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Cerebral palsy (CP) is a broad diagnosis unbound by aetiology and is based on a clinical examination demonstrating abnormalities of movement or posture. CP represents a static neurological condition, provided that neurodegenerative conditions, leukoencephalopathies and neuromuscular disorders are excluded. In paediatrics, the genetic conditions associated with CP are rapidly increasing, with primary and overlapping neurodevelopmental conditions perhaps better categorised by the predominant clinical feature such as CP, intellectual disability, autism spectrum disorder or epilepsy. Progress in molecular genetics may challenge what constitutes CP, but a genetic diagnosis does not negate the CP diagnosis. As clinicians working in the field, we discuss the changing tide of CP. Neuroimaging provides essential information through pattern recognition and demonstration of static brain changes. We present examples of children where a layered clinical diagnosis or dual aetiologies are appropriate. We also present examples of children with genetic causes of CP to highlight the challenges and limitations of neuroimaging to provide an aetiological diagnosis. In consultation with a geneticist, access to genomic testing (exome or genome sequencing) is now available in Australia under Medicare billing for children under the age of 10 with dysmorphic features, one or more major structural organ anomalies, (an evolving) intellectual disability or global developmental delay. We encourage the uptake of genomic testing in CP, because it can be difficult to tell whether a child has an environmental or genetic cause for CP. A specific genetic diagnosis may change patient management, reduce guilt and enable more distinctive research in the future to assist with understanding disease mechanisms.

Background: Data from the early pandemic revealed that 0.62% of children hospitalized with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) had an acute arterial ischemic stroke (AIS). In a larger cohort from June 2020 to December 2020, we sought to determine whether our initial point estimate was stable as the pandemic continued and to understand radiographic and laboratory data that may clarify mechanisms of pediatric AIS in the setting of SARS-CoV-2. Methods: We surveyed international sites with pediatric stroke expertise to determine numbers of hospitalized SARS-CoV-2 patients Results: Sixty-one centers from 21 countries provided AIS data. Forty-eight centers (78.7%) provided SARS-CoV-2 hospitalization data. SARS-CoV-2 testing was performed in 335/373 acute AIS cases (89.8%) compared with 99/166 (59.6%) in March to May 2020, P P =0.78. Of the 22 of 23 AIS cases with SARS-CoV-2 in whom we could collect additional data, SARS-CoV-2 was the main stroke risk factor in 6 (3 with arteritis/vasculitis, 3 with focal cerebral arteriopathy), a contributory factor in 13, and incidental in 3. Elevated inflammatory markers were common, occurring in 17 (77.3%). From centers with SARS-CoV-2 hospitalization data, of 7231 pediatric patients hospitalized with SARS-CoV-2, 23 had AIS (0.32%) compared with 6/971 (0.62%) from March to May 2020, P =0.14. Conclusions: The risk of AIS among children hospitalized with SARS-CoV-2 appeared stable compared with our earlier estimate. Among children in whom SARS-CoV-2 was considered the main stroke risk factor, inflammatory arteriopathies were the stroke mechanism.

Background and Objectives:Bell’s palsy is the third most frequent diagnosis in children with sudden onset neurological dysfunction. The cost-effectiveness of treating Bell’s palsy with prednisolone in children is unknown. We aimed to assess the cost-effectiveness of prednisolone in treating Bell’s Palsy in children compared with placebo.Methods:This economic evaluation was a prospectively planned secondary analysis of a double-blinded, randomized, placebo-controlled superiority trial (BellPIC) conducted from 2015 to 2020. Time horizon was 6 months since randomization. Children aged 6 months to Results:The mean cost per patient was A$760 in the prednisolone group and A$693 in the placebo group over the 6-month period (difference A$66, 95% confidence interval [CI]: -A$47, A$179). QALYs over 6-months was 0.45 in the prednisolone group and 0.44 in the placebo group (difference 0.01, 95%CI: -0.01, 0.03). The incremental cost to achieve one additional recovery was estimated to be A$1577 using prednisolone compared with placebo, and cost per additional QALY gained was A$6625 using prednisolone compared with placebo. Given a conventional willingness-to-pay threshold of A$50,000 per QALY gained (equivalent to US$35,000 or £28,000), prednisolone is very likely cost-effective (probability is 83%), . Sub-group analysis suggests that this was primarily driven by the high probability of prednisolone being cost-effective in children aged 12-18 years (probability is 98%) and much less so for those Discussion:This provides new evidence to stakeholders and policy makers when considering whether to make prednisolone available in treating Bell’s palsy in children aged 12-18 years.Trial Registration:Australian New Zealand Clinical Trials Registry ACTRN12615000563561.

Background: There appears to be a gap in the literature with regard to nursing students’ participation in designing learning resources to prepare them for the reality of clinical placements. In addition, the existing research on preparation focuses on skills competency and overlooks the emotional preparation required to navigate the practice context, while preparation of early-year students is not prioritised. Aim: This study had two aims: to provide nursing students with processes and tools to give them a voice in the development of learning resources to prepare for clinical placement; and to address some of the power imbalances between nursing students and clinical supervisors. Methods: This action research study was undertaken collaboratively with students and academic staff as co-researchers and was underpinned by the theoretical perspectives of transformational learning and person-centredness. It used a range of creative methods, such as workshops, critical creativity and critical dialogue. Conclusion: Emotional connection and vulnerability were found to enhance healthful (supervisory) relationships. The co-researchers developed a Student-Led Conversation Form and a process to support students to undertake and lead a conversation with their clinical supervisors and create shared values. Significantly, emotional preparation for clinical placements was found to be important for nursing students facing the reality of practice. Implications for practice: • Innovative approaches to curriculum development are enabled when academic staff authentically engage with nursing students • First-year nursing students experience a sense of empowerment when they engage in student-led learning • Person-centred learning requires academic staff and nursing students to be challenged to develop emotional literacy skills

Background and Purpose: Recent studies using automated perfusion imaging software have identified adults most likely to benefit from reperfusion therapies in extended time windows. The time course of penumbral tissue is poorly characterized in childhood arterial ischemic stroke (AIS). We explore the feasibility of using automated perfusion-diffusion imaging software to characterize penumbra in childhood AIS. Methods: An observational cohort study of children with acute unilateral AIS presenting to our institution. Diffusion-weighted imaging and dynamic susceptibility contrast perfusion magnetic resonance imaging performed within 72 hours of symptom onset were necessary for inclusion. Perfusion-diffusion mismatch was estimated using RAPID software. Ischemic core was defined as apparent diffusion coefficient −6 mm 2 /s and hypoperfusion as Tmax >6 seconds. Favorable mismatch profile was defined as core volume Results: Twenty-nine children (median 8 years old, interquartile range, 4.4–14.6) were included (26 unilateral middle cerebral artery and 3 unilateral cerebellar infarcts). Median Pediatric National Institutes of Health Stroke Scale was 4 (interquartile range, 3–11). Most cases had cryptogenic (n=11) or focal cerebral arteriopathy (n=9) causes. Median time-to-imaging =13.7 hours (interquartile range, 7.5–25.3). RAPID detected an ischemic core in 19 (66%) patients. In the remaining cases, the mean apparent diffusion coefficient values were mostly higher than the threshold as the majority of these presentations were delayed (median >21 hours) and infarct volumes were small ( Conclusions: This study demonstrates it is feasible to rapidly assess perfusion-diffusion mismatch in childhood AIS using automated software. Favorable mismatch profiles, using adult-based parameters, persisted beyond the standard 4.5 hours window for thrombolysis, suggesting potential therapeutic benefit of RAPID use. Further work is required to determine the utility of perfusion-based imaging to guide clinical decision making, whether adult thresholds require modification in childhood AIS, and to investigate the effect of time-delay and cause on mismatch characteristics.

Background and Purpose: The aims of this study were to assess the prevalence of multidimensional fatigue symptoms 5 years after pediatric arterial ischemic stroke and identify factors associated with fatigue. Methods: Thirty-one children (19 males) with pediatric arterial ischemic stroke, participating in a larger prospective, longitudinal study, were recruited to this study at 5 years poststroke. Parent- and self-rated PedsQL Multidimensional Fatigue Scale scores were compared with published normative data. Associations between parent-rated PedsQL Multidimensional Fatigue Scale, demographics, stroke characteristics, and concurrent outcomes were examined. Results: Parent-rated total, general and cognitive fatigue were significantly poorer than population norms, with more than half of all parents reporting fatigue symptoms in their children. One-third of children also reported experiencing fatigue symptoms, but their ratings did not differ significantly from normative expectations, as such, all further analyses were on parent ratings of fatigue. Older age at stroke and larger lesion size predicted greater general fatigue; older age, female sex, and higher social risk predicted more sleep/rest fatigue. No significant predictors of cognitive fatigue were identified and only older age at stroke predicted total fatigue. Greater fatigue was associated with poorer adaptive functioning, motor skills, participation, quality of life, and behavior problems but not attention. Conclusions: Fatigue is a common problem following pediatric arterial ischemic stroke and is associated with the functional difficulties often seen in this population. This study highlights the importance of long-term monitoring following pediatric arterial ischemic stroke and the need for effective interventions to treat fatigue in children.

A Taylor–Galerkin finite element time marching scheme is derived to numerically simulate the flow of a compressible and nonisothermal viscoelastic liquid between eccentrically rotating cylinders. Numerical approximations to the governing flow and constitutive equations are computed over a custom refined unstructured grid of piecewise linear Galerkin finite elements. An original extension to the DEVSS formulation for compressible fluids is introduced to stabilise solutions of the discrete problem. The predictions of two models: the extended White–Metzner and FENE-P-MP are presented. Comparisons between the torque and load bearing capacity predicted by both models are made over a range of viscoelastic parameters. The results highlight the significant and interacting effects of elasticity and compressibility on journal torque and resultant load, and the stability of the journal bearing system.

Background and Purpose: Childhood and adolescence coincide with rapid maturation of distributed brain networks supporting social cognition; however, little is known about the impact of early ischemic brain insult on the acquisition of these skills. This study aimed to examine the influence of arterial ischemic stroke (AIS) on facial emotion recognition and theory of mind (ToM) abilities of children and adolescents initially recruited to a single-center, prospective longitudinal study of recovery following AIS. Methods: The study involved 67 participants, including 30 children with AIS (mean time since stroke=5 years) and 37 age-matched typically developing controls who were assessed on measures of cognitive ToM, facial emotion recognition, and affective ToM. Acute clinical magnetic resonance imaging, including diffusion-weighted imaging sequences, were used to evaluate prospective structure-function relationships between acute lesion characteristics (size, location, and arterial territories affected) and long-term social cognitive abilities. Results: Relative to age-matched typically developing controls, children with AIS showed significantly worse performance on measures of basic facial emotion processing, cognitive ToM, and affective ToM. In univariate models, poorer ToM was associated with larger infarcts, combined cortical-subcortical pathology, and involvement of multiple arterial territories. In multivariate analyses, larger lesions and multiterritory infants were predictive of ToM processing but not facial emotion recognition. Poorer cognitive ToM predicted less frequent prosocial behavior and increased peer problems. Conclusions: Social cognitive skills appear vulnerable to disruption from early ischemic brain insult. In the first study to examine social cognition in a prospective cohort of children with AIS, our findings suggest that acute magnetic resonance imaging-based lesion characteristics may have predictive value for long-term social cognitive outcomes and may assist to identify children at elevated risk for social cognitive dysfunction.

This study aimed to investigate the prevalence of fractures and non-fracture injuries, including associated risk factors, in children with epilepsy prescribed antiseizure medications (ASM). A controlled, cross-sectional study was conducted in a hospital outpatient setting, comparing children with epilepsy prescribed ASMs with their non-epileptic siblings. Information was collected by questionnaire included history of fractures, non-fracture injuries and epilepsy, comorbidities and ASM use. 261 participants completed the questionnaire, 133 children with epilepsy (aged 10.7 ± 3.5 years, mean ± SD) and 128 siblings (10.1 ± 3.7 years). There were 49 non-seizure-related fractures in 34 ASM patients while prescribed ASMs, compared with 21 lifetime fractures in 15 controls, giving a 2.7 (95% CI 1.3-5.3, p = 0.007) times greater fracture prevalence in children treated with ASMs compared to healthy siblings. The rates of non-fracture injuries were similar across groups, except that concussion was more common in children taking ASMs (9.0% vs 1.6%, p = 0.026). Duration of ASM use and generalized tonic-clonic seizures (GTCS) were independent predictors of fractures (OR 1.55; 95% CI 1.03-2.31, p = 0.03; OR 2.50; 95% CI 1.05-5.94, p = 0.04, respectively). Fewer than 20% of participants and/or their families were aware that ASM use was related to bone health. Children with epilepsy treated with ASMs had a higher fracture prevalence than their sibling controls. Duration of ASM treatment and GTCS were associated with fracture risk. Longitudinal prospective studies are required to further explore risk and the direct impact of epilepsy on bone health.

Background and Objective:Corticosteroids are used to treat the early stages of idiopathic facial paralysis (Bell’s palsy) in children, but their effectiveness is uncertain. We set out to determine if prednisolone improves the proportion of children with Bell’s palsy with complete recovery at one month.Methods:We conducted a double-blind, placebo-controlled, randomised trial of prednisolone in children presenting to emergency departments with Bell’s palsy. Patients aged 6 months to less than 18 years, recruited within 72 hours after symptom onset, were randomly assigned to receive 10 days of treatment with oral prednisolone (approximately 1 mg/kg) or placebo. The primary outcome was complete recovery of facial function at 1 month rated on the House–Brackmann scale. Secondary outcomes included facial function, adverse events and pain up to 6 months. Target recruitment was n=540 (270 per group).Results:Between 13 October 2015 to 23 August 2020, 187 children were randomised (94 to prednisolone and 93 to placebo) and included in the intention-to-treat analysis. At 1 month, the proportions of patients who had recovered facial function were 49% (n=43/87) in the prednisolone group compared with 57% (n=50/87) in the placebo group (risk difference -8.1%, 95% CI -22.8 to 6.7; adjusted odds ratio [aOR] 0.7, 95% CI 0.4 to 1.3). At 3 months these proportion were 90% (n=71/79) for the prednisolone group versus 85% (n=72/85) for the placebo group (risk difference 5.2%, 95%, CI -5.0 to 15.3; aOR 1.2, 95% CI 0.4 to 3.0) and at 6 months 99% (n=77/78) and 93% (n=76/82) respectively (risk difference 6.0%, 95% CI -0.1 to 12.2; aOR 3.0 95% CI 0.5 to 17.7) There were no serious adverse events and little evidence for group differences in secondary outcomes.Discussion:In children with Bell’s palsy the vast majority recover without treatment. The study, although underpowered, does not provide evidence that early treatment with prednisolone improves complete recovery.Registration:Registered with the Australian New Zealand Clinical Trials Registry ACTRN12615000563561, registered 1 June 2015, ://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=368505&isReview=trueClassification of evidence:This study provides Class I evidence that for children with Bell's palsy, prednisolone does not significantly change recovery of complete facial function at one month. However, the study lacked the precision to exclude an important harm or benefit from prednisolone.

Contains fulltext : 231688.pdf (Publisher’s version ) (Closed access) PURPOSE: Pathogenic variants in the X-linked gene NEXMIF (previously KIAA2022) are associated with intellectual disability (ID), autism spectrum disorder, and epilepsy. We aimed to delineate the female and male phenotypic spectrum of NEXMIF encephalopathy. METHODS: Through an international collaboration, we analyzed the phenotypes and genotypes of 87 patients with NEXMIF encephalopathy. RESULTS: Sixty-three females and 24 males (46 new patients) with NEXMIF encephalopathy were studied, with 30 novel variants. Phenotypic features included developmental delay/ID in 86/87 (99%), seizures in 71/86 (83%) and multiple comorbidities. Generalized seizures predominated including myoclonic seizures and absence seizures (both 46/70, 66%), absence with eyelid myoclonia (17/70, 24%), and atonic seizures (30/70, 43%). Males had more severe developmental impairment; females had epilepsy more frequently, and varied from unaffected to severely affected. All NEXMIF pathogenic variants led to a premature stop codon or were deleterious structural variants. Most arose de novo, although X-linked segregation occurred for both sexes. Somatic mosaicism occurred in two males and a family with suspected parental mosaicism. CONCLUSION: NEXMIF encephalopathy is an X-linked, generalized developmental and epileptic encephalopathy characterized by myoclonic-atonic epilepsy overlapping with eyelid myoclonia with absence. Some patients have developmental encephalopathy without epilepsy. Males have more severe developmental impairment. NEXMIF encephalopathy arises due to loss-of-function variants.

Background Focal cerebral arteriopathy (FCA), a major cause of childhood arterial ischemic stroke (AIS), can progress and lead to increased infarct size and/or recurrent stroke. Evaluating treatment options depends on the ability to quantify reliably the degree of stenosis in FCA. Aims We validated the recently introduced FCA severity score (FCASS) in an independent cohort from the Swiss Neuro-Paediatric Stroke Registry (SNPSR). Materials and methods We included children with FCA who had MR or CT angiography and a Pediatric Stroke Outcome Measure (PSOM) at 6-months and 2-years post-stroke. A paediatric neuroradiologist applied the FCASS and the modified pediatric Alberta Stroke Program Early Computed Tomography Score (ASPECTS), a measure of infarct volume, to all available imaging. Two senior paediatric stroke neurologists and a neuroradiology fellow independently assigned FCASS scores to test interrater reliability. Pairwise correlations between FCASS, pedASPECTS, and PSOM were examined. Results Thirty-two children [median (IQR) age = 5.9 (1.8, 9.6), 19 males] were included. The median maximum FCASS score at any time was 9 (IQR 6, 12; range 3, 16). Larger infarct volume scores correlated with both higher maximum FCASS scores and worse post-stroke outcomes, although we found no direct correlation between FCASS and outcomes. Stroke neurologists tended to assign lower FCASS scores than the neuroradiologist, but interrater reliability was predominantly good. Conclusions In this independent validation cohort, higher maximum FCASS correlated with greater infarct volume scores that also correlated with worse neurological outcomes. Scoring by non-imaging specialists seems to be valuable, although differences are present.

ObjectiveTo determine that children with arterial ischemic stroke (AIS) due to an identifiable arteriopathy are distinct from those without arteriopathy and that each arteriopathy subtype has unique and recognizable clinical features.MethodsWe report a large, observational, multicenter cohort of children with AIS, age 1 month to 18 years, enrolled in the International Pediatric Stroke Study from 2003 to 2014. Clinical and demographic differences were compared by use of the Fisher exact test, with linear step-up permutation min-p adjustment for multiple comparisons. Exploratory analyses were conducted to evaluate differences between cases of AIS with and without arteriopathy and between arteriopathy subtypes.ResultsOf 2,127 children with AIS, 725 (34%) had arteriopathy (median age 7.45 years). Arteriopathy subtypes included dissection (27%), moyamoya (24.5%), focal cerebral arteriopathy–inflammatory subtype (FCA-i; 15%), diffuse cerebral vasculitis (15%), and nonspecific arteriopathy (18.5%). Children with arteriopathic AIS were more likely to present between 6 and 9 years of age (odds ratio [OR] 1.93, p = 0.029) with headache (OR 1.55, p = 0.023), multiple infarctions (OR 2.05, p < 0.001), sickle cell anemia (OR 2.9, p = 0.007), and head/neck trauma (OR 1.93, p = 0.018). Antithrombotic use and stroke recurrence were higher in children with arteriopathy. Among arteriopathy subtypes, dissection was associated with male sex, older age, headache, and anticoagulant use; FCA-i was associated with hemiparesis and single infarcts; moyamoya was associated with seizures and recurrent strokes; and vasculitis was associated with bilateral infarctions.ConclusionSpecific clinical profiles are associated with cerebral arteriopathies in children with AIS. These observations may be helpful indicators in guiding early diagnosis and defining subgroups who may benefit most from future therapeutic trials.

Background: The Student International Community of Practice is a global network of more than 30 doctoral candidates affiliated with the Centre for Person-centred Practice Research, at Queen Margaret University, Edinburgh. An ongoing challenge the community faces is its changing and growing membership; as members progress and complete their doctoral studies they leave the group, and as the centre grows new community members (doctoral candidates) join. Aim: To explore and describe the experience of being a member of the Student International Community of Practice, using a creative process of reflection and collaborative analysis, and to identify the implications for the future of the community and the integration of new members. Conclusion: The Student International Community of Practice is a valuable social learning experience for those who are members. It will continue to be a flourishing safe space if, despite its changing membership, we pay explicit attention to our agreed purpose, ways of working and values. Implications for practice/academic research: Belonging to a sustainable and flourishing community of practice enhances learning, and decreases isolation and loneliness on the doctoral journey A community of practice is sustainable when it is underpinned by a clear purpose, agreed ways of working and values, to which all members consistently pay explicit attention

In paediatric migraine, ibuprofen, acetaminophen and triptans are safe, effective therapies but there is scant paediatric data informing second-line emergency treatment.Retrospective cohort study of children diagnosed with migraine at a tertiary children's hospital ED.There were 207 children with migraine over a 1 year period. 46% received simple oral analgesia. 25% intravenous chlorpromazine, of whom 45% received further analgesia.While intravenous chlorpromazine as second-line agent was mostly safe, it had unclear efficacy given the requirement for further treatment and hospital admissions.

Objective To determine the frequency of paroxysmal nonepileptic events in children with cerebral palsy due to brain injury who have epilepsy and to describe the factors associated with paroxysmal nonepileptic events. Methods: Retrospective, population-based study of children from the Victorian CP Register born 1999–2006. Neuroimaging, medical records, electroencephalograms (EEG), and EEG requests were analyzed. Results: Of the included 256 children, 87 had epilepsy. EEGs (with video correlation) were available for 82 of 87. Eighteen (18/82, 22%) had epileptic events captured on EEG. Twenty-one (21/82, 26%) had paroxysmal nonepileptic events captured on EEG. The majority (13/18, 77%) of children with epileptic events also had paroxysmal nonepileptic events captured. Ten parents and carers continued to report events as epileptic despite there being no ictal EEG correlate for specific events on multiple EEGs. There were no clear associations to identify which children would have ongoing paroxysmal nonepileptic events reported. Conclusions: Paroxysmal nonepileptic events were captured on EEG in one-fourth of children from this cerebral palsy cohort with epilepsy and available EEG. Half the parents and carers reported previously identified paroxysmal nonepileptic events as epileptic on subsequent EEGs, highlighting the need for clearer counseling so that parents better understand seizure semiology in children with EEG-proven paroxysmal nonepileptic events.

Objective:Recent trials provide high-quality evidence for second-line treatment of convulsive status epilepticus (CSE) in children. However, the most effective medications for other seizure emergencies are poorly understood without established treatment algorithms. We investigated children presenting to the emergency department with repetitive or prolonged convulsions who required intravenous long-acting antiseizure medications, to determine the relative importance and treatment responsiveness of status epilepticus and seizure clusters. Methods: Retrospective observational study in the emergency department, Royal Children's Hospital, Melbourne, Australia (annual census 90 000) using hospital electronic medical records data of patients presenting with seizures in 2018. For patients receiving parenteral long-acting antiseizure medications, seizures were categorized as convulsive status epilepticus, nonconvulsive status epilepticus, and seizure clusters. Results: 1468 patients (2% of all visits) presented with seizures to the emergency department in 2018. Long-acting antiseizure medications were administered to 97 (7%) children for the emergency management of seizures. The majority presented with seizure clusters (n = 69; 71%). Only 11 (11%) were in convulsive status epilepticus and 17 (18%) in nonconvulsive status epilepticus. In convulsive status epilepticus, nonconvulsive status epilepticus, and seizure clusters, phenytoin was used in 27%, 53%, and 58% and levetiracetam in 73%, 47%, and 32%, respectively. Conclusions:Convulsive status epilepticus represents a small portion of patients requiring parenteral long-acting antiseizure medications. Seizure clusters accounted for >6 times the number of convulsive status epilepticus, yet evidence and treatment algorithms are lacking.

Background: Maternal and newborn stroke occur during the same period when physiological and anatomic connections between mother and infant may provide a rationale for common pathogenetic mechanisms. The similarities and differences between maternal and newborn stroke remain poorly understood. We created the International Maternal Newborn Stroke Registry - the first database to explore these relationships. Methods: Eleven international sites participated; 9 enrolled cases for this analysis. Eligible cases were newborns (from 28 weeks gestation to 28 postnatal days) or mothers (peripartum or within 6 weeks postpartum) diagnosed with stroke (ischemic, hemorrhagic, cerebral sinovenous thrombosis). Preterm infants (< 28 weeks gestation) and neonates with germinal matrix/intraventricular hemorrhage were excluded. Descriptive statistics were performed to compare stroke sub-types, demographic and pregnancy-related risk factors. Results: We enrolled 103 cases, 69 newborn and 34 maternal. Median maternal age was 29 years (IQR 25-36) for the maternal group and 32 years (IQR 30-36) for the neonatal group. Women with maternal strokes were more likely to have pre-eclampsia (15%) and migraine (44%). Compared to maternal strokes, newborn strokes occurred more often in women from urban locations, were ischemic, and were less likely to be associated with alcohol use and pre-eclampsia during pregnancy (Table). Conclusion: In this novel registry, we describe factors that impact both maternal and newborn stroke. Urban area of residence and maternal age > 30 were associated with newborn strokes, while migraine and pre-eclampsia were strongly associated with maternal strokes. Differing mechanisms and stroke subtypes may explain these differences. In addition, lack of access to healthcare in certain geographical locations may lead to under-identification. Further research is needed to determine if these results are consistent when applied to a larger sample size.

Background: Despite its importance in being among the top 10 causes of childhood death, there is limited data on the incidence of stroke in children and whether this has changed over time. Aims: We performed a systematic review and meta-analysis to estimate the worldwide incidence rate of pediatric ischemic stroke, identify population differences, and assess trends in incidence. Methods: We screened three databases (Medline, Embase, and Cumulative Index of Nursing and Allied Health Literature (CINAHL)) and a Google Search was performed up to October 2021. The protocol was pre-registered: PROSPERO: CRD42021273749. Data extraction and quality assessment were independently undertaken by two reviewers. A random-effects model was used for meta-analysis using Stata SE17 to calculate the overall incidence rate. Heterogeneity was assessed using I2. Meta-regression and assessment for bias were performed. Results: Out of 4166 records identified, 39 studies were included in the qualitative synthesis and the quantitative meta-analysis. The pooled incidence rate for all ischemic strokes varied from 0.9 to 7.9 per 100,000 person-years, with a pooled incidence of 2.09 (95% confidence interval (CI): 1.57–2.76). The pooled incidence was 1.28 (95% CI: 0.75–2.19) per 100,000 person-years for arterial ischemic stroke, and 0.56 (95% CI: 0.31–1.02) per 100,000 person-years for cerebral venous sinus thrombosis. The incidence of arterial ischemic stroke was high in neonates, less than 28 days old (18.51, 95% CI: 12.70–26.97). Significant heterogeneity was observed in the initial analyses of stroke incidence estimates, and geographical region, cohort age upper limit, length of study, study quality, and study design could not explain this. The incidence rate of childhood stroke appeared remained relatively stable over time. Conclusion: Our review provides estimates of global stroke incidence, including stroke subtypes, in children. It demonstrates a particularly high stroke incidence in neonates.

Rationale: Early evidence for the benefit of mechanical thrombectomy (MT) in pediatric patients with intracranial large vessel occlusion has been shown in previous retrospective cohorts. Higher-level evidence is needed to overcome the limitations of these studies such as the lack of a control group and the retrospective design. Randomized trials will very likely not be feasible, and several open questions remain, for example, the impact of arteriopathic etiologies or a possible lower age limit for MT. Save ChildS Pro therefore aims to demonstrate the safety and effectiveness of MT in pediatric patients compared to the best medical management and intravenous thrombolysis.Design: Save ChildS Pro is designed as a worldwide multicenter prospective registry comparing the safety and effectiveness of MT to the best medical care alone in the treatment of pediatric arterial ischemic stroke (AIS). It will include pediatric patients (Outcomes: The primary endpoint of Save ChildS Pro is the modified Rankin Scale score at 90 days post-stroke. Secondary endpoints will comprise the decrease of the Pediatric National Institutes of Health Stroke Scale score from admission to discharge and rate of complications.Discussion: Save ChildS Pro aims to provide high-level evidence for MT for pediatric patients with AIS, thereby improving functional outcome and quality of life and reducing the individual, societal, and economic burden of death and disability resulting from pediatric stroke.Clinical Trial Registration: Save ChildS Pro is registered at the German Clinical Trials Registry (DRKS; identifier: DRKS00018960).

Purpose of review Focal cerebral arteriopathy (FCA) is one of the most common causes of arterial ischaemic stroke in a previously healthy child. Distinguishing between different subtypes of arteriopathy is challenging and has significant management implications. Recent findings Recent studies have helped to define the subtypes of focal cerebral arteriopathies and improved understanding of their clinical and radiological features. In addition, they have reported new evidence for the association between viral infection and inflammation in the pathogenesis of FCA and proposed new radiological, serum and cerebrospinal fluid biomarkers to guide diagnosis and management. There is limited evidence to guide treatment of FCA but a role for steroids and antiviral therapies have been reported. Summary Despite the recent advances there is a limited knowledge of the pathophysiology and outcomes following FCA. Research priorities include the identification of biomarkers to improve accuracy of initial diagnosis and predict progression, and interventional trials to determine best treatments to reduce stroke recurrence risk.

We describe the risk factors for peri-procedural and spontaneous arterial ischemic stroke (AIS) in children with cardiac disease.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.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.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.

Background: This research forms the initial part of a PhD research study, based in the critical paradigm, with an emphasis on developing education strategies that impact on learning in the non-classroom setting. The focus of this article is the education required to enable clinical supervisors to undertake their role supporting nursing students in clinical practice. Aims: There are two aims of this literature review. First, to determine what peer-reviewed, published literature reveals concerning effective learning and teaching strategies for clinical supervisor education. Second, to gain an understanding from a group of clinical supervisors of their perspective on the literature review findings, and develop recommendations for their preparatory education. Methods: This research used a literature review with a participatory phase built in. A traditional review was completed and then extended to include the clinical supervisors reviewing the findings, providing their perspectives and developing recommendations for their own future education development. Findings: During phase one of the research, 22 peer-reviewed articles were included for review and four themes identified and explored. In phase two, 36 clinical supervisors provided their perspectives on these themes. They highlighted the need for a variety of modalities for education to ensure supervisors have shared values, knowledge and skills to work in clinical practice with nursing students and, more broadly, to influence the development of person-centred learning cultures. Conclusions: The literature review revealed a limited amount of peer-reviewed literature. Further, there is little published literature on person-centred situated education for clinical supervisors. When evidence is shared with clinical supervisors, they can contribute to designing their education needs. Implications for practice: • Overall, as person-centred curricula develop, there will be a need to focus on transferring the person-centred pre-registration curriculum into the clinical practice context • There is presently both a gap and an opportunity for person-centred research and development with clinical supervision

Background The association between left hemisphere stroke and acute speech and language impairment is well documented in adults. However, little is known about this association in childhood arterial ischemic stroke. Here we examined potential predictors of acute speech (dysarthria and apraxia) and language impairments after childhood arterial ischemic stroke, including site of lesion. Methods Children with radiologically confirmed acute arterial ischemic stroke, admitted to a tertiary pediatric hospital from 2004 to 2012, were identified from an institutional registry. We examined the prevalence of dysarthria, apraxia, and language impairment within two weeks of the stroke. Associations with age at stroke event, lesion side (left, right, or bilateral), and arterial territory affected (anterior, posterior, or both) were assessed using logistic regression. Results Sixty-two children with mean age eight years (range three to 17 years) were identified. Strokes were located in the left (32%), right (44%), or both hemispheres (24%). Dysarthria (74%) and language impairment (50%) were frequent. Verbal dyspraxia was less common (11%). There was little evidence that variables of interest, including site of lesion, were significantly associated with increased odds of dysarthria or language impairment (all P > 0.49). Conclusions Regardless of age, children are at high risk of communication disorders after stroke. Unlike adults, left hemisphere stroke was not associated with either speech or language impairment in our cohort, suggesting there may be bihemispheric contribution to language function. Future studies are needed to examine whether the predictors examined here determine long-term outcomes.

Pediatric stroke is unfortunately not a rare condition. It is associated with severe disability and mortality because of the complexity of potential clinical manifestations, and the resulting delay in seeking care and in diagnosis. Neuroimaging plays an important role in the multidisciplinary response for pediatric stroke patients. The rapid development of adult endovascular thrombectomy has created a new momentum in health professionals caring for pediatric stroke patients. Neuroimaging is critical to make decisions of identifying appropriate candidates for thrombectomy. This review article will review current neuroimaging techniques, imaging work-up strategies and special considerations in pediatric stroke. For resources limited areas, recommendation of substitute imaging approaches will be provided. Finally, promising new techniques and hypothesis-driven research protocols will be discussed.

Background and ObjectivesWe aimed to determine whether a modified pediatric Alberta Stroke Program Early CT Score (modASPECTS) is associated with clinical stroke severity, hemorrhagic transformation, and 12-month functional outcomes in children with acute arterial ischemic stroke (AIS).MethodsChildren (age 29 days–ResultsOne hundred thirty-one children were included; 91 were ≥2 years of age. Median time from stroke to MRI was 1 day (interquartile range [IQR] 0–1 day). Median modASPECTS was 4 (IQR 3–7). ModASPECTS correlated with PedNIHSS score (ρ = 0.40, p = 0.0001). ModASPECTS was associated with hemorrhagic transformation (odds ratio [OR] 1.13, 95% confidence interval [CI] 1.02–1.25, p = 0.018). Among children with follow-up (n = 128, median 12.2 months, IQR 9.5–15.4 months), worse outcomes were associated with higher modASPECTS (common OR 1.14, 95% CI 1.04–1.24, p = 0.005). The association between modASPECTS and outcome persisted when we adjusted for age at stroke ictus and the presence of tumor or meningitis as stroke risk factors (common OR 1.14, 95% CI 1.03–1.25, p = 0.008).DiscussionModASPECTS correlates with PedNIHSS scores, hemorrhagic transformation, and 12-month functional outcome in children with acute AIS. Future pediatric studies should evaluate its usefulness in predicting symptomatic intracranial hemorrhage and outcome after acute revascularization therapies.Classification of EvidenceThis study provides Class II evidence that the modASPECTS on MRI is associated with stroke severity (as measured by the baseline PedNIHSS score), hemorrhagic transformation, and 12-month outcome in children with acute supratentorial ischemic stroke.

This paper aims to share a program that took a whole- hospital approach in considering the wellbeing of staff at a time of recovery following the 2019– 2020 bushfires. The SEED Program enlisted a person-centred participatory methodology that was embedded within a transformational learning approach. This methodology included collaboration, authentic participation, critical reflection, critical dialogue and listening where the staff voice was the driving factor in the development of strategies for recovery. The SEED Program resulted in the development of five initi ati ves that included four strategies and a celebration event where staff celebrated their New Year’s Eve in February 2020. The four strategies included the establishment of a quiet room, coffee buddies, Wellness Warriors and 24/7 Wellness. The outcomes from the SEED Program resulted in the development of a more person-centred culture and transformation of staff perspectives in how they understood their role in their learning and learning of others in recovery and support at a time of crisis. The key learnings were the effect of authentic collaboration, the benefit from enabling authentic leadership at all levels within a hospital, and the power of a staff connection to the ‘ CORE’ values of the hospital and Local Health District. In conclusion, the staff involved hold the hope that others may benefit from their experience of transformational learning in creating more person-centred workplace cultures while supporting each other to move forward during a crisis. The limitation of the SEED Program was that it was a bespoke practice innovation designed in the moment, responding to an identified need for the staff following a crisis in the local community rather than a formal research approach to meeting the needs of this group of staff.

The ‘Cyber Security Governance in the Indo-Pacific: Policy Futures in Australia, Indonesia and the Pacific’ Policy Engagement Program (PEP) highlights the strengths and weaknesses in regional cyber security governance. Importantly, it identifies key policy priorities from an Australian, Indonesian and Pacific Island Countries’ (PIC) perspective and, through its recommendations, identifies new paths for cooperative engagement between Australia and its regional partners.Notwithstanding the fact that Indonesia and the PICs have unique socio-political and economic contexts, the PEP online Roundtable Discussion, which formed the foundation of this think piece, highlights many areas of commonality around threats and challenges in the cyber domain. Through an online exchange of ideas between experts from The University of Queensland (UQ), the Department of Home Affairs, Indonesia’s National Cyber and Crypto Agency (BSSN), the Democracy and Integrity for Peace (DIP) Institute, Indonesia and the Indonesian Defence University (Unhan), one can see there are models and initiatives that can be replicated by countries pursuing their own paths to cyber security resilience in the face of rapid technological change.In practical terms, the PEP Roundtable Discussion highlights how Australia has defined its own regulatory and policy approach to defend governments, businesses, and communities from malicious cyber activity. Such an approach has lessons for countries like Indonesia, which is contending with legislative and policy gaps, weak wholeof-government coordination, and a relatively low knowledge base among legislators and policy makers. This is a problem not unique to Indonesia and, indeed, represents a challenge common also to many of the PICs. Ideas and expertise flow both ways, however, and our Indonesian experts have made innovative policy recommendations and identified areas for further engagement.Based on the contributions of our panellists and contributors, there is clearly scope to further tailor regional capacity building programs through increased coordination, knowledge exchange and workshopping of national cyber security legislation and strategic guidance. Inherent in Australia’s Cyber Security Strategy 2020 also are solutions to Indonesia and the PICs outreach and engagement objectives, particularly in the expansion of Joint Cyber Security Centres (JCSCs), which link federal government agencies to their subnational counterparts and also to Micro, Small and Medium Enterprises (MSMEs). Finally, production of popular cyber security media programs, which could be subtitled and streamed across the region, hold promise for raising vital public awareness. Moreover, the think piece highlights the importance of universities as a source of innovation, interdisciplinary research and as a testbed for cyber security competitions, exercises, and ideas. We commend the recommendations to you.

Background: In adults, time since stroke onset correlates with efficacy and risk of recanalization therapies; beyond this temporal window there is increased risk of adverse events in particular hemorrhage with thrombolysis and decreased benefit of recanalization due to irreversible tissue injury. In adults the appearance of fluid attenuated inversion recovery (FLAIR) signal is a proxy for time, and is typically present by 6 hours. The time to FLAIR signal hyperintensity in childhood stroke is unknown but is of interest with potential utility due to the often greater uncertainty as to timing symptom onset. Methods: Time to FLAIR signal hyperintensity on brain MRI performed on children within 24 hours of stroke onset was studied with logistic regression. Results: A total of 68 MRIs with FLAIR imaging were available from 54 children (27 female), age 0.8 to 17.9 years, median 12.0 years. Seventy-four percent (40/54) of children and 72% (49/68) of scans had anterior circulation stroke. Interquartile range for time to FLAIR presence was 7.8 to 19.1 hours. The 90% probability of FLAIR change was reached at 11.2 hours for all strokes (Figure, dotted line; 80% highest density interval (HDI): 1.2-11.2 hours), and 9.3 hours for anterior circulation only strokes (Figure, solid line; 80% HDI: 3.3-9.3 hours), though nearly all had FLAIR change by 6 hours. FLAIR change was absent in 4 children after 6 hours, two with anterior circulation stroke (16 year-old at 6.1 hours, 10 year-old at 7.0 hours) and 2 with posterior circulation stroke (15 year-old at 7.3 hours, 9 month-old at 18.2 hours). Conclusion: Similar to adults, FLAIR hyperintensity can be used to estimate time since stroke ictus in childhood stroke. Children may have somewhat delayed time to FLAIR signal change compared with adults, suggesting that they may have a longer window for effective recanalization therapies.

This study aimed to investigate the prevalence of fractures and non-fracture injuries, including associated risk factors, in children with epilepsy prescribed antiseizure medications (ASM). A controlled, cross-sectional study was conducted in a hospital outpatient setting, comparing children with epilepsy prescribed ASMs with their non-epileptic siblings. Information was collected by questionnaire included history of fractures, non-fracture injuries and epilepsy, comorbidities and ASM use. 261 participants completed the questionnaire, 133 children with epilepsy (aged 10.7 ± 3.5 years, mean ± SD) and 128 siblings (10.1 ± 3.7 years). There were 49 non-seizure-related fractures in 34 ASM patients while prescribed ASMs, compared with 21 lifetime fractures in 15 controls, giving a 2.7 (95% CI 1.3-5.3, p = 0.007) times greater fracture prevalence in children treated with ASMs compared to healthy siblings. The rates of non-fracture injuries were similar across groups, except that concussion was more common in children taking ASMs (9.0% vs 1.6%, p = 0.026). Duration of ASM use and generalized tonic-clonic seizures (GTCS) were independent predictors of fractures (OR 1.55; 95% CI 1.03-2.31, p = 0.03; OR 2.50; 95% CI 1.05-5.94, p = 0.04, respectively). Fewer than 20% of participants and/or their families were aware that ASM use was related to bone health. Children with epilepsy treated with ASMs had a higher fracture prevalence than their sibling controls. Duration of ASM treatment and GTCS were associated with fracture risk. Longitudinal prospective studies are required to further explore risk and the direct impact of epilepsy on bone health.

In this chapter, we consider the scope of published research on person-centredness in nursing education and acknowledge the Position Statement on Person-centredness in Health and Social Care curricula [1]. In addition, exemplars of emerging research on person-centred nursing education will be explored. Each of the authors will share a vignette to illustrate how their doctoral research is contributing to the further development of knowledge in this field. The vignettes reflect the progress of each of the authors in their doctoral research journey. It is anticipated that readers who are at similar points in their studies or who wish to consider doctoral research will connect with the contemporary approaches to person-centred nursing education research shared.

Background: Neurocognitive outcomes beyond childhood in people with a Fontan circulation are not well defined. This study aimed to investigate neurocognitive functioning in adolescents and adults with a Fontan circulation and associations with structural brain injury, brain volumetry, and postnatal clinical factors. Methods: In a binational study, participants with a Fontan circulation without a preexisting major neurological disability were prospectively recruited from the Australia and New Zealand Fontan Registry. Neurocognitive function was assessed by using Cogstate software in 107 participants with a Fontan circulation and compared with control groups with transposition of the great arteries (n=50) and a normal circulation (n=41). Brain MRI with volumetric analysis was performed in the participants with a Fontan circulation and compared with healthy control data from the ABIDE I and II (Autism Brain Imaging Data Exchange) and PING (Pediatric Imaging, Neurocognition, and Genetics) data repositories. Clinical data were retrospectively collected. Results: Of the participants with a Fontan circulation who had a neurocognitive assessment, 55% were male and the mean age was 22.6 years (SD 7.8). Participants with a Fontan circulation performed worse in several areas of neurocognitive function compared with those with transposition of the great arteries and healthy controls ( P P P =0.01 and working memory, P =0.02). Structural brain injury was present in the entire Fontan cohort; the presence of white matter injury was associated with worse paired associate learning ( P P P P P ≤0.04). Conclusions: Neurocognitive impairment is common in adolescents and adults with a Fontan circulation and is associated with smaller gray and white matter brain volume. Understanding modifiable factors that contribute to brain injury to optimize neurocognitive function is paramount.

OBJECTIVE: To study the epilepsy syndromes among the severe epilepsies of infancy and assess their incidence, etiologies, and outcomes. METHODS: A population-based cohort study was undertaken of severe epilepsies with onset before age 18 months in Victoria, Australia. Two epileptologists reviewed clinical features, seizure videos, and electroencephalograms to diagnose International League Against Epilepsy epilepsy syndromes. Incidence, etiologies, and outcomes at age 2 years were determined. RESULTS: Seventy-three of 114 (64%) infants fulfilled diagnostic criteria for epilepsy syndromes at presentation, and 16 (14%) had "variants" of epilepsy syndromes in which there was one missing or different feature, or where all classical features had not yet emerged. West syndrome (WS) and "WS-like" epilepsy (infantile spasms without hypsarrhythmia or modified hypsarrhythmia) were the most common syndromes, with a combined incidence of 32.7/100 000 live births/year. The incidence of epilepsy of infancy with migrating focal seizures (EIMFS) was 4.5/100 000 and of early infantile epileptic encephalopathy (EIEE) was 3.6/100 000. Structural etiologies were common in "WS-like" epilepsy (100%), unifocal epilepsy (83%), and WS (39%), whereas single gene disorders predominated in EIMFS, EIEE, and Dravet syndrome. Eighteen (16%) infants died before age 2 years. Development was delayed or borderline in 85 of 96 (89%) survivors, being severe-profound in 40 of 96 (42%). All infants with EIEE or EIMFS had severe-profound delay or were deceased, but only 19 of 64 (30%) infants with WS, "WS-like," or "unifocal epilepsy" had severe-profound delay, and only two of 64 (3%) were deceased. SIGNIFICANCE: Three quarters of severe epilepsies of infancy could be assigned an epilepsy syndrome or "variant syndrome" at presentation. In this era of genomic testing and advanced brain imaging, diagnosing epilepsy syndromes at presentation remains clinically useful for guiding etiologic investigation, initial treatment, and prognostication.

Childhood stroke results in long-term, multifaceted difficulties, affecting motor, cognitive, communication, and behavioral domains of function which impact on participation and quality of life. The Childhood Stroke Consensus Rehabilitation Guideline was developed to improve the care of children with stroke by providing health professionals with recommendations to assist in their rehabilitative treatment. Clinical questions were formulated to inform systematic database searches from 2001 to 2016, limited to English and pediatric studies. SIGN methodology and the National Health and Medical Research Council system were used to screen and classify the evidence. The Grade of Recommendation, Assessment, Development and Evaluation system was used to grade evidence as strong or weak. Where evidence was inadequate or absent, a modified Delphi consensus process was used to develop consensus-based recommendations. The guideline provides 56 recommendations (1 evidence-based recommendation and 55 consensus recommendations). These relate to the framework of rehabilitation service delivery as well as domain-specific rehabilitation treatment strategies for each domain of function. It is anticipated that this guideline will provide health professions with recommendations to improve the subacute care of children with stroke both in Australia and internationally.

Background and Purpose— Focal cerebral arteriopathy (FCA)—a common cause of arterial ischemic stroke in previously healthy children—often progresses over days to weeks, increasing the risk of recurrent stroke. We developed a novel severity scoring system designed to quantify FCA progression and correlate with clinical outcomes. Methods— The VIPS study (Vascular Effects of Infection in Pediatric Stroke) prospectively enrolled 355 children with arterial ischemic stroke (2010–2014), including 41 with centrally confirmed FCA. Two neuroradiologists independently reviewed FCA cerebrovascular imaging, assigning a graded severity score of zero (no involvement) to 4 (occlusion) to individual arterial segments. The FCA severity score (FCASS) was the unweighted sum. In an iterative process, we modeled scores derived from different combinations of arterial segments to identify the model that optimized correlation with clinical outcome, simplicity, and reliability. Results— The optimal FCASS summed scores from 5 arterial segments: supraclinoid internal carotid artery, A1, A2, M1, and M2. The median (interquartile range) baseline FCASS was 4 (2–6). Of 33 children with follow-up imaging, the maximum FCASS (at any time point) was 7 (5–9). Twenty-four (73%) had FCA progression on follow-up with their maximum FCASS at a median of 8 (5–35.5) days poststroke; their median FCASS increase was 4 (2.5–6). FCASS did not correlate with recurrent arterial ischemic stroke. Maximum (but not baseline) FCASS correlated with 1-year pediatric stroke outcome measures ( P =0.037). Conclusions— Our novel scoring system for FCA severity correlates with neurological outcomes in the VIPS cohort and provides a tool for FCA treatment trials under development.

Stroke is among the top 10 causes of death in children and survivors carry resulting disabilities for decades, at substantial cost to themselves and their families. Children are not currently able to access reperfusion therapies, due to limited evidence supporting safety and efficacy and long diagnostic delays. The Australian Clinical Consensus Guideline for the Diagnosis and Acute Management of Childhood Stroke was developed to minimize unwarranted variations in care and document best evidence on the risk factors, etiologies, and conditions mimicking stroke that differ from adults. Clinical questions were formulated to inform systematic database searches from 2007 to 2017, limited to English and pediatric studies. SIGN methodology and the National Health and Medical Research Council system were used to screen and classify the evidence. The Grades of Recommendation, Assessment, Development, and Evaluation system (GRADE) was used to grade evidence as strong or weak. The Guideline provides more than 60 evidence-based recommendations to assist prehospital and acute care clinicians in the rapid identification of childhood stroke, choice of initial investigation, to confirm diagnosis, determine etiology, selection of the most appropriate interventions to salvage brain at risk, and prevent recurrence. Recommendations include advice regarding the management of intracranial pressure and congenital heart disease. Implementation of the Guideline will require reorganization of prehospital and emergency care systems, including the development of regional stroke networks, pediatric Code Stroke, rapid magnetic resonance imaging and accreditation of primary pediatric stroke centers with the capacity to offer reperfusion therapies. The Guideline will allow auditing to benchmark timelines of care, access to acute interventions, and outcomes. It will also facilitate the development of an Australian childhood stroke registry, with data linkage to international registries, to allow for accurate data collection on stroke incidence, treatment, and outcomes.

Aim To determine the role of inflammation in pediatric transient focal cerebral arteriopathy using cerebrospinal fluid cytokine/chemokines as biomarkers. Methods We measured 32 cytokine/chemokines in acute cerebrospinal fluid collected from children with stroke due to focal cerebral arteriopathy (n = 5) using multiplex immunoassay and compared with two patients with arterial ischemic stroke due to other causes (non-focal cerebral arteriopathy group, vertebral dissection, n = 1; cryptogenic, n = 1), pediatric encephalitis (n = 43), and non-inflammatory neurological disease controls (n = 20). Results Median age in the focal cerebral arteriopathy group was 9.3 years (range, 2.8–13 years). In the focal cerebral arteriopathy group (n = 5), four patients had middle cerebral ± distal carotid arteriopathy; one patient had posterior circulation arteriopathy. The median time from symptom onset to cerebrospinal fluid sampling was four days (range, 0.6–7 days). Only IL-6, IL-8, CXCL1, and CXCL10 levels were significantly higher in the acute cerebrospinal fluid of focal cerebral arteriopathy patients compared to non-inflammatory neurological disease controls and non-focal cerebral arteriopathy stroke. In contrast to focal cerebral arteriopathy, a broad array of Th1, Th2, Treg, Th17, B-cell related, and other broad spectrum cytokine/chemokines were elevated in encephalitis. Conclusion The elevated cerebrospinal fluid cytokine/chemokines support innate, T cell, and granulocyte inflammatory mechanisms in children with focal cerebral arteriopathy. This warrants larger cohort studies to discriminate primary inflammatory signals of the arteriopathy from secondary inflammation due to the stroke itself.

This review will discuss important developments in childhood arterial ischemic stroke over the past decade, focusing on improved understanding of the causes, consequences, and targets for intervention. Risk factors for childhood arterial ischemic stroke are different to adults. Infections, particularly herpes group viruses, are important precipitants for stroke. Non-atherosclerotic arteriopathies are the most common cause of childhood arterial ischemic stroke and an important predictor of recurrent events. Recent advances include the identification of serum biomarkers for inflammation and endothelial injury, and imaging biomarkers to monitor for vascular progression. Multicenter trials of immunotherapies in focal cerebral arteriopathies are currently in development. Recognition of clinical and radiological phenotypic patterns has facilitated the discovery of multisystem disorders associated with arterial ischemic stroke including ACTA2 arteriopathy and adenosine deaminase 2 deficiency. Identification of these Mendelian disorders provide insights into genetic mechanisms of disease and have implications for medical and surgical management. In contrast to adults, there are long diagnostic delays in childhood arterial ischemic stroke. Refinement of pediatric Code Stroke protocols and clinical decision support tools are essential to improve diagnostic certainty and improve access to reperfusion therapies. Children do not recover better than adults following arterial ischemic stroke, with more than half of survivors having long-term impairments. The physical, cognitive, and behavioral consequences of childhood arterial ischemic stroke are increasingly reported but further research is required to understand their impact on participation, quality of life, psychosocial, and family functioning. Longitudinal studies and the use of advanced imaging techniques, to understand neurobiological correlates of functional reorganization, are essential to developing targeted intervention strategies to facilitate recovery.

The aim was to describe clinical presentation, management, and outcomes of stroke in a tertiary emergency department (ED) of a developing country. Retrospective case series of patients aged 1 month to 18 years presenting to an ED with radiological confirmed acute stroke during a 7-year period were studied. Ninety-five patients were identified. Twenty-five patients were excluded because of incomplete records (8) or not presenting via ED (17). Thirty-four (48.5%) were diagnosed with hemorrhagic stroke (HS), 30 (42.8%) with arterial ischemic stroke (AIS), and 6 (8.5%) with sinus venous thrombosis (SVT). Mean age was 5.3 years, and 55.3% were male. The median time from onset of symptoms to ED presentation was 24 hours (mean, 55 hours; interquartile range [IQR], 14–72) for AIS, 24 hours (mean, 46.9 hours; IQR, 9–48) for HS, and 120 hours (mean,112 hours; IQR, 72–168) for SVT. Congenital cardiac disease was the most common risk factor (9%). For AIS, the most common symptoms were focal numbness 56.6% (95% confidence interval [CI], 37.8%–75.4%), focal weakness 56.6% (95% CI, 37.8%–75.4%), and seizures 50% (95% CI, 31%–68.8%). For HS, the most common symptoms were headache 64.7% (95% CI, 47.7%–81.6%), vomiting 79.4 (95% CI, 65–93.7), and altered mental status 64.7% (95% CI, 47.7–81.6). Computed tomography scan was done in 100% of the patients and magnetic resonance imaging in 54%. Twenty-five (36%) patients were admitted to intensive care unit and required intubation. Long-term deficit was identified in 24 (36%) patients based on medium-term follow-up. The spectrum of stroke in a developing country was similar to published series from developed countries in terms of final diagnosis, risk factors, and delay to ED presentation, neuroimaging, and long-term neurodeficits. No tropical diseases were identified as risk factors.

Background There is a lack of consensus regarding how best to screen children with facial port-wine stains for Sturge-Weber syndrome. Many favor brain magnetic resonance imaging, and adjunctive electroencephalography is increasingly used. However, the sensitivity, specificity, and negative and positive predictive value of magnetic resonance imaging and electroencephalography and whether screening improves seizure recognition is unclear. Methods A retrospective review of children with high-risk port-wine stains presenting consecutively to the outpatient laser clinic of a tertiary pediatric hospital between December 2015 and November 2016 was undertaken. Primary outcome measures were yield, accuracy, age of and protocols for screening magnetic resonance imaging and electroencephalography, type of and age at presenting seizure, and percentage referred to neurology. Results Of 126 patients with facial port-wine stains, 25.4% (32/126) were at high risk of Sturge-Weber syndrome (hemifacial, median, and forehead PWS phenotypes); 43.7% of these (14/32) underwent screening magnetic resonance imaging. Sturge-Weber syndrome was detected in 7.1% (1/14). Magnetic resonance imaging had false-negative results in 23.1% (3/13) of those screened. Screening magnetic resonance imaging had sensitivity of 25%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 76.9% for the detection of Sturge-Weber syndrome (hemifacial, median and forehead PWS phenotypes). Only one-third of those with false-negative magnetic resonance imaging were referred to neurology. Mean age of first seizure in those with false-negative screening magnetic resonance imaging was 28 months, vs 14 months in those not screened. Abnormal electroencephalographic signs were detected in the two infants who underwent presymptomatic electroencephalography. Conclusions Findings from this small cohort of individuals with port-wine stains that put them at high risk of Sturge-Weber syndrome suggest that children with positive screening magnetic resonance imaging will almost certainly develop Sturge-Weber syndrome but that negative screening magnetic resonance imaging cannot exclude Sturge-Weber syndrome (in up to 23.1% of cases). False-negative magnetic resonance imaging may delay seizure recognition. Seizure education, monitoring, and consideration of adjunctive electroencephalography are important irrespective of magnetic resonance imaging findings.

We describe an infant with an early-onset demyelinating neuropathy who presented with an upper extremity monoplegia and progressive asymmetric weakness. Neurophysiologic testing revealed a generalized severe neuropathy with marked slowing of nerve conduction. The disproportionate severity and asymmetry of upper extremity involvement at presentation was atypical of inherited neuropathies, and an initial diagnosis of chronic inflammatory demyelinating polyneuropathy was considered. Nerve biopsy showed severe depletion of large myelinated fibers without inflammatory cells, and focally folded myelin sheaths were seen on electron microscopy. Genetic testing revealed a de novo heterozygous mutation in the myelin protein zero gene.

In this paper, the authors will describe the journey of registered nurses across a series of workshops as part of a research project that was undertaken in a regional aged care service in New South Wales, Australia. The aim of the project was to empower the participant registered nurses to positively influence the health care workplace culture within the residential care home by raising consciousness about their own practice. Registered nurses were actively involved in this reconnaissance phase of a participatory action research project through practice development principles and methods. Registered nurses determined the content and the outcomes of the overall program. The researchers evaluated the impact of a series of workshops, designed to develop skills and knowledge using nominal group technique. Results revealed registered nurses perceived they were empowered to flourish, and developed an understanding of the uniqueness of their role. A shared understanding of the role of the registered nurse in the aged care setting was fundamental in enabling them to feel empowered to lead their team and contribute positively to the workplace culture. Overall, the outcomes of this project have positively impacted workplace culture.