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Background: Stroke is a major cause of mortality in children. Conditions that mimic stroke also cause severe morbidity and require prompt diagnosis and treatment. We have investigated the time to diagnosis in a cohort of children with stroke., Methods: A population-based cohort of children with stroke was prospectively identified in the south of England. Case notes, electronic hospital admission databases and radiology records were reviewed. Timing of symptom onset, presentation to hospital, first neuroimaging, first diagnostic neuroimaging and presenting clinical features were recorded., Results: Ninety-six children with an arterial ischaemic stroke (AIS) and 43 with a haemorrhagic stroke (HS) were identified. The median time from symptom onset to diagnostic neuroimaging was 24.3 h in AIS and 2.9 h in HS. The initial imaging modality was CT in 68% of cases of AIS. CT was diagnostic of AIS in 66% of cases. MRI was diagnostic in 100%. If initial neuroimaging was non-diagnostic in AIS, then median time to diagnosis was 44 h. CT was diagnostic in 95% of HS cases. Presentation outside normal working hours resulted in delayed neuroimaging in AIS (13 vs 3 h, p=0.032). Diffuse neurological signs or a Glasgow Coma Scale <9 resulted in more expeditious neuroimaging in both HS and AIS., Conclusions: The diagnosis of AIS in children is delayed at every stage of the pathway but most profoundly when the first neuroimaging is CT scanning, which is non-diagnostic. MRI should be the initial imaging modality of choice in any suspected case of childhood AIS., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Acute hemiparesis in children is a common clinical syndrome presenting to a variety of care settings. The recognition and the differential diagnosis is challenging, particularly in young children. Arterial ischaemic stroke (AIS) is the primary diagnosis to be considered as this requires emergency investigations and management; however, there are several conditions collectively described as 'stroke mimics' that need consideration. Accurate diagnosis is essential for appropriate management. Clinical data combined with neuroimaging are important for accurate diagnosis and management. This review and the accompanying illustrative case vignettes suggest a practical approach to differential diagnosis and management of children presenting with acute hemiparesis., (© 2015 Mac Keith Press.)

Background: Arterial ischaemic stroke is an important cause of acquired brain injury in children. Few prospective population-based studies of childhood arterial ischaemic stroke have been undertaken. We aimed to investigate the epidemiology and clinical features of childhood arterial ischaemic stroke in a population-based cohort., Methods: Children aged 29 days to less than 16 years with radiologically confirmed arterial ischaemic stroke occurring over a 1-year period (July 1, 2008, to June 30, 2009) residing in southern England (population denominator 5·99 million children) were eligible for inclusion. Cases were identified using several sources (paediatric neurologists and trainees, the British Paediatric Neurology Surveillance Unit, paediatricians, radiologists, physiotherapists, neurosurgeons, parents, and the Paediatric Intensive Care Audit Network). Cases were confirmed by personal examination of cases and case notes. Details of presenting features, risk factors, and investigations for risk factors were recorded by analysis of case notes. Capture-recapture analysis was used to estimate completeness of ascertainment., Findings: We identified 96 cases of arterial ischaemic stroke. The crude incidence of childhood arterial ischaemic stroke was 1·60 per 100 000 per year (95% CI 1·30-1·96). Capture-recapture analysis suggested that case ascertainment was 89% (95% CI 77-97) complete. The incidence of arterial ischaemic stroke was highest in children aged under 1 year (4·14 per 100 000 per year, 95% CI 2·36-6·72). There was no difference in the risk of arterial ischaemic stroke between sexes (crude incidence 1·60 per 100 000 per year [95% CI 1·18-2·12] for boys and 1·61 per 100 000 per year [1·18-2·14] for girls). Asian (relative risk 2·14, 95% CI 1·11-3·85; p=0·017) and black (2·28, 1·00-4·60; p=0·034) children were at higher risk of arterial ischaemic stroke than were white children. 82 (85%) children had focal features (most commonly hemiparesis) at presentation. Seizures were more common in younger children (≤1 year) and headache was more common in older children (>5 years; p<0·0001). At least one risk factor for childhood arterial ischaemic stroke was identified in 80 (83%) cases., Interpretation: Age and racial group, but not sex, affected the risk of arterial ischaemic stroke in children. Investigation of such differences might provide causative insights., Funding: The Stroke Association, UK., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Intracranial arteriovenous shunts (AVSs) in children can be divided into pial arteriovenous malformations, vein of Galen malformations, and arteriovenous fistulae (AVF). Dural AVF and dural sinus malformations are rare entities within this group. The relative immaturity of the anatomy and physiology of the neonatal and infant brain results in the inability of the hydrovenous system to compensate in the face of such disorders. Thus, the clinical presentation reflects this difference in the underlying anatomy, physiology, and disorder between children and adults. In this article, we briefly review the presentation, natural history and management of these entities., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Acute ischemic stroke affects 3.3 of 100,000 children per year. The causes of AIS in children can be broadly divided into the following 6 categories: cardiac disese, sickle cell disease, moyamoya, arterial dissection, other arteriopathies, and other causes. Approximately 24% of the cases are classified as idiopathic. Magnetic resonance imaging (MRI) and cerebral angiography play an important role in the determining the causes of an AIS in children. Medical approaches, including anticoagulation, anti-inflammatories, and antiplatelet therapies, surgical revascularization and endovascular approaches may have a role in the management of AIS in children., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background and Purpose: The implementation of uniform nomenclature and classification in adult arterial ischemic stroke (AIS) has been critical for defining outcomes and recurrence risks according to etiology and in developing risk-stratified treatments. In contrast, current classification and nomenclature in childhood AIS are often overlapping or contradictory. Our purpose was to develop a comprehensive consensus-based classification system for childhood AIS., Methods: Using a modified-Delphi method, members of the International Pediatric Stroke Study (IPSS) developed the Childhood AIS Standardized Classification And Diagnostic Evaluation (CASCADE) criteria. Two groups of pediatric stroke specialists from the IPSS classified 7 test cases using 2 methods each: (1) classification typical of the individual clinician's current clinical practice; and (2) classification based on the CASCADE criteria. Group 1 underwent in-person training in the utilization of the CASCADE criteria. Group 2 classified the same cases via an online survey, including definitions but without training. Inter-rater reliability (IRR) was assessed via multi-rater unweighted κ-statistic., Results: In Group 1 (with training), IRR was improved using CASCADE criteria (κ=0.78, 95% CI=[0.49, 0.94]), compared with typical clinical practice (κ=0.40, 95% CI=[0.11, 0.60]). In Group 2 (without training), IRR was lower than among trained raters (κ=0.61, 95% CI=[0.29, 0.77]), but higher than current practice (κ=0.23, 95% CI=[0.03, 0.36])., Conclusions: A new, consensus-based classification system for childhood AIS, the CASCADE criteria, can be used to classify cases with good IRR. These preliminary findings suggest that the CASCADE criteria may be particularity useful in the setting of prospective multicenter studies in childhood-onset AIS, where standardized training of investigators is feasible.

Childhood stroke syndromes are an important cause of mortality and morbidity. This paper focuses on the recent advances in arterial ischaemic stroke beyond the neonatal period. Vascular risk factors are identified in the majority of children and guide both acute and longer term treatments, as well as determining prognosis. Contrary to popular belief many children have residual impairments encompassing a wide range of domains. National and international collaborations are facilitating an increase in the understanding of childhood stroke and have the eventual aim of conducting trials of potential therapeutic interventions.

Background: Cerebral arteriography (CA) remains the gold standard in delineating both intra- and extracranial vascular anatomy. Most data relating to the safety of CA are drawn from studies of adult patients in whom the practicalities of the procedure, range of potential pathologies and comorbid factors are different from those in children., Objective: To evaluate the current local and neurological complication rates of paediatric CA in the setting of a tertiary level children's hospital in the UK., Materials and Methods: Data from patients who had undergone CA between January 1998 and July 2003 were reviewed. The medical, anaesthetic and nursing records, and angiography reports were reviewed for all identified patients. The following parameters were extracted and entered into a proforma: gender, age, ethnicity, diagnosis, cerebrovascular diagnosis, referral source, date of CA, number of vessels catheterized and local and neurological complications., Results: A total of 176 CA studies were undertaken in 150 patients (median age 7.3 years, range neonate to 19 years; 83 males, 67 females) during the 5.5-year study period. The majority of referrals originated from the neurology (58%) and neurosurgery services (27.8%). No neurological complications or deaths occurred. Local complications occurred in eight children (4.5%). Five children had a groin haematoma and two had bleeding at the puncture site. A single child had a reduced pedal pulse distal to the site of catheterization, but Doppler imaging was normal., Conclusions: CA has a continuing role in the evaluation of cerebrovascular pathologies in children. Neurological complications are rare and local complications are not uncommon (around 5%), but are not usually serious.

Introduction and Aims: The role of and optimal surgical revascularisation technique for paediatric moyamoya syndrome (MMS) are controversial. In this literature review our primary aim was to evaluate the evidence base for the efficacy of surgical revascularisation for the treatment of paediatric MMS. Secondary aims were to estimate the rate of peri-operative complications and to ascertain whether direct or indirect revascularisation techniques resulted in differences in clinical or radiological outcomes., Methods: Papers describing surgical revascularisation and its outcome in the treatment of children with MMS were identified from the OVID Medline database (1966-2004). Only papers in English were reviewed. Data were abstracted using a standardised form., Results: Fifty-seven studies, including data on 1,448 patients, were reviewed. Most were Japanese; 10% were from Western institutions. Indications for revascularisation were described in <15% of studies and varied between centres. Indirect procedures were most commonly performed (alone in 73% of cases, combined with direct procedures in 23%). The rates of peri-operative stroke and reversible ischaemic events were 4.4 and 6.1% respectively. Out of 1,156 (87%) patients, 1,003 derived symptomatic benefit from surgical revascularisation (complete disappearance or reduction in symptomatic cerebral ischaemia), with no significant difference between the indirect and direct/combined groups. Data on developmental and functional outcomes were limited and of uncertain significance as they were not related to pre-operative status. Good collateral formation was significantly more frequent in the direct/combined group than in the indirect group (chi(2), p<0.001)., Conclusions: Data from the medical literature suggest that surgical revascularisation is a safe intervention for paediatric MMS and most treated patients derive some symptomatic benefit. However, paucity of data on selection criteria and more global outcome measures means that the impact of surgical revascularisation on natural history remains uncertain. Direct and/or combined procedures provide better revascularisation, but this is not associated with differences in symptomatic outcome. International standardisation of the clinical approach to the treatment of paediatric MMS is urgently needed to critically evaluate the optimal indications for and timing of surgical revascularisation.

Objective: Motor impairment after neonatal and childhood-onset ischemic stroke (IS) is common, although the prevalence and type of hemiparesis differs between the 2 age groups. Lesion topography is an important predictor of hemiparesis after neonatal IS, but it is not known if the same topographic predictors of adverse motor outcome apply to childhood-onset IS. We used a consistent approach to define lesion topography and evaluate motor outcome in both age groups to (1) investigate whether early topographic predictors of hemiparesis after unilateral middle cerebral artery-territory stroke are the same in neonates and older children and (2) compare the prevalence of dystonia and loss of independent finger movements between the 2 age groups., Design: Twenty-eight patients with neonatal-onset IS (Hammersmith Hospital, London, United Kingdom) were studied together with 43 patients with childhood-onset IS (Great Ormond Street Hospital, London, United Kingdom). All patients had exclusive unilateral middle cerebral artery-territory IS. Lesion topography was studied by using the first magnetic resonance image acquired after the onset of symptoms and was coded for involvement of cerebral cortex (CC), posterior limb of the internal capsule (PLIC), basal ganglia (BG), and white matter. The primary outcome was hemiparesis, and secondary outcomes were dystonia and loss of age-appropriate independent finger movements., Results: Hemiparesis was more common after childhood-onset IS (56%) than neonatal-onset IS (24%). In neonatal-onset IS, concomitant involvement of BG, CC, and PLIC predicts the development of hemiparesis (odds ratio: 99; 95% confidence interval: 5.2-1883.8), and no child with 1 or 2 of these structures involved developed hemiparesis. In contrast, in childhood-onset IS, concomitant BG, CC, and PLIC lesions tended to be associated with hemiparesis (9 of 11), but this adverse outcome was seen also among patients with 1- or 2-site involvement. However, hemiparesis was less likely if the infarction involved BG only (odds ratio: 0.162; 95% confidence interval: 0.036-0.729). Dystonia was present in 15 of 24 in the childhood-onset group with hemiparesis but was not seen after neonatal-onset IS. In both age groups upper-limb impairment was more severe than lower-limb impairment, with frequent loss of independent hand function among hemiparetic patients., Conclusions: In neonatal and childhood-onset IS, early magnetic resonance imaging provides useful prognostic information about subsequent motor outcome. There are differences in the functional response of the neuromotor system to injury between the 2 age groups that cannot be attributed to methodological differences alone.