with an increased risk of in-hospital death for children presenting with arterial ischemic stroke. Further prospective study of blood pressure in children with stroke is needed. [ABSTRACT FROM AUTHOR]

Background Cerebral arteriopathy is a risk factor for incident and recurrent childhood AIS. There are no standardized criteria to quantify arteriopathy severity. Aims To evaluate a method of scoring severity of steno-occlusive arteriopathy in childhood arterial ischemic stroke ( AIS) and its association with recurrence. Methods In a single-center prospectively enrolled cohort of 49 children with first AIS and arteriopathy, a composite cerebrovascular stenosis score ( CVSS) was measured by two independent raters as the sum of stenosis scores in each of 18 intracranial large and medium arteries, where 0 = none; 1 = low-grade, 1-50%; 2 = high-grade, >50-99%; 3 = occlusion, 100%. Cox proportional-hazards models were used to determine the association of CVSS with recurrence. The analysis was stratified by presence or absence of moyamoya arteriopathy (syndrome or disease). Results At a median follow-up period of 2·5 years (range: 0·8-9), 18/49 children (37%) experienced a recurrence. Median time to recurrence was 0·2 (range: 0·02-2·8) years. Interrater agreement was good, with an intraclass correlation coefficient of 0·77 [95% confidence interval ( CI) 0·63-0·87, P < 0·001). Higher CVSS was associated with higher recurrence rate [hazard ratio ( HR) per point 1·09, 95% CI 1·04-1·16, P = 0·001]. Among those with moyamoya arteriopathy, CVSS was associated with recurrence ( HR per point of CVSS 1·11, 95% CI 1·03-1·19, P = 0·004), but there was no association in those without moyamoya arteriopathy ( HR per point of CVSS 0·91, 95% CI 0·75-1·09, P = 0·32). Conclusions The CVSS is a reliable measure of severity of steno-occlusive arteriopathy in childhood stroke. This preliminary study suggests that higher CVSS is associated with stroke recurrence in children with moyamoya arteriopathy. [ABSTRACT FROM AUTHOR]

Objective: Few data regarding causes and outcomes of haemorrhagic stroke (HS) in term neonates are available. We characterised risk factors, mechanism and short-term outcomes in term and late preterm neonates with acute HS. Design: Prospective cohort. Setting: Single-centre tertiary care stroke registry. Subjects Term and late preterm neonates (≥34 weeks gestation), born 2004-2010, with acute HS ≤28 days of life were identified, and clinical information was abstracted. Short-term outcomes were assessed via standardised neurological exam and rated using the Paediatric Stroke Outcome Measure (PSOM). Results Among 42 neonates, median gestational age was 39.7 weeks (IQR 38-40.7 weeks). Diagnosis occurred at a median of 1 day (IQR 0-7 days) after delivery. Twentyseven (64%) had intraparenchymal and intraventricular haemorrhage. Mechanism was haemorrhagic transformation of venous or arterial infarction in 22 (53%). Major risk factors included congenital heart disease (CHD), fetal distress and haemostatic abnormalities. Common presentations included seizure, apnoea, and poor feeding or vomiting. Acute hydrocephalus was common. Mortality was 12%. Follow-up occurred in 36/37 survivors at a median of 1 year (IQR 0.5-2.0 years). Among 17/36 survivors evaluated in stroke clinic, 47% demonstrated neurologic deficits. Deficits were mild (PSOM 0.5-1.5) in 9/36 (25%), and moderate-to-severe (PSOM ≥2.0) in 8/36 (22%). Conclusions: In our cohort with acute HS, most presented with seizures, apnoea and/or poor feeding. Fetal distress and CHD were common. Nearly two-thirds had intraparenchymal with intraventricular haemorrhage. Over half were due to haemorrhagic transformation of infarction. Short-term neurologic deficits were present in 47% of survivors. [ABSTRACT FROM AUTHOR]

Objective: Brain tumors are rare in infants who are younger than six months of age. These tumors can be challenging to treat surgically. We analyzed a modern series of patients treated by a multidisciplinary team at a tertiary care center and performed a literature review of this unique population. Methods: Retrospective clinical data were collected for patients surgically treated for intracranial mass lesions at The Children''s Hospital of Philadelphia from 1998 to 2007. Dermoid cysts and other skull-based lesions were excluded from the analysis. Results: Sixteen patients younger than six months of age underwent surgery for primary intracranial mass lesions. The median age of the patients at surgery was 5.2 months (range, 1.4-6 months of age). Children most often presented with a bulging fontanelle, hydrocephalus, or macrocephaly (seven patients). Vomiting was seen in five patients, cranial nerve palsies in one patient, and seizures in three patients. All patients had tumor resections and postoperatively were monitored in the intensive care unit. The final pathology consisted of atypical teratoid/rhabdoid tumor (three patients), primitive neuroectodermal tumor/medulloblastoma (three patients), choroid plexus papilloma (two patients), astrocytoma (two patients), ganglioglioma (two patients), desmoplastic infantile ganglioglioma (two patients), glioblastoma multiforme (one patient), and choroid plexus carcinoma (one patient). Two intraoperative deaths occurred. Of the surviving 14, a gross total resection was achieved in four. Adjuvant therapy was determined by a multidisciplinary team composed of neuro-oncology, neurosurgery, and radiation oncology. Seven patients were treated with chemotherapy, and one patient had proton beam therapy. Five-year overall survival was 45%. The eight surviving patients had neurological sequelae, and developmental outcome was variable. Conclusions: Brain tumors are uncommon in children younger than six months of age. Patients present with a variety of tumor pathologies. Children who survive have neurological sequelae. More studies are necessary to understand the impact that different treatment options, tumor pathology, and tumor location have on neurological outcome. [Copyright &y& Elsevier]

Previous studies of pediatric intracerebral hemorrhage have investigated isolated intraparenchymal hemorrhage. The authors investigated whether detailed assessment of intraventricular hemorrhage enhanced outcome prediction after intracerebral hemorrhage. They prospectively enrolled 46 children, full-term to 17 years, median age 2.7 years, with spontaneous intraparenchymal hemorrhage and/or intraventricular hemorrhage. Outcome was assessed with the King’s Outcome Scale for Childhood Head Injury. Twenty-six (57%) had intraparenchymal hemorrhage, 10 (22%) had pure intraventricular hemorrhage, and 10 (22%) had both. There were 2 deaths, both with intraparenchymal hemorrhage and intraventricular hemorrhage volume ≥4% of total brain volume. Presence of intraventricular hemorrhage was not associated with poor outcome, but hydrocephalus showed a trend (P = .09) toward poor outcome. In receiver operating characteristic curve analysis, combined intraparenchymal hemorrhage and intraventricular hemorrhage volume also showed a trend toward better outcome prediction than intraparenchymal hemorrhage volume alone. Although not an independent outcome predictor, future studies should assess intraventricular hemorrhage qualitatively and quantitatively. [ABSTRACT FROM AUTHOR]

Purpose: Population-based estimates of the annual incidence of childhood stroke range from 2 to 13 per 100,000 person-years. More than half of children who have had a stroke have long-term neurological sequelae. The goal of this article is to review recent literature on both hemorrhagic and ischemic stroke in children with a focus on cerebral arteriopathy and vascular malformations as stroke risk factors. Additionally, we review diagnostic studies for childhood stroke, outcome data, and regional and geographic practice differences. Methods: PubMed was searched using the terms child, childhood, pediatric, stroke, ischemic, intracerebral hemorrhage, vasculopathy, and vascular malformation. Reference lists of these articles were reviewed for additional key publications. Preference was given to articles published since the year 2000; however, seminal articles in the field were also reviewed. Results: Pediatric stroke is a heterogeneous disorder and a major cause of morbidity in the pediatric population. Five-year recurrence risk is estimated to be 5–19%. Children with cerebrovascular abnormalities are at the highest risk of recurrence (66% at 5 years for ischemic stroke in one study). Furthermore, cerebral arteriopathy including arterial dissection may account for up to 80% of childhood stroke in otherwise healthy children. Conclusion: In many cases, evaluation and treatment of pediatric stroke is not evidence-based, and regional and geographic variations in practice patterns exist. Ultimately multicenter cohort studies and dedicated pediatric clinical trials are essential to establish comprehensive evidence-based guidelines for pediatric stroke care. [ABSTRACT FROM AUTHOR]

A 12-year-old boy who presented with headache and papilledema was diagnosed with cerebral sinus venous thrombosis and promyelocytic leukemia. Cerebral sinus venous thrombosis is an uncommon manifestation of acute promyelocytic leukemia, and acute promyelocytic leukemia is an unusual etiology for cerebral sinus venous thrombosis. This case highlights the importance of a complete evaluation for risk factors in a child presenting with cerebral sinus venous thrombosis. Anticoagulation was initiated for treatment of cerebral sinus venous thrombosis, and new asymptomatic hemorrhage was identified in the cerebellum 1 day later. Anticoagulation was discontinued for 5 days and then reinitiated in the intensive care unit without hemorrhage expansion or other complications. We review the literature regarding anticoagulation in children with cerebral sinus venous thrombosis and issues specific to acute promyelocytic leukemia. This case suggests that in a controlled setting, anticoagulation may be safe in selected patients with cerebellar hemorrhage. [ABSTRACT FROM AUTHOR]

We present serial MR findings in a child ultimately diagnosed with the early infantile form of Krabbe disease. MR showed typical features of Krabbe disease including cerebellar and brainstem hyperintensity, periventricular and deep white matter hyperintensity, and cerebral atrophy. In addition, the combination of both enlargement and enhancement of multiple cranial nerves in conjunction with unusual cystic lesions adjacent to the frontal horns of the lateral ventricles was previously unreported and expands the spectrum of imaging findings in early Krabbe disease. [ABSTRACT FROM AUTHOR]

Background: Surgical revascularization decreases the long-term risk of stroke in children with moyamoya arteriopathy but can be associated with an increased risk of stroke during the perioperative period. Evidence-based approaches to optimize perioperative management are limited and practice varies widely. Using a modified Delphi process, we sought to establish expert consensus on key components of the perioperative care of children with moyamoya undergoing indirect revascularization surgery and identify areas of equipoise to define future research priorities. Methods: Thirty neurologists, neurosurgeons, and intensivists practicing in North America with expertise in the management of pediatric moyamoya were invited to participate in a three-round, modified Delphi process consisting of a 138-item practice patterns survey, anonymous electronic evaluation of 88 consensus statements on a 5-point Likert scale, and a virtual group meeting during which statements were discussed, revised, and reassessed. Consensus was defined as ≥ 80% agreement or disagreement. Results: Thirty-nine statements regarding perioperative pediatric moyamoya care for indirect revascularization surgery reached consensus. Salient areas of consensus included the following: (1) children at a high risk for stroke and those with sickle cell disease should be preadmitted prior to indirect revascularization; (2) intravenous isotonic fluids should be administered in all patients for at least 4 h before and 24 h after surgery; (3) aspirin should not be discontinued in the immediate preoperative and postoperative periods; (4) arterial lines for blood pressure monitoring should be continued for at least 24 h after surgery and until active interventions to achieve blood pressure goals are not needed; (5) postoperative care should include hourly vital signs for at least 24 h, hourly neurologic assessments for at least 12 h, adequate pain control, maintaining normoxia and normothermia, and avoiding hypotension; and (6) intravenous fluid bolus administration should be considered the first-line intervention for new focal neurologic deficits following indirect revascularization surgery. Conclusions: In the absence of data supporting specific care practices before and after indirect revascularization surgery in children with moyamoya, this Delphi process defined areas of consensus among neurosurgeons, neurologists, and intensivists with moyamoya expertise. Research priorities identified include determining the role of continuous electroencephalography in postoperative moyamoya care, optimal perioperative blood pressure and hemoglobin targets, and the role of supplemental oxygen for treatment of suspected postoperative ischemia. [ABSTRACT FROM AUTHOR]

Cerebral sinovenous thrombosis (CSVT) is a rare form of pediatric stroke with significant morbidity. We determined cumulative incidence and predictors of acute seizures, remote seizures, and epilepsy after pediatric CSVT. Retrospective analysis of 131 neonates and children with neuroimaging-confirmed CSVT enrolled between 2008 and 2020 from a single-center prospective consecutive cohort. Acute seizures occurred within 7 days of CSVT. Remote seizures occurred >7 days after CSVT. Epilepsy was defined as 2 or more remote seizures at least 24 hours apart. Survival methods determined the incidence of and risk factors for remote seizures and epilepsy. Acute seizures occurred in 14/33 neonates (42%) and 19/98 children (19%). Among children, hemorrhage predicted acute seizures (OR 6.6, 95% CI 1.9 to 22.4, P = 0.003). Remote seizures occurred in six neonates; five developed epilepsy. Remote seizures occurred in 14 children; 10 developed epilepsy. In neonates, 1- and 3-year epilepsy-free survival were 86% (95% CI 62% to 95%) and 66% (95% CI 32% to 87%). One- and 3-year epilepsy-free survival in children were 88% (95% CI 76% to 92%) and 84% (95% CI 59% to 86%). In multivariable analysis for children, acute seizures predicted epilepsy (HR 3.8, 95% CI 1.1-13.3, P = 0.039). In both cohorts, Pediatric Stroke Outcome Measure scores at last follow-up were worse in those with epilepsy compared to those without. Acute seizures occurred in approximately one quarter of our cohort and are an epilepsy risk factor in children with CSVT. Neonates and children with epilepsy had worse outcomes than those without. [ABSTRACT FROM AUTHOR]

Background Epilepsy is a common complication of pediatric stroke. Aim In this study, we aim to measure the association between epilepsy and neurologic outcome after childhood arterial ischemic stroke. Methods Prospective cohort study of children (29 days–19 years) enrolled after an acute arterial ischemic stroke at 21 international pediatric stroke centers and followed to identify epilepsy. One year post-stroke, outcomes were scored using the examination-based Pediatric Stroke Outcome Measure (range = 0–10); higher values reflect greater disability. Ordinal logistic regression was used to measure the association of Pediatric Stroke Outcome Measure scores (categorized as 0–1, 1.5–3, 3.5–6, 6.5–10) with epilepsy. Results Investigators enrolled 86 children (median age = 6.1 years, interquartile range (IQR) = 1.4–12.2 years) with acute stroke. At 1 year, 18/80 (23%) remained on an anticonvulsant including 8/80 (10%) with epilepsy. Among the 70 with Pediatric Stroke Outcome Measure scored, the median was 0.5 (IQR = 0–1.5) for children without epilepsy (n = 63), and 6 (IQR = 0.5–10) for children with epilepsy (n = 7). In univariable analyses, poorer 1-year outcome was associated with middle cerebral artery stroke, cortical infarcts, hemorrhagic transformation, hospital disposition not to home, and epilepsy. In multivariable analysis, middle cerebral artery stroke (odds ratio (OR) = 4.9, 95% confidence intervals (CI) = 1.1–21.3) and epilepsy (OR = 24.1, CI = 1.5–380) remained associated with poorer outcome. Conclusions Children who developed epilepsy during the first year post-stroke had poorer neurologic outcomes than those without epilepsy. [ABSTRACT FROM AUTHOR]

Neuroimaging protocols play an important role in the timely evaluation and treatment of pediatric stroke and its mimics. MRI protocols for stroke in the pediatric population should be guided by the clinical scenario and neurologic examination, with consideration of age, suspected infarct type and underlying risk factors. Acute stroke diagnosis and causes in pediatric age groups can differ significantly from those in adult populations, and delay in stroke diagnosis among children is a common problem. An awareness of pediatric stroke presentations and risk factors among pediatric emergency physicians, neurologists, pediatricians, subspecialists and radiologists is critical to ensuring timely diagnosis. Given special considerations related to unique pediatric stroke risk factors and the need for sedation in some children, expert consensus guidelines for the imaging of suspected pediatric infarct have been proposed. In this article the authors review standard and rapid MRI protocols for the diagnosis of pediatric stroke, as well as the key differences between pediatric and adult stroke imaging. [ABSTRACT FROM AUTHOR]

Neurological morbidity is common after pediatric stroke, with moderate to severe deficits that can significantly impact education and social function. Care and recovery occur in phases distinguished by the time interval after stroke onset. These phases include the hyperacute and acute periods in which the focus is on cerebral reperfusion and prevention of neurological deterioration, followed by the subacute and chronic phases in which the focus is on secondary stroke prevention and mitigation of disability through rehabilitation, adaptation, and reintegration into the community. In this article, a multidisciplinary group of pediatric stroke experts review the stages of recovery after pediatric stroke with an emphasis on critical assessment time points. Our goal is to encourage increased standardization of outcome assessment to facilitate future clinical trials comparing various treatment and intervention options and advance optimized care for children with stroke. [ABSTRACT FROM AUTHOR]

Purpose: To provide consensus-based, suggested imaging protocols to facilitate the accurate and timely diagnosis of a neonate with symptoms concerning for stroke.Methods: The Writing Group, an international collaboration of pediatric neurologists and neuroradiologists with expertise in perinatal and childhood stroke, participated in a series of pediatric stroke neuroimaging symposia. These discussions, in conjunction with extensive literature review, led to a consensus for imaging protocols to guide practitioners in the diagnosis of neonatal stroke subtypes as defined by the National Institute of Neurological Disorders and Stroke Common Data Elements. The epidemiology, clinical presentation, and associated risk factors for arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke are reviewed, with a focused discussion regarding the role of neuroimaging for each subtype.Results: In a neonate with suspected stroke, magnetic resonance imaging is the preferred modality, given the lack of X-irradiation, superior anatomic resolution, and sensitivity for acute ischemia. Core recommended sequences include diffusion-weighted imaging and apparent diffusion coefficient mapping to diagnose acute ischemia, gradient-recalled echo or susceptibility-weighted imaging to detect intracranial blood and its breakdown products, and T1- and T2-weighted imaging to assess for myelination, extra-axial blood, and edema. Magnetic resonance angiography of the brain may be useful to detect vascular abnormalities, with venography if venous sinus thrombosis is suspected. The application of more novel sequences, as well as the utility of follow up-imaging, is also discussed. [ABSTRACT FROM AUTHOR]

Background: Moyamoya syndrome (MMS) is a progressive cerebral arteriopathy with increased incidence in children with neurofibromatosis type 1 (NF1). Despite the potential for significant neurological morbidity including stroke, little is known about the natural history, and no guidelines exist for screening and management of NF1-associated MMS.Methods: We identified 152 literature cases of children aged ≤18 years with NF1-associated MMS. A meta-analysis was performed evaluating clinical and neuroimaging findings and patient outcomes. Data from 19 patients with NF1-associated MMS from our center treated from January 1995 to July 2020 were abstracted via chart review and similarly analyzed for clinical and neuroimaging features.Results: Meta-analysis of literature cases showed a median age of MMS diagnosis of 6 years (interquartile range 3 to 10.8 years). Optic pathway gliomas were more common in patients with MMS (42%) compared with historical prevalence. Stroke or transient ischemic attack (TIA) was present at diagnosis in 46%. TIA and stroke were more common in patients with bilateral versus unilateral MMS (62% vs 34%, P = 0.001) and in children aged <4 years versus those aged ≥4 years (61% vs 40%, P = 0.02). Compared with the literature cases, our cohort was more frequently asymptomatic (42% vs 25%) and less likely to present with TIA or stroke (32% vs 46%) at diagnosis.Conclusions: These data suggest there is an aggressive form of MMS in children with NF1 <4 years of age. Therefore, early screening should be considered to facilitate early detection and treatment of cerebral arteriopathy. [ABSTRACT FROM AUTHOR]

While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis. [ABSTRACT FROM AUTHOR]

We assessed factors associated with mortality and potential targets for intervention in a large national sample of children with nontraumatic intracerebral hemorrhage. Using Healthcare Cost and Utilization Project Kids’ Inpatient Database ICD-9-CM code 431 identified children aged 1 to 18 years with nontraumatic intracerebral hemorrhage in 2003, 2006 and 2009. Intracerebral hemorrhage was the primary diagnosis for 1172 children (ages 1-18 years) over the 3-year sample. Factors associated with mortality based on multivariable logistic regression included Hispanic ethnicity (odds ratio 1.9, 95% confidence interval 1.1-3.3), older age (11-18 vs 1-10 years, odds ratio 2.5, 95% confidence interval 1.3-5.0), coagulopathy (odds ratio 3.0, 95% confidence interval 1.6-6.0), and coma (odds ratio 9.0, 95% confidence interval 3.2-24.6). From 2003 to 2009, there was a non-significant decrease in mortality with a significant increase in length of stay from 9 to 11 days (P < .003). In children with intracerebral hemorrhage, coma and coagulopathy had the strongest association with mortality; coagulopathy is a potentially modifiable risk factor. [ABSTRACT FROM AUTHOR]

Perihaematomal oedema (PHO) is an important pathophysiological marker of secondary injury in intracerebral haemorrhage (ICH). In this Review, we describe a novel method to conceptualize PHO formation within the framework of Starling's principle of movement of fluid across a capillary wall. We consider progression of PHO through three stages, characterized by ionic oedema (stage 1) and progressive vasogenic oedema (stages 2 and 3). In this context, possible modifiers of PHO volume and their value in identifying patients who would benefit from therapies that target secondary injury are discussed; the practicalities of using neuroimaging to measure PHO volume are also considered. We examine whether PHO can be used as a predictor of neurological outcome following ICH, and we provide an overview of emerging therapies. Our discussion emphasizes that PHO has clinical relevance both as a therapeutic target, owing to its augmentation of the mass effect of a haemorrhage, and as a surrogate marker for novel interventions that target secondary injury. [ABSTRACT FROM AUTHOR]

Vein of Galen aneurysmal malformations (VGAM) are rare but clinically significant intracranial arteriovenous shunt lesions that most often present in neonates and infants. Retrospective clinical data were collected for patients evaluated with a diagnosis of VGAM from 1994 to 2007. Thirteen patients with VGAM were evaluated from 1994 to 2007. Seven patients presented emergently with medically intractable cardiac failure, and six were treated in the first 2 weeks of life. Five children treated after this period (1.5–31 months of age) manifested enlarging head circumference, abnormal development, or subarachnoid hemorrhage. Eleven patients were managed endovascularly. Four disease or procedure-related complications occurred. Two complications were associated with poor outcome, both of which occurred in patients treated at less than 2 weeks of age. Two other patients experienced transient neurological deficits with no evidence of permanent sequelae. Outcome in the six patients treated emergently in the first 2 weeks of life included two patients who developed normally, one with mild to moderate neurological deficits, one with severe neurological deficits, and two deaths. Outcome in the five older patients (treated between 1.5 and 31 months) was considerably better than in the group treated early and included three with normal outcome and two with mild neurological deficits. Contemporary endovascular techniques remain the preferred treatment for VGAM in all age groups. Early diagnosis and multimodality treatment are essential for the best management and treatment of the complex constellation of clinical problems often arising from this disorder. [ABSTRACT FROM AUTHOR]

Molecular testing identified a pathogenic variant in the I ATP7A i gene (c.897del [p.Leu299Phefs*7]) confirming the diagnosis of Menkes disease, a rare X-linked neurodegenerative disorder of copper metabolism. Imaging is characterized by cerebral and cerebellar atrophy, white matter abnormalities, particularly in the temporal lobes, subdural hematomas, tortuous vessels, and deep gray nuclei signal abnormalities. [Extracted from the article]

Background: The prevalence of cancer among children with stroke is unknown. This study sought to evaluate cancer- and tumor-associated childhood ischemic stroke in a multinational pediatric stroke registry.Methods: Children aged 29 days to less than 19 years with arterial ischemic stroke or cerebral sinovenous thrombosis enrolled in the International Pediatric Stroke Study between January 2003 and June 2019 were included. Data including stroke treatment and recurrence were compared between subjects with and without cancer using Wilcoxon rank sum and chi-square tests.Results: Cancer or tumor was present in 99 of 2968 children (3.3%) with arterial ischemic stroke and 64 of 596 children (10.7%) with cerebral sinovenous thrombosis. Among children in whom cancer type was identified, 42 of 88 arterial ischemic stroke cases (48%) had brain tumors and 35 (40%) had hematologic malignancies; 45 of 58 cerebral sinovenous thrombosis cases (78%) had hematologic malignancies and eight (14%) had brain tumors. Of 54 cancer-associated arterial ischemic stroke cases with a known cause, 34 (63%) were due to arteriopathy and nine (17%) were due to cardioembolism. Of 46 cancer-associated cerebral sinovenous thrombosis cases with a known cause, 41 (89%) were related to chemotherapy-induced or other prothrombotic states. Children with cancer were less likely than children without cancer to receive antithrombotic therapy for arterial ischemic stroke (58% vs 80%, P = 0.007) and anticoagulation for cerebral sinovenous thrombosis (71% vs 87%, P = 0.046). Recurrent arterial ischemic stroke (5% vs 2%, P = 0.04) and cerebral sinovenous thrombosis (5% vs 1%, P = 0.006) were more common among children with cancer.Conclusions: Cancer is an important risk factor for incident and recurrent childhood stroke. Stroke prevention strategies for children with cancer are needed. [ABSTRACT FROM AUTHOR]

Background: Perinatal hemorrhagic stroke in late preterm and term neonates is understudied. We describe two-month and two-year neurological outcomes in a prospective cohort.Methods: Neonates ≥36 weeks' gestation with spontaneous hemorrhagic stroke (parenchymal and intraventricular) presenting at age ≤28 days were enrolled between March 2007 and May 2015 at three tertiary pediatric centers. Hemorrhagic transformation of arterial ischemic stroke or cerebral sinovenous thrombosis was excluded. The Pediatric Stroke Outcome Measure (PSOM) assessed outcomes. Wilcoxon signed-rank tests evaluated change over time.Results: Twenty-six neonates were included (median age: 1 day, interquartile range [IQR] 0 to 16; median gestational age: 38.3 weeks, IQR 37.0 to 39.0). Hemorrhage was isolated intraventricular in seven (27%), isolated intraparenchymal in six (23%), and a combination in 10 (39%). Three neonates (12%) died during hospitalization; one died later due to cardiac disease. Among 22 survivors, outcomes were assessed at a median of 2.1 months (IQR 1.7 to 3.3) in 96% and 1.9 years (IQR 1.3 to 2.0) in 73%. Median PSOM scores were 0.0 (IQR 0.0 to 1.0) and 0.25 (IQR 0.0 to 1.3), respectively. At two years, 45% of patients had no or nonimpairing deficits (PSOM <1.0), 30% had mild deficits (PSOM 1.0 to 2.0), and 5% had moderate deficits (PSOM 2.5 to 4.5). Over time, 31% worsened and 6% improved. Although total PSOM scores did not change significantly (P = 0.08), language subscores worsened (P = 0.009). No child developed epilepsy.Conclusions: Perinatal hemorrhagic stroke survivors had favorable outcomes in early childhood; at two years moderate to severe deficits occurred in 5%. Language deficits may emerge over time, warranting close follow-up. [ABSTRACT FROM AUTHOR]

Objective: We describe the risk factors for peri-procedural and spontaneous arterial ischemic stroke (AIS) in children with cardiac disease.Methods: We identified children with cardiac causes of AIS enrolled in the International Pediatric Stroke Study registry from January 2003 to July 2014. Isolated patent foramen ovale was excluded. Peri-procedural AIS (those occurring during or within 72 hours of cardiac surgery, cardiac catheterization, or mechanical circulatory support) and spontaneous AIS that occurred outside of these time periods were compared.Results: We identified 672 patients with congenital or acquired cardiac disease as the primary risk factor for AIS. Among these, 177 patients (26%) had peri-procedural AIS and 495 patients (74%) had spontaneous AIS. Among non-neonates, spontaneous AIS occurred at older ages (median 4.2 years, interquartile range 0.97 to 12.4) compared with peri-procedural AIS (median 2.4 years, interquartile range 0.35 to 6.1, P < 0.001). About a third of patients in both groups had a systemic illness at the time of AIS. Patients who had spontaneous AIS were more likely to have a preceding thrombotic event (16 % versus 9 %, P = 0.02) and to have a moderate or severe neurological deficit at discharge (67% versus 33%, P = 0.01) compared to those with peri-procedural AIS.Conclusions: Children with cardiac disease are at risk for AIS at the time of cardiac procedures but also outside of the immediate 72 hours after procedures. Many have acute systemic illness or thrombotic event preceding AIS, suggesting that inflammatory or prothrombotic conditions could act as a stroke trigger in this susceptible population. [ABSTRACT FROM AUTHOR]

Background: Preclinical models of stroke have shown that intravenous glyburide reduces brain swelling and improves survival. We assessed whether intravenous glyburide (RP-1127; glibenclamide) would safely reduce brain swelling, decrease the need for decompressive craniectomy, and improve clinical outcomes in patients presenting with a large hemispheric infarction.Methods: For this double-blind, randomised, placebo-controlled phase 2 trial, we enrolled patients (aged 18-80 years) with a clinical diagnosis of large anterior circulation hemispheric infarction for less than 10 h and baseline diffusion-weighted MRI image lesion volume of 82-300 cm(3) on MRI at 18 hospitals in the USA. We used web-based randomisation (1:1) to allocate patients to the placebo or intravenous glyburide group. Intravenous glyburide was given as a 0·13 mg bolus intravenous injection for the first 2 min, followed by an infusion of 0·16 mg/h for the first 6 h and then 0·11 mg/h for the remaining 66 h. The primary efficacy outcome was the proportion of patients who achieved a modified Rankin Scale (mRS) score of 0-4 at 90 days without undergoing decompressive craniectomy. Analysis was by per protocol. Safety analysis included all randomly assigned patients who received the study drug. This trial is registered with ClinicalTrials.gov, number NCT01794182.Findings: Between May 3, 2013, and April 30, 2015, 86 patients were randomly assigned but enrolment was stopped because of funding reasons. The funder, principal investigators, site investigators, patients, imaging core, and outcomes personnel were masked to treatment. The per-protocol study population was 41 participants who received intravenous glyburide and 36 participants who received placebo. 17 (41%) patients in the intravenous glyburide group and 14 (39%) in the placebo group had an mRS score of 0-4 at 90 days without decompressive craniectomy (adjusted odds ratio 0·87, 95% CI 0·32-2·32; p=0·77). Ten (23%) of 44 participants in the intravenous glyburide group and ten (26%) of 39 participants in the placebo group had cardiac events (p=0·76), and four of 20 had serious adverse events (two in the intravenous glyburide group and two in the placebo group, p=1·00). One cardiac death occurred in each group (p=1·00).Interpretation: Intravenous glyburide was well tolerated in patients with large hemispheric stroke at risk for cerebral oedema. There was no difference in the composite primary outcome. Further study is warranted to assess the potential clinical benefit of a reduction in swelling by intravenous glyburide.Funding: Remedy Pharmaceuticals. [ABSTRACT FROM AUTHOR]

Background: This study describes educational placement of school-aged children after spontaneous intracerebral hemorrhage and examines whether educational placement is associated with severity of neurological deficits.Methods: Children with spontaneous intracerebral hemorrhage presenting from 2007 to 2013 were prospectively enrolled at three tertiary children's hospitals. The Pediatric Stroke Outcome Measure and parental interview gathered information about neurological outcome, school attendance, and educational placement.Results: The cohort of 92 enrolled children included 42 school-aged children (6 to 17 years) with intracerebral hemorrhage. Four children died; one was excluded because of preexisting cognitive deficits. Thirty-seven children completed three-month follow-up, and 30 completed 12-month follow-up. At 12 months, 14 children (46.7%) received regular age-appropriate programming, 12 (40%) attended school with in-class services, three (10%) were in special education programs, and one child (3.3%) received home-based services because of intracerebral hemorrhage-related deficits. Of 30 children with three- and 12-month follow-up, 14 (46.7%) improved their education status, 13 (43.3%) remained at the same education level, and three (10%) began to receive in-class services. An increasing Pediatric Stroke Outcome Measure score predicted the need for educational modifications at three months (odds ratio, 3.3; 95% confidence interval, 1.4 to 7.9; P = 0.007) and at 12 months (odds ratio, 2.1; 95% confidence interval, 1.1 to 3.9; P = 0.025).Conclusions: Most children returned to school within a year after intracerebral hemorrhage, and many had a reduction in the intensity of educational support. However, a great need for educational services persisted at 12 months after intracerebral hemorrhage with fewer than half enrolled in regular age-appropriate classes. Worse deficits on the Pediatric Stroke Outcome Measure were associated with remedial educational placement. [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) spine showed a small annular tear of the C4-C5 disc (Fig. Diagnosis is made radiographically by spine diffusion-weighted and T2-weighted MRI, which demonstrate signal change within the anterior spinal artery territory and can show evidence of disc injury. Pediatr Neurol. 2019, 99, 3 - 6 4 Beslow L. A., Ichord R. N., Zimmerman R. A., Smith S. E., Licht D. J. Role of diffusion MRI in diagnosis of spinal cord infarction in children. [Extracted from the article]

Background Perinatal and childhood stroke result in neurological impairment in the majority of survivors, but less is known about patient and parent perception of function following stroke in children. Our aim was to characterize parent-proxy and child-reported health status in children following unilateral arterial ischemic stroke or intraparenchymal hemorrhage. Methods Fifty-nine children 2-18 years (30 girls, 29 boys) with unilateral arterial ischemic stroke or spontaneous intraparenchymal hemorrhage at least 6 months before evaluation were enrolled from a single center. The PedsQL version 4.0 Generic Short Form and PedsQL version 3.0 Cerebral Palsy Module were administered to childhood stroke subjects and parents. Generic PedsQL Inventory scores were compared between children with stroke and published data from healthy children. Reported health status scores for children with varying degrees of hemiparesis were compared. Results Children with stroke had lower reported health status scores on the Generic PedsQL Inventory than healthy children. Children with moderate-severe hemiparesis had worse scores than children without hemiparesis on several measures of the Cerebral Palsy Module as reported by both parents and children. The parents of children with epilepsy reported worse scores on several measures compared with children without epilepsy, and the parent scores were lower on several measures for children with lower intelligence quotients. Agreement between parent and child scores was better on the Cerebral Palsy Module than on the Generic Inventory. Conclusions Children with stroke have worse reported health status than healthy controls. Degree of hemiparesis, epilepsy, and lower intelligence quotient affect reported health status on some measures. Agreement between parent-proxy and child scores ranges from slight to good which suggests that both provide useful information. [ABSTRACT FROM AUTHOR]

Background Cardiac disease is a leading cause of stroke in children, yet limited data support the current stroke prevention and treatment recommendations. A multidisciplinary panel of clinicians was convened in February 2014 by the International Pediatric Stroke Study group to identify knowledge gaps and prioritize clinical research efforts for children with cardiac disease and stroke. Results Significant knowledge gaps exist, including a lack of data on stroke incidence, predictors, primary and secondary stroke prevention, hyperacute treatment, and outcome in children with cardiac disease. Commonly used diagnostic techniques including brain computed tomography and ultrasound have low rates of stroke detection, and diagnosis is frequently delayed. The challenges of research studies in this population include epidemiologic barriers to research such as small patient numbers, heterogeneity of cardiac disease, and coexistence of multiple risk factors. Based on stroke burden and study feasibility, studies involving mechanical circulatory support, single ventricle patients, early stroke detection strategies, and understanding secondary stroke risk factors and prevention are the highest research priorities over the next 5-10 years. The development of large-scale multicenter and multispecialty collaborative research is a critical next step. The designation of centers of expertise will assist in clinical care and research. Conclusions There is an urgent need for additional research to improve the quality of evidence in guideline recommendations for cardiogenic stroke in children. Although significant barriers to clinical research exist, multicenter and multispecialty collaboration is an important step toward advancing clinical care and research for children with cardiac disease and stroke. [ABSTRACT FROM AUTHOR]

Cerebral sinovenous thrombosis (CSVT) is a postulated cause of subdural hemorrhage (SDH) that is hypothesized to mimic abusive head trauma (AHT). Minimal data exists directly investigating this relationship. To evaluate the frequency of SDH in children with CSVT, identify factors associated with CSVT and SDH, and to assess if any association supports the hypothesis that CSVT causes SDH. The International Pediatric Stroke Study (IPSS) prospectively collects data on subjects birth to 19 years of age with intracranial thrombosis. IPSS subjects with CSVT and SDH were compared to those with CSVT and no SDH. For subjects with CSVT and SDH, neuroimaging reports further characterized the findings. In any case with no known risk factors for SDH, neuroimaging studies were reviewed by a pediatric neuroradiologist. Of 216 subjects with CSVT, 20 (9.3%) had SDH. Those with SDH (median age 0.3 years) were younger than those without SDH (median age 4.2 years), p < 0.001. Subjects with CSVT and SDH more frequently had anoxia (OR = 10.8; 95% CI: 1.4, 81.1), head/neck injury (OR = 4.0; 95% CI: 1.3, 12.6), or abnormal consciousness (OR = 3.0; 95% CI: 1.2, 7.6). Of 20 subjects with CSVT and SDH, 19 had known risk factors for SDH. The remaining subject had a chronic SDH identified concomitantly to a newly symptomatic CSVT with accompanying venous infarctions. SDH in the setting of CSVT is typically identified in children with independent risk factors for SDH. This study does not support the hypothesis that CSVT causes SDH. • Cerebral sinovenous thrombosis has been hypothesized to cause subdural hemorrhage. • This hypothesis is frequently proposed in legal settings involving abusive head trauma. • Little data exists evaluating this hypothesis. • This study evaluates this hypothesis using a large international database. • Results of this study does not support the hypothesis that CSVT causes SDH. [ABSTRACT FROM AUTHOR]

The article presents a case study of 13-year-old boy who was admitted to the child psychiatric unit in setting of an emotional outburst and suicidal ideation. Topics discussed include his examination revealing moderate dysarthria, writhing tongue movements and Kayser-Fleischer rings; laboratory studies demonstrated leukopenia; and the patient diagnosed with Wilson disease.