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Background Vascular access in neonates is limited by the small size of the femoral artery and the risk of complications, including thrombosis and limb ischemia. Although umbilical artery access has been described, previous reports detail exchange of the umbilical artery catheter (UAC) for a conventional arterial sheath, which can be difficult and cumbersome. We aim to describe direct use of the UAC as the sheath and guide catheter in neonatal patients requiring endovascular treatment for life- threatening intracranial arteriovenous shunts. Methods We reviewed all cases between 2016 and 2021 of neonatal intervention using a direct UAC approach. We describe our technique of utilizing UACs for cerebral angiography and neurointerventions in newborns without catheter exchange, allowing for repeated, straightforward endovascular access in neonates across multiple interventions. Results Seven consecutive neonates underwent endovascular neurointerventional procedures for the treatment of life- threatening, high- flow arteriovenous shunts using a direct UAC access technique without procedural morbidity. Five of seven patients underwent more than one procedure in the neonatal period, between day 1 and day 10 of life. Conclusions Use of the UAC itself as a diagnostic catheter for cerebral angiography or as a guide catheter for neurointerventions greatly facilitates endovascular interventions in newborns and is ideal for patients requiring multiple interventions in the neonatal period. This technique helps to mitigate the risk of neonatal femoral artery access and its complications, including thrombosis, dissection, spasm, stenosis, and limb ischemia. [ABSTRACT FROM AUTHOR]

Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular disease comprised of a diffuse network of blood vessels, often spread over several lobes. Due to the presence of normal brain tissue within the malformation, complete resection is not desirable. Several imaging characteristics help to distinguish CPA from brain arteriovenous malformations. We report the case of a patient diagnosed with CPA five years after a spontaneous intraventricular hemorrhage and an initial negative angiogram. Teaching Point: The radiologist should be able to differentiate CPA from brain AVM to prevent potentially harmful treatment. [ABSTRACT FROM AUTHOR]

Neonatal brain hypoxia is a disease that affects the nervous system in children. Salidroside is a compound that has an anti-hypoxic effect, but the mechanism of salidroside in neonatal cerebral hypoxia is unclear. Hence, we investigated the regulatory effect and mechanism of salidroside on hypoxic-induced injury of neural stem cells (NSCs). NSCs derived from embryo 14 Sprague-Dawley rats were treated by hypoxia, followed by the treatment of 0.8 mM salidroside. The expression levels of miR-210 and BTG3 in NSCs were altered by transfection. Cell viability and apoptosis were examined by CCK-8 and flow cytometry analysis. qRT-PCR and Western blot were performed to assess the expression changes of miR-210, BTG3, apoptosis-related factors and core factors in PI3K/AKT/mTOR pathway. We found that hypoxia induced an apoptosis-dependent death in NSCs. Salidroside exerted bFGF-like effect, as it alleviated hypoxia-induced viability impairment and apoptosis in NSCs. Further studies showed that hypoxia plus salidroside elevated miR-210 expression, and the protective actions of salidroside on hypoxia-modulated death in NSCs were attenuated by miR-210 suppression, while were enhanced by miR-210 overexpression. Besides, BTG3 was negatively regulated by miR-210. Overexpression of BTG3 inhibited the activation of PI3K/AKT/mTOR signaling pathway; of contrast, suppression of BTG3 promoted it. To conclude, this study provide in vitro evidence that salidroside protected NSCs against hypoxia-induced injury by up-regulation of miR-210, which in turn inhibited the expression of BTG3 and activated PI3K/AKT/mTOR signaling pathway. [ABSTRACT FROM AUTHOR]

Hypoxic-ischemic encephalopathy (HIE) in newborn infants is generally considered to result from decreased arterial oxygen content or cerebral blood flow. Cerebral injury similar to that of HIE has been noted with hypoglycemia. Studies in fetal lambs have shown that ventilation with 3% oxygen did not change cerebral blood flow, but ventilation with 100% oxygen resulted in marked reduction in cerebral blood flow, glucose delivery and glucose consumption. Blood glucose concentration falls markedly after birth; this, associated with the fall in cerebral blood flow, greatly reduces glucose supply to the brain. In preterm infants, blood glucose levels tend to be very low. Also persistent patency of the ductus arteriosus may reduce cerebral flow in diastole, thus exaggerating the decrease in glucose supply. I propose that glycopenic-ischemic encephalopathy is a more appropriate term for the cerebral insult. We should consider more aggressive management of the low blood glucose concentrations in the neonate, and particularly in preterm infants. Administration of high levels of oxygen in inspired air should be avoided to reduce the enhancement of cerebral vasoconstriction and decreased flow that normally occurs after birth. [ABSTRACT FROM AUTHOR]

Background: Bevacizumab (BVZ) is a vascular endothelial growth factor inhibitor that has been widely accepted since its introduction into the cancer pharmacopoeia. Anecdotal reports suggested improvements in vision in children with visual pathway glioma.Case presentation: We report a boy with visual pathway glioma whose vision had deteriorated significantly on vincristine and carboplatin, to the point that he was registered blind. Following bevacizumab therapy, there was a dramatic improvement in vision with reduction in tumour volume. However, following 20 doses of BVZ given over 19 months, he developed a significant cerebrovascular stenosis.Conclusion: The BVZ-induced cerebrovascular diseases in children are extremely rare but potentially serious. Importantly, stenosis has not been previously described in literature. [ABSTRACT FROM AUTHOR]

Background: Acute stroke protocols improve delivery of care but it is unclear whether these resource intensive protocols are able to differentiate stroke from mimics in children. The aim of this study is to describe our institution's experience with stroke mimics identified through our pediatric stroke clinical pathway (PSCP).Methods: The PSCP was implemented in our level 1 pediatric emergency department in June 2014 for children aged one month to 18 years. For patients managed using the PSCP from June 2014 to December 2016, demographic and clinical data were compared for patients diagnosed with stroke or a stroke mimic.Results: A total of 59 children were evaluated with the PSCP. Fourteen children were identified as having a stroke and 45 children had stroke mimics. The most common stroke mimics were functional neurological disorders (20.0%), transient neurological deficits (17.8%), migraine (15.6%), and seizure (11.1%). Patient demographics and time to neuroimaging did not differ between patients with and without stroke. Vomiting was commonly reported by patients with stroke (odds ratio: 4.00, 95% confidence interval: 1.12 to 14.35), whereas weakness was not (odds ratio: 0.7, 95% confidence interval: 0.07 to 0.90), but the physical examination did not differ between patients with and without stroke.Conclusions: The PSCP ensures timely evaluation of patients presenting with neurological deficits but fails to reliably differentiate between patients with stroke and patients with stroke mimics. Further multicentered studies are needed to develop a "stroke screen" that reliably distinguishes pediatric stroke from its mimics. [ABSTRACT FROM AUTHOR]

Background. The risk for cerebrovascular late effects among childhood cancer survivors is considerable. According to recent studies it is not clear which marker could be reliable for the screening of cerebrovascular diseases among the long-term survivors of childhood cancer. The purpose of this study is to analyse arterial stiffness and intima-media thickness as possible early markers of later occurring stroke in long-term survivors of childhood cancer after neck radiotherapy. Patients and methods. Twenty-three patients, treated for Hodgkin disease (HD) in childhood, were included. They had received radiation therapy to the neck with 20-65 (median 30) Gy. Twenty-six healthy controls, matched in age, sex, body mass index, arterial hypertension, smoking history and total cholesterol levels were compared. Highresolution colour-coded duplex sonography and power Doppler sonography of the carotid arteries were performed and intima-media thickness, number and quality of plaques were measured. Arterial stiffness indices were calculated. Results. Plaque deposits and/or arterial wall calcinations were found in 24 out of 43 (55.8%) irradiated vessels in cancer survivors group and 0 out of 52 vessels in the group of healthy controls (p < 0.01). We found significant group differences for all the stiffness parameters we used (P < 0.05), but there was no difference in intima-media thickness between cases and controls (p = 0.92). In a multivariate model, carotid pulse wave velocity was positively associated with smoking. Conclusions. The arterial stiffness has appeared as a possible surrogate marker for stroke in long-term survivors of childhood cancer. Smoking habit might have an additional negative influence on vascular aging in the group of patients after neck radiotherapy. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM PUBLISHER]

Purpose: Cavernous malformations (CMs) are rare developmental cerebrovascular malformations of the central nervous system with a childhood prevalence of 0.3 to 0.53%. Our purpose was to assess the clinical features and microsurgical outcome in pediatric central nervous system (CNS) CMs. Material and methods: We retrospectively enrolled all the CM patients admitted to our institute from 1 January 2001 to 31 December 2014. Data was analyzed for their clinical features and surgical outcome. Results: A total of 50 patients with CMs (30 supratentorial, 14 infratentorial, and 6 spinal) with a mean age of 14 years (3-18 years, SD ±4.64) were enrolled into the study. Most of these patients (78%) were male. Size varied from 1.2 to 6 cm. Three patients had multiple CMs. Symptoms of CMs were site specific. Seizure was the most common symptom (63.3%) of CMs at supratentorial location followed by headache (46%) and neurodeficiency (26%), while all brainstem and spinal CMs presented with neurodeficiencies. History of clinically significant acute hemorrhage was present in 19.2% of supratentorial (ST) superficial CMs, 50% of ST deep CMs, 25% of cerebellar CMs, 44.4% of brainstem CMs, and 50% of spinal CMs. Forty-five CMs in 44 patients were surgically excised. Their follow-up ranged from 6 to 162 months (mean 47.2 months, SD ±53). All supratentorial CM patients showed improvement in their symptoms. Patients with preoperative seizure showed good seizure control with Engel scale I in 16 (94.1%) and Engel scale II in 1 (5.9%). In infratentorial (IT) and spinal CM patients, 92.3 and 66.7% had improvement in their neurodeficiencies, respectively. There was no mortality in our series. Conclusion: Microsurgical excision of CNS CM results in excellent neurological outcome in pediatric patients. Early intervention is necessary in spinal CMs for better outcome. [ABSTRACT FROM AUTHOR]

Sickle cell disease (SCD) is a common cause of cerebrovascular disease in childhood. Primary stroke prevention is effective using transcranial Doppler (TCD) scans to measure intracranial blood velocities, and regular blood transfusions or hydroxycarbamide when these are abnormal. Inadequate TCD scans occur when it is not possible to measure velocities in all the main arteries. We have investigated the prevalence and significance of this in a retrospective audit of 3915 TCD scans in 1191 children, performed between 2008 and 2015. 79% scans were normal, 6.4% conditional, 2.8% abnormal and 12% inadequate. 21.6% of 1191 patients had an inadequate scan at least once. The median age of first inadequate scan was 3.3 years (0.7–19.4), with a U-shaped frequency distribution with age: 28% aged 2–3 years, 3.5% age 10 years, 25% age 16 years. In young children reduced compliance was the main reason for inadequate TCDs, whereas in older children it was due to a poor temporal ultrasound window. The prevalence of inadequate TCD was 8% in the main Vascular Laboratory at King’s College Hospital and significantly higher at 16% in the outreach clinics (P<0.0001), probably due to the use of a portable ultrasound machine. Inadequate TCD scans were not associated with underlying cerebrovascular disease. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

On June 5, 2015 the International Pediatric Stroke Study and the Stroke Imaging Laboratory for Children cohosted a unique workshop focused on developing neuroimaging research in pediatric stroke. Pediatric neurologists, neuroradiologists, interventional neuroradiologists, physicists, nurse practitioners, neuropsychologists, and imaging research scientists from around the world attended this one-day meeting. Our objectives were to (1) establish a group of experts to collaborate in advancing pediatric neuroimaging for stroke, (2) develop consensus clinical and research magnetic resonance imaging protocols for pediatric stroke patients, and (3) develop imaging-based research strategies in pediatric ischemic stroke. This article provides a summary of the meeting proceedings focusing on identified challenges and solutions and outcomes from the meeting. Further details on the workshop contents and outcomes are provided in three additional articles in the current issue of Pediatric Neurology. [ABSTRACT FROM AUTHOR]

Background: Pediatric arterial ischemic stroke remains incompletely understood. Population-based epidemiological data inform clinical trial design but are scant in this condition. We aimed to determine age-specific epidemiological characteristics of arterial ischemic stroke in neonates (birth to 28 days) and older children (29 days to 18 years).Methods: We conducted a 16-year, prospective, national population-based study, the Canadian Pediatric Ischemic Stroke Registry, across all 16 Canadian acute care children's hospitals. We prospectively enrolled children with arterial ischemic stroke from January 1992 to December 2001 and documented disease incidence, presentations, risk factors, and treatments. Study outcomes were assessed throughout 2008, including abnormal clinical outcomes (stroke-related death or neurological deficit) and recurrent arterial ischemic stroke or transient ischemic attack.Results: Among 1129 children enrolled with arterial ischemic stroke, stroke incidence was 1.72/100,000/year, (neonates 10.2/100,000 live births). Detailed clinical and radiological information were available for 933 children (232 neonates and 701 older children, 55% male). The predominant clinical presentations were seizures in neonates (88%), focal deficits in older children (77%), and diffuse neurological signs (54%) in both. Among neonates, 44% had no discernible risk factors. In older children, arteriopathy (49% of patients with vascular imaging), cardiac disorders (28%), and prothrombotic disorders (35% of patients tested) predominated. Antithrombotic treatment increased during the study period (P < 0.001). Stroke-specific mortality was 5%. Outcomes included neurological deficits in 60% of neonates and 70% of older children. Among neonates, deficits emerged during follow-up in 39%. Overall, an initially decreased level of consciousness, a nonspecific systemic presentation, and the presence of stroke risk factors predicted abnormal outcomes. For neonates, predictors were decreased level of consciousness, nonspecific systemic presentation, and basal ganglia infarcts. For older children, predictors were initial seizures, nonspecific systemic presentation, risk factors, and lack of antithrombotic treatment. Recurrent arterial ischemic stroke or transient ischemic attack developed in 12% of older children and was predicted by arteriopathy, presentation without seizures, and lack of antithrombotic treatment. Emerging deficit was predicted by neonatal age at stroke and by cardiac disease.Conclusions: This national data set provides a population-based disease incidence rate and demonstrates the protective effect of antithrombotic treatment in older children, and frequent long-term emerging deficits in neonates and in children with cardiac disorders. Further clinical trials are required to develop effective age-appropriate treatments for children with acute arterial ischemic stroke. [ABSTRACT FROM AUTHOR]

Background: Children with perinatal arterial ischemic stroke (PAIS) are at risk for later neurocognitive and behavioral deficits, yet the clinical predictors of these outcomes are understudied. We examined the influence of clinical and infarct characteristics on attention and executive functioning in children following PAIS.Methods: Forty children born at term (≥37 weeks' gestation) with PAIS (28 with neonatal arterial ischemic stroke and 12 with presumed PAIS) underwent a comprehensive neuropsychological battery at age three to 16 years (median age 7.2 years; 58% male) to assess attention and executive functioning. Parents also completed questionnaires regarding real-world functioning. Clinical variables including perinatal stroke subtype, infarct characteristics (location, laterality, and volume), and the presence of comorbid epilepsy were ascertained from the medical record.Results: Presumed PAIS, larger infarct volume, and comorbid epilepsy negatively influenced the performance on attention and executive functioning measures. These clinical variables were also associated with greater functional problems on parent reports, including a higher frequency of attention-deficit/hyperactivity disorder symptoms and greater difficulties in some subdomains of executive functioning. Infarct location and laterality were not associated with performance measures or parental report of functioning.Conclusion: Although all children with PAIS are at risk for later deficits in attention and executive functioning, those with presumed PAIS, larger infarct size, and comorbid epilepsy appear to be the most vulnerable. As they approach and reach school age, these children should undergo neuropsychological assessment to ensure timely implementation of therapeutic interventions and behavioral strategies. [ABSTRACT FROM AUTHOR]

Background: Previous data have shown that the 20210G>A polymorphism of the Factor II gene is related to an increased prothrombin level, which may in turn lead to a procoagulant state. The heterogeneous and multifactorial character of arterial ischemic stroke often results in contradictory reports describing the association between the 20210G>A polymorphism and arterial ischemic stroke in different populations. We performed a meta-analysis of available data addressing the relation between the FII 20210G>A polymorphism and arterial ischemic stroke, both in young adults and children.Methods: We searched PubMed using appropriate keywords. The inclusion criteria for the study were as follows: case-control study, study population consisting of children, study population consisting of young adults, arterial ischemic stroke confirmed by magnetic resonance imaging or computed tomography, and English language. The exclusion criteria included lack of genotype or allele frequencies, study design other than a case-control study, outcome definition other than arterial ischemic stroke, and previously overlapped patient groups. Finally, 30 case-control studies (14 in children and 16 in young adults) were included. Statistical analyses were conducted using R software. Heterogeneity between the studies was evaluated using the Dersimonian and Laird's Q test. In the case of significant between-studies heterogeneity, the pooled odds ratio was estimated with a random-effects model, otherwise a fixed-effects model was used.Results: The pooled analysis showed that carriers of 20210A allele (GA+AA genotypes) of the prothrombin gene are more common in arterial ischemic stroke patients, both in children and young adults, than in control subjects (P = 0.006; odds ratio, 1.83; 95% confidence interval, 1.19 to 2.80 and P = 0.001; odds ratio, 1.69; 95% confidence interval, 1.25 to 2.28, respectively).Conclusions: The results of the present meta-analysis have proven that the FII 20210G>A polymorphism is associated with arterial ischemic stroke in both pediatric and young adult patients. [ABSTRACT FROM AUTHOR]

BACKGROUND: Moya-moya disease in children is a cerebrovascular disorder that may cause cerebral ischemic or hemorrhage. CASE DETAILS: We report an 8-year-old boy that was admitted with the chief complaint of repeated sudden half left body paralyze. MRA showed acute thrombotic infarction in the right hemisphere and internal carotid artery stenosis in the form of puffs of smokes. Indirect revascularization surgical procedure with combination of Encephalo-myo-pial-synangiosis (EMS) and Encephalo-arterio-pial-synangiosis (EAS) was performed. It resulted in a good response. CONCLUSION: Moya-moya disease can lead to permanent neurological disability if untreated. Satisfactory outcome was noted following combination surgery management with EMS and EAS. [ABSTRACT FROM AUTHOR]

The biology of post-concussive symptoms is unclear. Symptoms are often increased during activities, and have been linked to decreased cerebrovascular reactivity and perfusion. The aim of this study was to examine cerebral blood flow (CBF) in children with different clinical recovery patterns following mild traumatic brain injury (mTBI). This was a prospective controlled cohort study of children with mTBI (ages 8 to 18 years) who were symptomatic with post-concussive symptoms at one month post-injury (symptomatic, n = 27) and children who had recovered quickly (asymptomatic, n = 24). Pseudo continuous arterial spin labeling magnetic resonance imaging (MRI) was used to quantify CBF. The mTBI groups were imaged at 40 days post-injury. Global and regional CBF were compared with healthy controls of similar age and sex but without a history of mTBI ( n = 21). Seventy-two participants (mean age: 14.1 years) underwent neuroimaging. Significant differences in CBF were found: global CBF was higher in the symptomatic group and lower in the asymptomatic group compared with controls, (F(2,69) 9.734; p < 0.001). Post-injury symptom score could be predicted by pre-injury symptoms and CBF in presence of mTBI (adjusted R2 = 0.424; p < 0.001). Altered patterns of cerebral perfusion are seen following mTBI and are associated with the recovery trajectory. Symptomatic children have higher CBF. Children who 'recovered' quickly, have decreased CBF suggesting that clinical recovery precedes the cerebral recovery. Further longitudinal studies are required to determine if these perfusion patterns continue to change over time. [ABSTRACT FROM AUTHOR]

Cerebrovascular disease is an important feature of pediatric sickle cell disease (SCD) and may lead to cognitive and motor impairment. Our cross-sectional study examined the incidence and severity of these impairments in a pediatric cohort without clinical cerebrovascular events from Berlin of mixed ethnic origin. Thirty-two SCD patients (mean age 11.14 years, range 7.0-17.25 years; males 14) were evaluated for full-scale intelligence (IQ) (German version WISC-III), fine motor function (digital writing tablet), and executive function (planning, attention, working memory, and visual-spatial abilities) with the Amsterdam Neuropsychological Tasks (ANT) program and the Tower of London (ToL). Data on clinical risk factors were retrieved from medical records. Full-scale IQ of patients was preserved, whereas performance IQ was significantly reduced (91.19 (SD 12.17) d = 0.7, p = 0.007). SCD patients scored significantly lower than healthy peers when tested for executive and fine motor functions, e.g., planning time in the ToL (6.73 s (SD 3.21) vs. 5.9 s in healthy peers (SD 2.33), d = 0.5, p = <0.001) and frequency on the writing tablet (mean z score -0.79, d = 0.7, p < 0.001). No clinical risk factors were significantly associated with incidence and severity of cognitive and motor deficits. Despite the preservation of full-scale IQ, our SCD cohort of mixed origin exhibited inferior executive abilities and reduced fine motor skills. Our study is limited by the small size of our cohort as well as the lack for control of sociodemographic and socioeconomic factors modulating higher functions but highlights the need for early screening, prevention, and specific interventions for these deficits. [ABSTRACT FROM AUTHOR]

Overt ischaemic stroke is one of the most devastating complications in children with sickle cell disease ( SCD). The compensatory response to anaemia in SCD includes an increase in cerebral blood flow ( CBF) by accessing cerebrovascular dilatory reserve. Exhaustion of dilatory reserve secondary to anaemic stress may lead to cerebral ischaemia. The purpose of this study was to investigate CBF and cerebrovascular reactivity ( CVR) using magnetic resonance imaging ( MRI) in children with SCD and to correlate these with haematological markers of anaemia. Baseline CBF was measured using arterial spin labelling. Blood-oxygen level-dependent MRI in response to a CO2 stimulus was used to acquire CVR. In total, 28 children with SCD (23 not on any disease-modifying treatment, 5 on chronic transfusion) and 22 healthy controls were imaged using MRI. Transfusion patients were imaged at two time points to assess the effect of changes in haematocrit after a transfusion cycle. In children with SCD, CBF was significantly elevated compared to healthy controls, while CVR was significantly reduced. Both measures were significantly correlated with haematocrit. For transfusion patients, CBF decreased and CVR increased following a transfusion cycle. Lastly, a significant correlation was observed between CBF and CVR in both children with SCD and healthy controls. [ABSTRACT FROM AUTHOR]

Aim: Paediatric arterial ischaemic stroke can lead to reduced quality of life (QoL). It is important to identify predictors of QoL to support recovery. We examined long-term QoL after arterial ischaemic stroke concerning different variables.Method: Children registered in the Swiss Neuropediatric Stroke Registry and suffering from arterial ischaemic stroke between 2000 and 2008 were included. Two years post-stroke, assessments included intelligence quotient tests for cognitive impairment and modified Rankin Scale (mRS) for neurological impairment; 5 years post-stroke, the Kidscreen-27 was used for QoL, DSM-IV criteria screening was used for attention deficits, and the ABILHAND-Kids was used for manual motor skills. Age at stroke, sex, socioeconomic status, lesion characteristics, neuropsychological and motor outcome, and mRS were correlated with QoL measures.Results: Seventy children were examined (49 males, 21 females; mean age 7y 2wks). Age at stroke, sex, socioeconomic status, and lesion characteristics did not influence QoL; IQ below average and attention deficits partially influenced QoL. The highest predictive value for QoL was found for manual motor impairment (p=0.002) and mRS scores (p=0.013). Combined motor, cognitive, and attention impairment negatively affected QoL (p=0.001).Interpretation: Neurological and cognitive impairments after paediatric arterial ischaemic stroke negatively influence QoL. Children with motor and neurological problems, as well as those with combined motor, cognitive, and attention problems, are at higher risk for low QoL. [ABSTRACT FROM AUTHOR]

Introduction: PHACE syndrome is a neurocutaneous disorder involving large facial hemangiomas in association with posterior fossa abnormalities, cerebral arterial anomalies, cardiac defects, and eye abnormalities. A recent consensus statement has delineated criteria necessary for the diagnosis of PHACE syndrome. Extracutaneous manifestations of PHACE syndrome predominately affect the cerebrovascular system. To date, there are no reports of cerebral cavernous malformations (CCMs) in children with PHACE syndrome. Methods: We reviewed the charts of children admitted to the Children''s Hospital of Pittsburgh who met criteria for PHACE syndrome, and evaluated neuroimaging for cerebrovascular abnormalities, including the finding of CCMs. Results: Six children met criteria for PHACE syndrome at our institution over a 10-year period. All children were female. All children had cerebrovascular abnormalities sufficient to meet major criteria for diagnosis. Four children (66.7 %) were found incidentally to have CCMs; all lesions measured less than 5 mm at the time of diagnosis and were asymptomatic. Conclusion: At present, CCMs are not listed among the diagnostic criteria for PHACE syndrome, and they have not previously been reported in association with PHACE syndrome. Hypoxic injury in utero may be the common denominator in the pathogenesis of many of the abnormalities already accepted in the criteria for PHACE syndrome and the formation of CCMs. In the setting of PHACE syndrome, we encourage clinicians to evaluate children for CCMs, which are readily apparent on the already-recommended screening MRIs. [ABSTRACT FROM AUTHOR]

There are limited data on outcomes of mechanical thrombectomy for pediatric stroke using modern devices. In this study, we report two cases of pediatric acute ischemic stroke treated with mechanical thrombectomy, both with good angiographic result (TICI 3) and clinical outcome (no neurological deficits at 90 days). In addition, we conducted a literature review of all previously reported cases describing the use of modern thrombectomy devices. Including our two cases, the aggregate rate of partial or complete vessel recanalization was 100% (22/22), and the aggregate rate of favorable clinical outcome was 91% (20/22). This preliminary evidence suggests that mechanical thrombectomy with modern devices may be a safe and effective treatment option in pediatric patients with acute ischemic stroke. [ABSTRACT FROM AUTHOR]

Acute ischemic stroke (AIS) in children has been difficult to diagnose, treat and study, due to atypical clinical presentation, imaging challenges and the rarity of large-vessel embolic occlusion as the etiology of acute neurological change in children. With endovascular thrombectomy showing success in randomized trials of adult AIS, the technique is increasingly being extrapolated to pediatric stroke. However, there is little evidence regarding the safety or efficacy of applying in children these devices developed and tested in adults. There is concern about a vessel-to-device size mismatch that may result in a different complication and benefit profile than typically seen. We report on the successful application in pediatric stroke of a newer-generation, smaller stent retriever, designed to be delivered through relatively smaller and more navigable microcatheters than the prior generation of this device. [ABSTRACT FROM AUTHOR]

Background Pediatric cerebral sparganosis has been seldom reported. In the current study, we retrospectively reviewed the clinicopathologic records of 9 consecutive pediatric cases of cerebral sparganosis and analyzed their epidemiologic characteristics and clinical outcomes. Methods Our cases included 6 boys and 3 girls, all from rural areas, and their median age at diagnosis was 9.4 (range, 5.8–12.9) years. The median duration of symptoms from onset to definite diagnosis was 21 months (range, 1 week to 3.7 years). Results Enzyme-linked immunosorbent assay revealed that serum anti-sparganosis antibody was positive in 9 of 9 patients and cerebrospinal fluid anti-sparganosis antibody was positive in 4 of 6 patients. Eight patients underwent craniotomy the removal of worms. The patients also received oral praziquantel. They were followed up for 2.2 years to 4.4 years. One patient died, and 8 patients survived. Three cases had poor outcomes whereas the outcome of the remaining 5 cases was satisfactory. Conclusions Children are more at risk for sparganosis and cerebral sparganosis may be missed because of unclear epidemiologic history and nonspecific manifestations. Cerebrospinal fluid eosinophil counts and enzyme-linked immunosorbent assay for anti-sparganosis antibody and computed tomography/magnetic resonance imaging scans may be relied on for an early and accurate diagnosis before surgery. [ABSTRACT FROM AUTHOR]

An estimated 3.2 million children worldwide have human immunodeficiency virus (HIV) infection. Antiretroviral therapy (ART) has resulted in prolonged survival, leading to an increase in complications previously recognized in adults. Children with HIV infection have increased risk of cerebrovascular disease from multiple aetiologies including HIV-associated vasculopathy, opportunistic vasculitis, cardioembolism or coagulopathy, all of which may be secondary to the infection. Prevalence of cerebrovascular disease in HIV-infected children is underestimated because of limited neuroimaging in low and middle income countries, silent events without overt motor manifestations, and mislabeling as HIV encephalopathy for non-motor manifestations such as behavioural and cognitive difficulties. No management guidelines for cerebrovascular disease in HIV-infected children exist but common practices target risk factors for stroke in low and middle income countries. Where capacity permits, screening for opportunistic infections, vasculitis, coagulopathy and cardioembolism is important. Optimising virological suppression, correction of anaemia, control of seizures and aspirin prophylaxis are management priorities. Neurosurgical interventions may have a role. [ABSTRACT FROM AUTHOR]

Objective Children with syndromic craniosynostosis are at risk of intracranial hypertension. This study aims to examine patient profiles of transcranial Doppler (TCD) cerebral blood flow velocity (CBFv) and systemic blood pressure (BP) in subjects with and without papilledema at the time of surgery, and subsequent effect of cranial vault expansion. Methods Prospective study of patients treated at a national referral center. Patients underwent TCD of the middle cerebral artery 1 day before and 3 weeks after surgery. Measurements included mean CBFv, peak systolic velocity, and end diastolic velocity; age-corrected resistive index (RI) was calculated. Systemic BP was recorded. Papilledema was used to indicate intracranial hypertension. Results Twelve patients (mean age 3.1 years, range 0.4–9.5) underwent TCD; 6 subjects had papilledema. Pre-operatively, patients with papilledema, in comparison to those without, had higher TCD values, RI, and BP (all p = 0.04); post-operatively, the distinction regarding BP remained (p = 0.04). There is a significant effect of time following vault surgery with a decrease in RI (p < 0.01). Conclusion Patients with syndromic craniosynostosis who have papilledema have a different TCD profile with raised BP. Vault surgery results in increased CBFv and decrease in RI, however the associated systemic BP response to intracranial hypertension remained at short-term follow-up. [ABSTRACT FROM AUTHOR]

Introduction: Syncope is one of the most common clinical problem in children. This disorder is characterized by transient, spontaneously self-terminating loss of consciousness with brief duration and complete recovery. This situation is usually alarming for the families of patients. The mechanism of syncope is transient global brain hypoperfusion to levels below those tolerated by cerebrovascular autoregulation. Syncope can occur with many different etiologies in the pediatric population. Classification: Syncopes are divided into three major categories as neurally mediated syncope, cardiovascular-mediated syncope, and non-cardiovascular syncope. Clinical features: The major challenge in the assessment of children with syncope is that most children are asymptomatic at the time of their presentation, therefore making a careful and detailed history and a comprehensive physical examination essential in all patients. A trigger stimulus is detected in some cases, and this is an important clinical clue for the diagnosis. Cardiac causes of syncope in children are rare but can be life threatening and have the highest risk of morbidity and mortality. Misdiagnosis of epilepsy is common in patients presenting with syncope; therefore, the differential diagnosis between epileptic seizures and syncope is very important. It should be remembered that the evaluation of syncope in children is costly and diagnostic workup has a limited diagnostic yield. Conclusion: The aim of this article is to present different types of syncope and to provide new practical clinical approaches to the diagnosis, investigation, and management in the pediatric population. [ABSTRACT FROM AUTHOR]

Neurofibromatosis type 1 is characterized by nerve sheath neurofibromas associated with a number of additional clinical features, including cerebrovascular disease. The aim of this study was to use transcranial Doppler as a screening method for identifying cerebral vasculopathy in children with neurofibromatosis type 1. Forty children with neurofibromatosis type 1, aged 5 to 18 years old, were examined by transcranial Doppler. Patients presenting with hemodynamic features of arterial stenosis/occlusion on transcranial Doppler underwent magnetic resonance angiography to confirm the findings. Magnetic resonance angiography was performed on 4 children who exhibited a transcranial Doppler hemodynamic pattern indicative of cerebral vasculopathy. Among these cases, 2 presented internal carotid artery stenosis/occlusion, 1 had bilateral middle cerebral artery stenosis, and 1 presented a normal magnetic resonance angiography result. Transcranial Doppler can be used routinely in the investigation of cerebrovascular disease in neurofibromatosis type 1 patients, where magnetic resonance angiography can be subsequently applied to confirm the diagnosis, further contributing to the prevention of cerebrovascular events. [ABSTRACT FROM AUTHOR]

Emergency physicians are often the first point of contact in children presenting with acute neurological disorders. Differentiating serious disorders, such as stroke, from benign disorders, such as migraine, can be challenging. Clinical assessment influences decision-making, in particular the need for emergent neuroimaging to confirm diagnosis. This review describes the spectrum of disorders causing 'brain attack' symptoms, or acute onset focal neurological dysfunction, with particular emphasis on childhood stroke, because early recognition is essential to improve access to thrombolytic treatments, which have improved outcomes in adults. Clues to diagnosis of specific conditions are discussed. Symptoms and signs, which discriminate stroke from mimics, are described, highlighting differences to adults. Haemorrhagic and ischaemic stroke have different presenting features, which influence choice of the most appropriate imaging modality to maximise diagnostic accuracy. Improvements in the care of children with brain attacks require coordinated approaches and system improvements similar to those developed in adults. [ABSTRACT FROM AUTHOR]

Background: The clinical pattern and etiology of stroke may vary over time or with geographical location. In Asian countries, specific etiology and outcome of childhood stroke have been rarely reported. Objective: To determine the clinical and etiological pattern of childhood stroke and their outcome in a Tertiary Care Center. Materials and Methods: This study was conducted in a Tertiary Care Hospital of Kolkata over a period of 3 years. All children from 6 months to 12 years, diagnosed as childhood stroke by radio-imaging were included in our study. Children presenting with paraplegia/paraparesis were excluded. Etiologies were determined on the basis of clinical examination, related blood investigations and radio-imaging findings. Data gathered from the stroke patients were entered into a preformed proforma and appropriate statistical analyses were done. Result: Most commonly found clinical presentation was hemiparesis (70.6%). Next in place was a seizure (61.8%) and alteration of consciousness (58.8%). The most common etiology of childhood stroke in our hospital was found to be an intracranial infection (41.2%), followed by vascular etiology. Stroke was ischemic in nature in 91.2% of cases. Among the clinical features, vomiting, alteration of sensorium, and fever were significantly (P < 0.01) more in infectious cases of stroke, but hemiparesis was significantly (P < 0.05) more common in noninfectious etiology. Most of the cases of noninfectious etiology (95%) completely recovered without any persistent neurodeficit or mortality. Conclusion: Intracranial infection is the commonest etiology of stroke in pediatric patients presenting at our hospital. Commonest type is an ischemic stroke. The most of the patients completely recovered from the acute neurological insult after proper and timely management. [ABSTRACT FROM AUTHOR]

Background: Malarial retinopathy is an important finding in Plasmodium falciparum cerebral malaria, since it strengthens diagnostic accuracy, predicts clinical outcome and appears to parallel cerebral disease processes. Several angiographic features of malarial retinopathy have been described, but observations in different populations can only be reliably compared if consistent methodology is used to capture and grade retinal images. Currently no grading scheme exists for fluorescein angiographic features of malarial retinopathy. Methods: A grading scheme for fluorescein angiographic images was devised based on consensus opinion of clinicians and researchers experienced in malarial retinopathy in children and adults. Dual grading were performed with adjudication of admission fluorescein images from a large cohort of children with cerebral malaria. Results: A grading scheme is described and standard images are provided to facilitate future grading studies. Intergrader agreement was >70 % for most variables. Intravascular filling defects are difficult to grade and tended to have lower inter-grader agreement (>57 %) compared to other features. Conclusions: This grading scheme provides a consistent way to describe retinal vascular damage in paediatric cerebral malaria, and can facilitate comparisons of angiographic features of malarial retinopathy between different patient groups, and analysis against clinical outcomes. Inter-grader agreement is reasonable for the majority of angiographic signs. Dual grading with expert adjudication should be used to maximize accuracy. [ABSTRACT FROM AUTHOR]

Sickle cell disease induces specific brain alterations that involve both the macrocirculation and the microcirculation. The main overt neurovascular complications in children are infarctive stroke, transient ischaemic attack and cerebral haemorrhage. Silent cerebral infarction, cognitive dysfunction and recurrent headache are also common. Cerebrovascular disease selectively affects children with the Hb SS or HbS-β0 genotypes (i.e. sickle cell anaemia). The incidence of stroke peaks between 2 and 5 years of age (1·02/100 patient-years) and increases with the severity of the anaemia. Most strokes can be prevented by annual transcranial Doppler screening from 2 to 16 years of age and providing chronic blood transfusion when this investigation shows elevated blood-flow velocities. The role for hydroxycarbamide in children with abnormal transcranial Doppler findings is under investigation. After a stroke, chronic blood transfusion is very strongly recommended, unless haematopoietic stem cell transplantation can be performed. Routine magnetic resonance imaging shows that more than one-third of children have silent cerebral infarction, which is associated with cognitive impairments. Screening for silent infarcts seems legitimate, since their presence may lead to supportive treatments. The role for more aggressive interventions such as hydroxycarbamide or chronic blood transfusion is debated. [ABSTRACT FROM AUTHOR]

Children with perinatal stroke (PS) provide a unique opportunity to understand developing brain-behavior relations. Previous research has noted distinctive differences in behavioral sequelae between children with PS and adults with acquired stroke: children fare better, presumably due to the plasticity of the developing brain for adaptive reorganization. Whereas we are beginning to understand language development, we know little about another communicative domain, emotional expression. The current study investigates the use and integration of language and facial expression during an interview. As anticipated, the language performance of the five and six year old PS group is comparable to their typically developing (TD) peers, however, their affective profiles are distinctive: those with right hemisphere injury are less expressive with respect to affective language and affective facial expression than either those with left hemisphere injury or TD group. The two distinctive profiles for language and emotional expression in these children suggest gradients of neuroplasticity in the developing brain. [ABSTRACT FROM AUTHOR]

Purpose: To assess the impact of the duration and intensity of episodes of increased intracranial pressure on 6-month neurological outcome in adult and paediatric traumatic brain injury. Methods: Analysis of prospectively collected minute-by-minute intracranial pressure and mean arterial blood pressure data of 261 adult and 99 paediatric traumatic brain injury patients from multiple European centres. The relationship of episodes of elevated intracranial pressure (defined as a pressure above a certain threshold during a certain time) with 6-month Glasgow Outcome Scale was visualized in a colour-coded plot. Results: The colour-coded plot illustrates the intuitive concept that episodes of higher intracranial pressure can only be tolerated for shorter durations: the curve that delineates the duration and intensity of those intracranial pressure episodes associated with worse outcome is an approximately exponential decay curve. In children, the curve resembles that of adults, but the delineation between episodes associated with worse outcome occurs at lower intracranial pressure thresholds. Intracranial pressures above 20 mmHg lasting longer than 37 min in adults, and longer than 8 min in children, are associated with worse outcomes. In a multivariate model, together with known baseline risk factors for outcome in severe traumatic brain injury, the cumulative intracranial pressure-time burden is independently associated with mortality. When cerebrovascular autoregulation, assessed with the low-frequency autoregulation index, is impaired, the ability to tolerate elevated intracranial pressures is reduced. When the cerebral perfusion pressure is below 50 mmHg, all intracranial pressure insults, regardless of duration, are associated with worse outcome. Conclusions: The intracranial pressure-time burden associated with worse outcome is visualised in a colour-coded plot. In children, secondary injury occurs at lower intracranial pressure thresholds as compared to adults. Impaired cerebrovascular autoregulation reduces the ability to tolerate intracranial pressure insults. Thus, 50 mmHg might be the lower acceptable threshold for cerebral perfusion pressure. [ABSTRACT FROM AUTHOR]

Purpose: The descriptions of collateral circulation in moyamoya have so far been a mixture of topography-based and vessels' source-based analyses. We aimed to investigate the anatomy and systematize the vascular anastomotic networks in pediatric moyamoya disease. Methods: From a series of 25 consecutive complete angiographic studies of newly diagnosed children with moyamoya, 14 children had moyamoya disease and 11 were diagnosed with moyamoya syndrome, i.e., moyamoya angiopathy with some additional concomitant systemic disease. We retrospectively analyzed the arterial branches supplying the moyamoya anastomotic networks, their origin, course, location, and connections with the recipient vessels. Results: We describe four types of anastomotic networks in children with moyamoya disease, two superficial-meningeal and two deep-parenchymal. As superficial-meningeal, we defined the leptomeningeal and the durocortical networks. Apart from the previously described leptomeningeal network observed in the convexial watershed zones, we report on the basal temporo-orbitofrontal leptomeningeal network. The second superficial-meningeal network is the durocortical network, which can be basal or calvarian in location. We define as deep-parenchymal networks the nonpreviously described subependymal network and the inner striatal and inner thalamic networks. The subependymal network is fed by the intraventricular branches of the choroidal system and diencephalic perforators, which at the level of the periventricular subependymal zone, anastomose with medullary-cortical arteries as well as with striatal arteries. The inner striatal and thalamic networks are constituted by intrastriatal connections among striatal arteries and intrathalamic connections among thalamic arteries when the disease compromises the origin of one or more sources of their supply. Conclusion: The previously inexplicitly described 'moyamoya abnormal network' in pediatric moyamoya disease can be described as a composition of four anastomotic networks with distinct angioarchitecture. A better understanding of the collateralization in moyamoya may help in defining a new staging system of the disease with clinical relevance. [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/Aim To assess the impact of sevoflurane and anesthesia-induced hypotension on brain perfusion in children younger than 6 months. Background Safe lower limit of blood pressure during anesthesia in infant is unclear, and inadequate anesthesia can lead to hypotension, hypocapnia, and low cerebral perfusion. Insufficient cerebral perfusion in infant during anesthesia is an important factor of neurological morbidity. In two previous studies, we assessed the impact of sevoflurane anesthesia on cerebral blood flow ( CBF) by transcranial Doppler ( TCD) and on brain oxygenation by NIRS, in children ≤2 years. As knowledge about consequences of anesthesia-induced hypotension on cerebral perfusion in children ≤6 months is scarce, we conducted a retrospective analysis to compare the data of CBF and brain oxygenation, in this specific population. Methods We performed a retrospective analysis of data collected from our two previous studies. Baseline values of TCD or NIRS were recorded and then during sevoflurane anesthesia. From a database of 338 patients, we excluded all patients older than 6 months. Then, we compared physiological variables of TCD and NIRS population to ensure that the two groups were comparable. We compared rSO2c and TCD measurements variation according to MAP value during sevoflurane anesthesia, using anova and Student-Newman-Keuls for posthoc analysis. Results One hundred and eighty patients were included in the analysis. TCD and NIRS groups were comparable. CBF velocities (CBFV) or rSO2c reflects a good cerebral perfusion when MAP is above 45 mmHg. When MAP is between 35 and 45 mmHg, CBFV variation reflects a reduction of CBF, but rSO2c increase is the consequence of a still positive balance between CMRO2 and O2 supply. Below 35 mmHg of MAP during anesthesia, CBFV decrease and rSO2c variation from baseline is low. For each category of MAP and for the two groups, etCo2 and expired fraction of sevoflurane (FeSevo) were comparable ( anova P > 0.05). Conclusion In a healthy infant without dehydration, with normal PaCO2 and hemoglobin value, scheduled for short procedures, MAP is a good proxy of cerebral perfusion as we found that CBF assessed by CBFV and rSO2c decreased proportionally with cerebral perfusion pressure. During 1 MAC sevoflurane anesthesia, maintaining MAP beyond 35 mmHg during anesthesia is probably safe and sufficient. But when MAP decreases below 35 mmHg, CBF decreases and rSO2c variation from baseline is low despite CMRO2 reduction. In this situation, cerebral metabolic reserve is low and further changes of systemic conditions may be poorly tolerated by the brain. [ABSTRACT FROM AUTHOR]