Search

Seizures occur in approximately one-third of children with cerebral palsy. This study aimed to determine epilepsy syndromes in children with seizures and cerebral palsy due to vascular injury, anticipating that this would inform treatment and prognosis. We studied a population-based cohort of children with cerebral palsy due to prenatal or perinatal vascular injuries, born 1999–2006. Each child's MRI was reviewed to characterize patterns of grey and white matter injury. Children with syndromic or likely genetic causes of cerebral palsy were excluded, given their inherent association with epilepsy and our aim to study a homogeneous cohort of classical cerebral palsy. Chart review, parent interview and EEGs were used to determine epilepsy syndromes and seizure outcomes. Of 256 children, 93 (36%) had one or more febrile or afebrile seizures beyond the neonatal period and 87 (34%) had epilepsy. Children with seizures were more likely to have had neonatal seizures, have spastic quadriplegic cerebral palsy and function within Gross Motor Function Classification System level IV or V. Fifty-six (60%) children with seizures had electroclinical features of a self-limited focal epilepsy of childhood; we diagnosed these children with a self-limited focal epilepsy-variant given the current International League Against Epilepsy classification precludes a diagnosis of self-limited focal epilepsy in children with a brain lesion. Other epilepsy syndromes were focal epilepsy—not otherwise specified in 28, infantile spasms syndrome in 11, Lennox–Gastaut syndrome in three, genetic generalized epilepsies in two and febrile seizures in nine. No epilepsy syndrome could be assigned in seven children with no EEG. Twenty-one changed syndrome classification during childhood. Self-limited focal epilepsy-variant usually manifested with a mix of autonomic and brachio-facial motor features, and occipital and/or centro-temporal spikes on EEG. Of those with self-limited focal epilepsy-variant, 42/56 (75%) had not had a seizure for >2 years. Favourable seizure outcomes were also seen in some children with infantile spasms syndrome and focal epilepsy-not otherwise specified. Of the 93 children with seizures, at last follow-up (mean age 15 years), 61/91 (67%) had not had a seizure in >2 years. Children with cerebral palsy and seizures can be assigned specific epilepsy syndrome diagnoses typically reserved for normally developing children, those syndromes commonly being age-dependent and self-limited. Compared to typically developing children with epilepsy, self-limited focal epilepsy-variant occurs much more commonly in children with cerebral palsy and epilepsy. These findings have important implications for treatment and prognosis of epilepsy in cerebral palsy, and research into pathogenesis of self-limited focal epilepsy. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: This study assessed the impact of the COVID-19 pandemic on emergency departments (EDs) in South Australia, measured by changes in the number and casemix of patients in the system over time. Methods: Data from the South Australia Emergency Department Dashboard, updated every 30 min, were analysed for the period 4 October–21 December 2020. The Dashboard reports live counts of the number and type of patients in each of the six adult metropolitan public EDs in Adelaide, South Australia. Results: There was a significant difference in the mean daily average occupied ED capacity before and during two distinct increases in COVID-19 cases in South Australia. An increase in COVID-19 cases coincided with a decrease in patients in EDs (Pearson's r = –0.93 and –0.67; P < 0.001 for both). Presentations in Australasian Triage Scale (ATS) Categories 2–5 decreased during these periods, whereas ATS Category 1 stayed constant. Mental health patients continued to present to the ED, despite the overall drop in ED presentations. Conclusions: During the two periods of COVID-19 case growth in South Australia, there was a significant drop in the number of patients presenting to the major public EDs and a change in the casemix of patients over time. What is known about the topic?: EDs in Australia often operate at or over capacity, with frequent reports of ambulance ramping, access block and long waiting times. There have been reports internationally of significant declines in ED presentations throughout the COVID-19 pandemic. What does this paper add?: This paper uses a novel publicly available data source that is available in real time to contribute a new perspective from South Australia, which has experienced two distinct periods of strict restrictions and lockdown. The research showed that the number of mental health presentations remained consistently high, despite a significant overall decline in ED occupancy. What are the implications for practitioners?: This study demonstrates that South Australians are accessing emergency medical treatment differently in response to the COVID-19 pandemic. In the context of an overall decline in presentation numbers, the number of mental health-related presentations has not changed significantly, suggesting that this trend should be closely monitored. The findings corroborate the national concern that unwell people have avoided accessing emergency medical care during the pandemic, leading to worse outcomes and increased need for healthcare resources at a later date. It will be important to monitor and quickly detect further changes in ED usage using real-time data as the pandemic evolves, as well as in any future significant health crises. [ABSTRACT FROM AUTHOR]

To study seizure manifestations and outcomes in children with cortical versus white matter injury, differences potentially explaining variability of epilepsy in children with cerebral palsy. In this population-based retrospective cohort study, MRIs of children with cerebral palsy due to ischemia or haemorrhage were classified according to presence or absence of cortical injury. MRI findings were then correlated with history of neonatal seizures, seizures during childhood, epilepsy syndromes, and seizure outcomes. Of 256 children studied, neonatal seizures occurred in 57 and seizures during childhood occurred in 93. Children with neonatal seizures were more likely to develop seizures during childhood, mostly those with cortical injury. Cortical injury was more strongly associated with (1) developing seizures during childhood, (2) more severe epilepsy syndromes (infantile spasms syndrome, focal epilepsy, Lennox-Gastaut syndrome), and (3) less likelihood of reaching > 2 years without seizures at last follow-up, compared to children without cortical injury. Children without cortical injury, mainly those with white matter injury, were less likely to develop neonatal seizures and seizures during childhood, and when they did, epilepsy syndromes were more commonly febrile seizures and self-limited focal epilepsies of childhood, with most achieving > 2 years without seizures at last follow-up. The presence of cortical injury also influenced seizure occurrence, severity, and outcome within the different predominant injury patterns of the MRI Classification System in cerebral palsy, most notably white matter injury. Epileptogenesis is understood with cortical injury but not well with white matter injury, the latter potentially related to altered postnatal white matter development or myelination leading to apoptosis, abnormal synaptogenesis or altered thalamic connectivity of cortical neurons. These findings, and the potential mechanisms discussed, likely explain the variability of epilepsy in children with cerebral palsy and epilepsy following early-life brain injury in general. • Acute symptomatic neonatal seizures, self-limited seizures and drug-resistant epilepsies occur in cerebral palsy. • Cortical injury is associated with neonatal and childhood seizures, severe epilepsy syndromes, and poorer seizure outcomes. • White matter injury is associated with self-limited epilepsies with favourable outcome. • In children, different mechanisms of epileptogenesis likely follow cortical and white matter injuries. [ABSTRACT FROM AUTHOR]

Abstract Background To minimise the risk of patient harm from results, laboratories should establish QC strategies and monitor the performance of assays in line with the analytical and clinical risk. Methods Steady state errors were calculated from a distribution normalized for an Analytical Performance Specification expressed as Assay Capability (imprecision) minus Assay Stability (drift). Inverting this error rate gave QC run length containing one error. Multiplying by error detection of a critical shift gave a QC functional run length for stable and unstable situations. Suitability of this technique was examined using laboratory EQA imprecision and drift data against various analytical and clinical performance specifications. Results Steady state errors and error detection, and hence QC functional run length, were dramatically affected by worsening imprecision, drift or changing performance specifications. For a single analyser type, laboratory steady state errors against RCPAQAP performance specification ranged over five orders of magnitude, with contributions from Assay Capability and Assay Stability varying by laboratory. Conclusions Steady state errors accumulate for all assays. Our functional QC run length based on steady state error rate adjusted for error detection of the QC algorithm, amounts to a risk approach using the first two elements of FMEA-like calculation and allows laboratories to examine the suitability of their combinations of QC run length, algorithm, workload and timing of QC challenges. An appropriate common performance specification is critical when assessing and comparing risk. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aim: To estimate the strengths of association between clinical features and migraine or arterial ischaemic stroke (AIS) in children presenting to the emergency department.Method: Eighty-four children with migraine, prospectively recruited from 2009 to 2010, were compared with 55 children with AIS, prospectively/retrospectively recruited from 2003 to 2010. Odds ratios were calculated via logistic regression to measure associations between clinical features and process-of-care factors, and migraine and AIS.Results: Median age was 13 years 5 months (interquartile range 12y 11mo-13y 10mo) for migraine and 5 years (interquartile range 3y 7mo-8y) for patients with AIS. All cases of AIS and 30% of migraine cases underwent neuroimaging. Over 40% of children with migraine had vomiting, numbness, or visual disturbance; other symptoms were uncommon. Fifty-five per cent had no signs on physician assessment. Weakness or speech disturbance were common in patients with AIS. Significant clinical features associated with increased odds of AIS included sudden symptom onset, weakness, seizures, speech disturbance, and ataxia, and signs of face, arm, or leg weakness, inability to walk, dysarthria, dysphasia, and altered consciousness (p<0.05). Significant features associated with decreased odds of AIS included older age, vomiting, visual, sensory, other symptoms, and absent focal signs on assessment (p<0.05).Interpretation: Presenting features can discriminate childhood AIS from migraine. These differences inform decisions about urgency and type of neuroimaging in children presenting to the emergency department with brain attack symptoms.What the Paper Adds: Weakness, seizures, ataxia, speech, or walking difficulties are more frequent in arterial ischaemic stroke (AIS). Vomiting, visual, or sensory disturbance and absent focal signs are more frequent in migraine. Identifying features of AIS and migraine guides neuroimaging in children with brain attack symptoms. [ABSTRACT FROM AUTHOR]

Abstract: 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. [ABSTRACT FROM AUTHOR]

Gaining a better understanding of Quality Control (QC) processes is a key requirement to improving performance and reducing patient risk. Detecting analytical error is dependent on a QC strategy that reliably detects a critical shift in a result away from the true value. Recently the concept of Six Sigma has been used by diagnostic laboratories to assess the performance of assays and to assist in the selection of QC rules. The sigma metric is one measure of an assay's ability to perform within specification. However an additional dimension to managing an assay is its stability in bias over time. The concept of long term stability is the same as measured QC drift (SE drift ) which is the effect of numerous calibrations, changes in reagent lots and other conditions i.e. a long term effect. This implies that the standard error budget is wrong because it is modelled on short term QC and misses this SE drift stability component. We show that SE drift provides a measure of Assay Stability that should be included in Quality Planning and that by including an allowance for this drift, determining target imprecision appropriate for matched QC algorithms that provide high error detection is as simple as dividing the Allowable Performance Specification by 4, 5 or 6. [ABSTRACT FROM AUTHOR]

OBJECTIVE: The goal of this study was to describe the prevalence, syndromes, and evolution of seizure disorders in children with cerebral palsy (CP) due to white matter injury (WMI). METHODS: For this population-based cohort study, brain MRI scans and medical records were reviewed in children in the Victorian Cerebral Palsy Register born between 1999 and 2006 recorded as having WMI. Children were excluded if they had features of an undiagnosed syndrome, associated cortical malformation or injury, or no medical contact in the preceding year. Included were 166 children with CP and isolated WMI due to presumed vascular insufficiency or hemorrhage; 87 were born preterm. Seizure and CP details were obtained from medical records and interviews, and EEG recordings were reviewed. RESULTS: Forty-one children (25%) had seizures beyond the neonatal period. Four children had West syndrome, which resolved with treatment. Thirteen children had febrile seizures that they outgrew. Thirty children had focal epilepsy with seizure manifestations and EEG discharges typical of early-onset childhood occipital epilepsy or childhood epilepsy with centrotemporal spikes; 23 have outgrown these seizures. Two children had idiopathic generalized epilepsy; it was ongoing in 1 child. Fourteen children had evolution from 1 epileptic syndrome to another. At last follow-up (median age, 12.7 years; minimum age, 9.7 years), 80% had not had a seizure for >2 years. CONCLUSIONS: The electroclinical features of seizure disorders associated with CP and WMI are those of the age-limited, epileptic syndromes of childhood, with favorable outcome in the majority. The findings have important implications for counseling and drug treatment. [ABSTRACT FROM AUTHOR]

Aim: To determine epilepsy risk factors after pediatric stroke.Method: A cohort of children with arterial ischemic stroke (birth-18y) was enrolled at 21 centers and followed for 1 year. Acute seizures (≤7d after stroke) and active epilepsy (at least one unprovoked remote seizure plus maintenance anticonvulsant at 1y) were identified. Predictors were determined using logistic regression.Results: Among 114 patients (28 neonates and 86 children) enrolled, 26 neonates (93%) and 32 children (37%) had an acute seizure. Acute seizures lasted longer than 5 minutes in 23 patients (40%) and were frequently recurrent: 33 (57%) had 2 to 10 seizures and 11 (19%) had more than 10. Among 109 patients with 1-year follow-up, 11 (10%) had active epilepsy. For each year younger, active epilepsy was 20% more likely (odds ratio [OR] 0.8, 95% confidence interval [CI] 0.6-0.99, p=0.041). Prolonged or recurrent acute seizures also increased epilepsy risk. Each additional 10 minutes of the longest acute seizure increased epilepsy risk fivefold (OR 4.7, 95% CI 1.7-13). Patients with more than 10 acute seizures had a 30-fold increased epilepsy risk (OR 30, 95% CI 2.9-305).Interpretation: Pediatric stroke survivors, especially younger children, have a high risk of epilepsy 1 year after stroke. Prolonged or recurrent acute seizures increase epilepsy risk in a dose-dependent manner. [ABSTRACT FROM AUTHOR]

Objective To document electroencephalogram ( EEG) changes and their correlation with clinical parameters in a newly diagnosed pediatric cohort of type 1 diabetes mellitus ( T1DM) patients with and without diabetic ketoacidosis ( DKA) and to define their medium term utility and significance. Research design and methods Prospective longitudinal study of children presenting with T1DM. EEGs were performed within 24 h of diagnosis, day 5, and at 6 months post-diagnosis and reviewed by a neurologist blinded to clinical status. Severity of encephalopathy was graded from 1 to 5 using the Aoki and Lombroso encephalopathy scale. Cognitive abilities were assessed using standardized tests of attention, memory, and intelligence. Results Eighty eight children were recruited; 34 presented with DKA. Abnormal background slowing was more often observed in the first 24 h in children with DKA (p = 0.01). Encephalopathy scores on day 1 correlated with initial pH, CO2, HCO3, base excess, respiratory rate, heart rate, diastolic blood pressure, and IV fluid intake (all parameters p < 0.05). EEG scores at day 1 did not correlate with contemporaneous mental state or cognition in the medium term. Conclusions DKA was associated with significant clinical and neurophysiologic signs of brain dysfunction at presentation. While EEG is sensitive to the detection of encephalopathy in newly diagnosed T1DM, it has limited use in identifying children at risk of later cognitive deficits. [ABSTRACT FROM AUTHOR]

Objectives We propose a simple way to reliably rank assays for improvement according to patient risk, based solely on EQA imprecision and biological variation data. Because the underlying technique aligns the imprecision class of an assay from EQA data, peer performance can be used to assess achievable imprecision and the risk ranking can not only prioritise improvement but also highlight laboratory QC operating parameters that are easy to manage and provide reliable, acceptable performance. Design and methods A modified Failure Modes Effects Analysis (FMEA) is applied to produce an analyte risk rating based on three factors, each of which is graded: 1) the ease of detecting analytical errors based on the ratio of allowable limits of performance to imprecision (Assay Capability) compared to absolute standards and to peers, 2) the predicted frequency of errors in patient monitoring based on the ratio of within-individual biological variation to laboratory imprecision, and 3) the clinical importance of the assay as a surrogate marker for harm arising from an error. Results We provide laboratory examples to illustrate these models. Conclusion The proposed models using only EQA data can objectively identify assays at risk of failing against biological variation goals for monitoring patients and suggest parameters for reliable performance. [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]

Clinical laboratories may systematically apply factors to assay results after analysis, but before reporting, in order to facilitate comparison of data from different methods. This may be done to align with other patient results, reference intervals or clinical decision points. These factors, which we term Post Analytical Correction Factors (PACF), may be applied to all types of results derived from the method, i.e. quality control (QC) and external quality assurance (EQA), as well as the patient results. As the principal use of PACF is comparing patient results, it is important that the laboratory use commutable materials (i.e. patient samples) and a formal process to establish, apply and manage PACF. We report on preliminary guidelines for PACF from a recent workshop. [ABSTRACT FROM AUTHOR]

Aim: To audit clinical practice and assess early outcomes for infants with epileptic spasms after an agreed initial treatment protocol was adopted. Methods: We reviewed all cases of epileptic spasms diagnosed between July 2007 and June 2009 and assessed adherence to protocol, remission by day 14, spasm recurrence and side effects. The protocol required that infants be treated with high-dose oral prednisolone except those with tuberous sclerosis complex (TSC) who were treated with vigabatrin. Results: Twenty-eight infants (age 3-14 months, 17 male) were newly diagnosed. Six (21%) had no cause identified (cryptogenic), six (21%) had TSC and 16 (57%) had other non-TSC symptomatic aetiologies. Twenty-three were treated per protocol and five were not. The proportion with remission by day 14 of treatment was 100% in the cryptogenic group (all treated per protocol), 64% in those with non-TSC symptomatic aetiologies treated per protocol, 20% in those with non-TSC symptomatic aetiologies treated not per protocol and 17% in infants with TSC (all treated per protocol). Of 17 infants who received prednisolone, two were admitted for management of febrile illness. Conclusion: Our experience with high-dose oral prednisolone for treatment of epileptic spasms suggests that it is effective and tolerable. The greater proportion of non-TSC symptomatic patients with timely cessation of spasms when treated by this protocol supports the use of high-dose oral prednisolone as the treatment of choice. Given the poor response of children with TSC to treatment with vigabatrin, early use of steroid therapy deserves consideration. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Cerebral venous sinus compression can mimic idiopathic intracranial hypertension. The authors report the case of a 12- year-old girl who presented with diplopia and papilledema 3 weeks after a head injury Lumbar puncture confirmed raised intracranial pressure, and neuroimaging subsequently identified a skull fracture compressing the right transverse sinus. Papilledema and diplopia resolved following surgical elevation of the bone fragment. Computer tomography or magnetic resonance venography are indicated in children presenting with isolated intracranial hypertension following head injury to exclude cerebral venous sinus compression secondary to skull fracture. [ABSTRACT FROM AUTHOR]

Objective : To report the efficacy and tolerability of the ketogenic diet (KD) in refractory paediatric epilepsy. Methods : Twenty-six consecutive children were treated with the classical KD from 1996 to 2001. The epilepsy syndromes included symptomatic generalized epilepsy (15), idiopathic generalized epilepsy (4), symptomatic partial epilepsy (1) and unclassified epilepsy (6). One child was lost to follow up. Results : Median age at initiation of the KD was 6.1 years. Median duration of the treatment was 9 months. Twelve children (48%) were treated for >12 months; one still remains on the KD. Four children (16%) became seizure-free. Five children (20%) had 50–99% reduction in seizures, seven (28%) had <50% reduction in seizures and eight (36%) had no improvement. Age, seizure-type and aetiology did not predict response. The medications were decreased in 33% of the children. The KD was discontinued in 64% of the children because of poor efficacy and in 12% because of side-effects. Problems during initiation of the KD included asymptomatic hypoglycaemia (24%) and vomiting (12%). Later complications included poor growth (20%), hyperlipidaemia (16%), hypercalcuria (8%), hypocarnitaemia (8%), constipation (8%), pancreatitis (4%) and decreased bone density (4%). There were no deaths. A 3-month trial of the KD costs $A3879. The first 12 months cost $A7275 with a cost of $A4528 each year, thereafter. Conclusions : The KD is an effective treatment for some children with refractory epilepsy, being generally well tolerated and rarely associated with side-effects. Response is not necessarily predicted by age, syndrome or aetiology. A prospective study of the KD is presently underway. [ABSTRACT FROM AUTHOR]

The objective of this study was to compare the efficacy of corticotropin (ACTH) versus vigabatrin in treating infantile spasms and to determine which medication has a more favorable long-term outcome in terms of cognitive function, evolution of epilepsy, and incidence of autism. Patients with infantile spasms were included in the study if they were 3 to 16 months old, had hypsarrhythmia, and had no previous treatment with vigabatrin or corticosteroids. Patient evaluation included electroencephalographic and psychometric measures before and after treatment. Patients were stratified based on etiology (idiopathic or symptomatic) and sex and then randomized between the ACTH and vigabatrin treatment groups. Each of the treatment groups received either ACTH or vigabatrin for 2 weeks. At the end of 2 weeks of treatment, patients were considered responders if spasms and hypsarrhythmia resolved. Nonresponders were crossed over and treated with the alternate drug. Nine patients were included in the study. Three patients received ACTH, one of whom was a responder. Six patients received vigabatrin, three of whom were responders. The five nonresponders received both therapies. All patients had some degree of developmental plateau or regression before the initiation of treatment. Four patients with idiopathic infantile spasms showed improved cognitive function following treatment. The remaining five patients remained significantly delayed. Five patients with symptomatic infantile spasms had epilepsy following treatment; three of them were in the autistic spectrum. The small number of infants in this pilot study is insufficient to determine which of the two drugs is more effective. However, the following trends were identified: vigabatrin may be more effective for patients with symptomatic infantile spasms; patients with idiopathic infantile spasms tend to have a better cognitive outcome; and patients with symptomatic infantile spasms tend to develop both epilepsy and autism.... [ABSTRACT FROM AUTHOR]

Purpose. The results of a study to determine the long-term (up to 14 days) stability of diluted dexmedetomidine kept in polypropylene syringes under typical pharmacy storage conditions are presented. Methods. Four samples of dexmedetomidine injection diluted to 4 mg/mL in 0.9% sodium chloride were prepared and divided into 25-mL portions for storage in syringes at ambient room temperature (20-25 °C) with light exposure or under refrigeration (5 °C) in darkness. At 24 and 48 hours, the percentage of the initial dexmedetomidine concentration remaining in all samples was assessed via high-performance liquid chromatography with diode-array detection; further stability testing of the refrigerated samples was performed on days 7 and 14. At each time point, the test samples were visually inspected for color, clarity, and signs of formation of particulate matter. Results. As determined by chromatographic analyses, the samples of diluted dexmedetomidine stored in syringes at room temperature exhibited a loss of drug concentration of <10% over 48 hours; the refrigerated samples exhibited a loss of drug concentration of <5% over 14 days. All of the syringe-stored samples remained clear and colorless on visual inspection for the duration of the study. Conclusion. Dexmedetomidine diluted to 4 mg/mL in 0.9% sodium chloride injection was stable for at least 48 hours at 20-25 °C and 14 days at 5 °C when stored in polypropylene syringes. [ABSTRACT FROM AUTHOR]

The article focuses on treatment of childhood stroke. Topics discussed include childhood stroke guidelines provided by Royal College of Paediatrics and Child Health; prognosis, rehabilitation and clinical management of the same; and development of national clinical networks for standardizing the management along the continuum of care.

Information on the medium-term recovery of children with Bell palsy or acute idiopathic lower motor neuron facial paralysis is limited. We followed up children aged 6 months to <18 years with Bell palsy for 12 months after completion of a randomized trial on the use of prednisolone. We assessed facial function using the clinician-administered House-Brackmann scale and the modified parent-administered House-Brackmann scale. One hundred eighty-seven children were randomized to prednisolone (n = 93) or placebo (n = 94). At six months, the proportion of patients who had recovered facial function based on the clinician-administered House-Brackmann scale was 98% (n = 78 of 80) in the prednisolone group and 93% (n = 76 of 82) in the placebo group. The proportion of patients who had recovered facial function based on the modified parent-administered House-Brackmann scale was 94% (n = 75 of 80) vs 89% (n = 72 of 81) at six months (OR 1.88; 95% CI 0.60, 5.86) and 96% (n = 75 of 78) vs 92% (n = 73 of 79) at 12 months (OR 3.12; 95% CI 0.61, 15.98). Although the vast majority had complete recovery of facial function at six months, there were some children without full recovery of facial function at 12 months, regardless of prednisolone use. [ABSTRACT FROM AUTHOR]

Objective: In paediatric migraine, ibuprofen, acetaminophen and triptans are safe, effective therapies but there is scant paediatric data informing second‐line emergency treatment. Methods: Retrospective cohort study of children diagnosed with migraine at a tertiary children's hospital ED. Results: 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. Conclusions: While intravenous chlorpromazine as second‐line agent was mostly safe, it had unclear efficacy given the requirement for further treatment and hospital admissions. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

• Diagnosis of stroke in children is delayed in patients who are sedated or unable to participate in the neurological exam. • EEG is a sensitive modality of neuromonitoring for the recognition of focal voltage attenuation associated with stroke. • The Correlate of Injury to the Nervous System (COIN) index provides a numerical trend and visualization of stroke territory. To describe and assess performance of the Correlate Of Injury to the Nervous system (COIN) index, a quantitative electroencephalography (EEG) metric designed to identify areas of cerebral dysfunction concerning for stroke. Case-control study comparing continuous EEG data from children with acute ischemic stroke to children without stroke, with or without encephalopathy. COIN is calculated continuously and compares EEG power between cerebral hemispheres. Stroke relative infarct volume (RIV) was calculated from quantitative neuroimaging analysis. Significance was determined using a two-sample t-test. Sensitivity, specificity, and accuracy were measured using logistic regression. Average COIN values were −34.7 in the stroke cohort compared to −9.5 in controls without encephalopathy (p = 0.003) and −10.5 in controls with encephalopathy (p = 0.006). The optimal COIN cutoff to discriminate stroke from controls was −15 in non-encephalopathic and −18 in encephalopathic controls with >92% accuracy in strokes with RIV > 5%. A COIN cutoff of −20 allowed discrimination between strokes with <5% and >5% RIV (p = 0.027). We demonstrate that COIN can identify children with acute ischemic stroke. COIN may be a valuable tool for stroke identification in children. Additional studies are needed to determine utility as a monitoring technique for children at risk for stroke. [ABSTRACT FROM AUTHOR]

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 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. [ABSTRACT FROM AUTHOR]

Background: The determination of reliable, practical Quality Indicators (QIs) from presentation of the patient with a pathology request form through to the clinician receiving the report (the Total Testing Process or TTP) is a key step in identifying areas where improvement is necessary in laboratories. Methods: The Australasian QIs programme Key Incident Monitoring and Management System (KIMMS) began in 2008. It records incidents (process defects) and episodes (occasions at which incidents may occur) to calculate incident rates. KIMMS also uses the Failure Mode Effects Analysis (FMEA) to assign quantified risk to each incident type. The system defines risk as incident frequency multiplied by both a harm rating (on a 1-10 scale) and detection difficulty score (also a 1-10 scale). Results: Between 2008 and 2016, laboratories participating rose from 22 to 69. Episodes rose from 13.2 to 43.4 million; incidents rose from 114,082 to 756,432. We attribute the rise in incident rate from 0.86% to 1.75% to increased monitoring. Haemolysis shows the highest incidence (22.6% of total incidents) and the highest risk (26.68% of total risk). "Sample is suspected to be from the wrong patient" has the second lowest frequency, but receives the highest harm rating (10/10) and detection difficulty score (10/10), so it is calculated to be the 8th highest risk (2.92%). Similarly, retracted (incorrect) reports QI has the 10th highest frequency (3.9%) but the harm/difficulty calculation confers the second highest risk (11.17%). Conclusions: TTP incident rates are generally low (less than 2% of observed episodes), however, incident risks, their frequencies multiplied by both ratings of harm and discovery difficulty scores, concentrate improvement attention and resources on the monitored incident types most important to manage. [ABSTRACT FROM AUTHOR]

The article discusses a study that examined the emotional impact of Bell's palsy on children and their parents. Topics discussed include the demographics and steroid use in children with Bell's palsy, corticosteroid prescription, the recovery of motor function, and the unrecognised emotional impacts of Bell's palsy in children.

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. [ABSTRACT FROM AUTHOR]

Aim: There is a paucity of data from randomized controlled treatment trials in childhood arterial ischaemic stroke. Our objectives were to identify and plan a trial through use of a Delphi consensus process.Method: The Delphi panel consisted of Australian, New Zealand, and European paediatric neurologists with interests in childhood stroke. Four rounds were conducted using a REDCap (Research Electronic Data Capture) Web-based application: the first consisted of open-ended questions; the second evaluated agreement for the most important trial; the third and fourth reached consensus on design.Results: Forty-seven out of 66 neurologists answered the first round. Eight areas of research for important and feasible trials were identified. In the second round, 43 paediatric neurologists ranked the three highest rated trials: (1) aspirin versus aspirin plus steroids in focal arteriopathy (n=31); (2) heparin versus aspirin (n=6); and (3) heparin versus aspirin versus modern anticoagulation (n=6). The third and fourth surveys reached consensus among 43 out of 44 respondents on design of the highest ranked trial, and allowed agreement on inclusion/exclusion criteria, clinical/neuroimaging data, and treatment protocols.Conclusion: The Delphi consensus process is an efficient method of identifying and planning paediatric stroke trials. An international, multicentre trial is now in preparation. [ABSTRACT FROM AUTHOR]

Introduction: Health informatics and digital health, two rapidly growing disciplines, are becoming increasingly important to the sustainability of health service provision, highlighted especially through the COVID-19 pandemic. To maximise the benefits of the adoption and growth of health informatics and digital health, health service managers play a critical role in leading and managing the implementation and transformation of the system, both strategically and operationally, whilst still needing to manage 'business as usual'.Objectives: The objectives of the paper are to present and discuss the findings from a scoping review identifying: 1) competencies required for health service managers leading the implementation and transformation of informatics and digital technology in the health sector; and 2) factors that are critical to building the management workforce capacity in the era of health informatics and digital health.Methods: A scoping review of the literature was conducted in 2020 focussing on identifying empirical articles published in the English language since the year 2000 using a number of keywords such as 'health informatics', 'digital health', 'electronic health', 'competencies', 'capability', 'proficiency', 'qualification', 'certification', 'health manager', 'health executive' and 'health administrator'. The literature search was guided by a PRISMA approach searching within eight databases: Scopus, ProQuest, Web of Science, ACM Digital Library, CINAHL, PubMed, Google Scholar and ProQuest Dissertations.Results: After duplicates were removed, 941 publications were included for title screening as the result of an initial review. Title screening selected 185 articles to be included for abstract screening by two reviewers confirming 19 papers relevant to the focus of the current paper which were included in data extraction and content analysis. The analysis identified the additional competency of 'information and data management' be included as a core competency for health service managers. The analysis also confirmed additional elements for the following four core management competencies that are important to health service managers working in the digital health context, including: 1) leadership; 2) operational and resource management; 3) personal, interpersonal and professional qualities, and 4) understanding the industry and environment. Factors that are critical to developing the system and organization capacity in the use of health informatics and digital health technology, and leading and managing the adoption in the healthcare organizations were identified in three categories: 1) policy/system; 2) organizational structure and processes; and 3) people factors.Conclusions: This paper has taken an important step in confirming the competency requirements for health services managers that are relevant to leading and managing in the health informatics and digital health space, consequently indicating the directions for developing a competent workforce in meeting the existing and emerging healthcare delivery challenges, both now and in the future. [ABSTRACT FROM AUTHOR]

The article focuses on a study that investigates the childhood arterial ischemic stroke (AIS) risk factors (RFs) and examines the relationship of AIS on age, geography, and infarct features. It references the findings from the International Pediatric Stroke Study (IPSS) which describes the condition of probable RFs associated with childhood AIS. Chi-square tests and logistic regression were used to gather data from 676 children included in IPSS. Results show that RFs are common in childhood AIS.

Ketogenic diet is a structured effective treatment for children with intractable epilepsy. Several reports have indicated poor linear growth in children on the diet but the mechanism of poor growth has not been elucidated. We aimed to explore whether the protein to energy ratio plays a role in linear growth of children on ketogenic diet. Data regarding growth and nutrition were, retrospectively, collected from the clinical histories of 35 children who were treated with ketogenic diet for at least 6 months between 2002 and 2010. Patients were stratified into groups according to periods of satisfactory or poor linear growth. Poor linear growth was associated with protein or caloric intake of <80% recommended daily intake, and with a protein-to-energy ratio consistently ≤1.4 g protein/100 kcal even when protein and caloric intakes were adequate. We recommend a protein-to-energy ratio of 1.5 g protein/100 kcal be prescribed to prevent growth retardation. [ABSTRACT FROM PUBLISHER]

Summary Purpose: Febrile infection-related epilepsy syndrome (FIRES) is an increasingly recognized epileptic syndrome that presents with multifocal refractory status epilepticus in previously normal children and evolves into a chronic, refractory, focal epilepsy with associated cognitive and behavioral difficulties. Herein we describe the features of the chronic epilepsy and critically review evidence for the etiology of this syndrome. Methods: Seven patients with FIRES were studied. The duration of follow-up in six survivors was 5-17 years. Clinical, electroencephalography (EEG), neuroimaging, and other investigative findings during the acute and chronic phases were reviewed. Key Findings: These previously normal children presented with a febrile illness and status epilepticus that was refractory to antiepileptic medications in all children, to immunotherapies (including immunoglobulin, corticosteroids, plasma exchange, and rituximab) in four, and to acute vagus nerve stimulation in one. Markers of cerebral inflammation were few and response to antiepileptic and immunomodulatory therapies was poor. Evolution to chronic epilepsy occurred without a silent period. Seizure characteristics in the chronic phase were strikingly stereotyped and similar to the acute phase, with head and eye version, unilateral facial jerking, asymmetric tonic posturing, and unilateral limb jerking in all patients. Electrographic ictal onset was lateralized in all recorded seizures, unilateral in one patient, and independent bilateral in three. Seizures were refractory to multiple antiepileptic medications in all patients and partly responsive to chronic vagus nerve stimulation in two patients. Moderate to severe intellectual impairment was noted in four patients, and borderline intellectual abilities were noted in two. Magnetic resonance imaging (MRI) in the chronic phase was normal in three patients and showed mild diffuse cortical atrophy and/or mild hippocampal atrophy or sclerosis in three. Significance: The similar perirolandic and perisylvian features of acute and chronic seizures, the lack of a silent period, the absence of evidence of cerebral inflammation, and the poor response to immunotherapies suggest FIRES is best conceptualized as a chronic epilepsy with explosive onset, not a remote-symptomatic epilepsy with an acute inflammatory antecedent. [ABSTRACT FROM AUTHOR]

Transient cerebral arteriopathy is a frequent cause of childhood arterial ischemic stroke. Differentiating this condition from intracranial carotid artery dissection is challenging but important for initial treatment. We describe 4 cases from the International Pediatric Stroke Study of intracranial carotid artery dissection, initially misdiagnosed as transient cerebral arteriopathy. Presentations were abrupt, with focal neurological deficits in 4, preceding headache in 3, and minor trauma in 1. Infarcts involved the anterior circulation, and magnetic resonance angiography showed unilateral arterial stenosis/occlusion. None had evidence of dissection. All received anticoagulation or thrombolysis. Three died from refractory intracranial hypertension. Intracranial carotid artery dissection was confirmed postmortem (n = 3) and on dedicated MR wall imaging showing intramural hematoma (n = 1). In differentiating transient cerebral arteriopathy from intracranial carotid artery dissection, routine magnetic resonance angiography is unreliable and adjunctive conventional angiography, gadolinium magnetic resonance angiography, or dedicated MRI wall imaging should be considered. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to describe the frequency, risk factors, manifestations, and outcome of epilepsy in children with hemiplegic cerebral palsy (CP) due to perinatal arterial ischaemic stroke (AIS). Method The study group comprised 63 participants (41 males, 22 females) from a population-based CP register whose brain imaging showed perinatal AIS. Information collected included occurrence of neonatal seizures, family history of epilepsy, motor function and epilepsy onset, treatment, and outcome. Electroclinical findings were classified according to seizure semiology, seizure type, and epilepsy syndrome. Results Mean age of participants at the time of study was 10 years 6 months (SD 4y 7mo, range 4-20y). Gross Motor Function Classification System levels I and II were reported in 96% of participants, and Manual Ability Classification System levels I and II were reported in 79% of children. Thirty-four children (54%) developed epilepsy. Term delivery and more severe motor impairment were associated with epilepsy, but neonatal seizures and family history of epilepsy were not. Initial seizures were epileptic spasms, focal seizures, or myoclonic seizures. Focal seizure semiology suggested Rolandic or occipital seizure origin in the majority of children. Focal epileptic discharges in children with focal seizures had features of idiopathic partial epilepsy. Only 15% of children had active epilepsy 10 years after onset. Interpretation Despite a high incidence of epilepsy in children with hemiplegic CP due to AIS, the prognosis for seizure remission is good. Many children have clinical features, electroencephalography findings, and remission typical of idiopathic partial epilepsy. [ABSTRACT FROM AUTHOR]

Aim: Hypoglycaemic seizures are common in children with diabetes and electroencephalogram abnormalities are well recognised in this patient group. Elevated antibodies to glutamic acid decarboxylase, a major auto-antigen in Type 1 diabetes, are also implicated in a number of neurological disorders. Despite these associations, the question of whether children with diabetes are more prone to epilepsy, possibly as a result of lowered seizure threshold, has not been previously studied. We aimed to determine the prevalence and type of epilepsy in a large paediatric diabetes clinic. Methods: An audit by chart review was carried out at the Diabetes Clinic at the Royal Children's Hospital, Melbourne, Australia. Clinical, demographical, biochemical, EEG and neuro-imaging data were recorded. Results: Of 1384 children and adolescents aged 0–19 years with Type 1 diabetes, we identified 12 with active epilepsy (prevalence of 8.7/1000), the majority of whom had idiopathic generalised epilepsy and benign focal epilepsy of childhood. These findings are similar to those in the general population. Conclusion: Childhood epilepsy is no more frequently encountered in children and adolescents with Type 1 diabetes than in the general paediatric population. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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<0.05). Clinical factors associated with worse neurocognitive outcomes included more inpatient days during childhood, younger age at Fontan surgery, and longer time since Fontan procedure (P<0.05). Adults with a Fontan circulation had more marked neurocognitive dysfunction than adolescents with a Fontan circulation in 2 domains (psychomotor function, 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<0.001), but neither the presence nor severity of infarct, subcortical gray matter injury, and microhemorrhage was associated with neurocognitive outcomes. Compared with healthy controls, people with a Fontan circulation had smaller global brain volumes (P<0.001 in all regions) and smaller regional brain volumes in most cerebral cortical regions (P<0.05). Smaller global brain volumes were associated with worse neurocognitive functioning in several domains (P<0.05). A significant positive association was also identified between global brain volumes and resting oxygen saturations (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. [ABSTRACT FROM AUTHOR]

Stroke is an important cause of mortality and long-term morbidity in children. The aetiology of stroke in childhood differs from that of adults, with vasculopathies and congenital heart disease being the most commonly identified risk factors. Recognition and diagnosis are often delayed, limiting access to acute medical interventions such as thrombolysis. Optimal management of stroke in children is still not known and existing guidelines are at the level of expert consensus. Interdisciplinary childhood stroke programmes are required to meet the needs of this population and to contribute to the development of evidence-based therapies. [ABSTRACT FROM AUTHOR]

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

Objective: To determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment. Methods: Identification of the underlying etiology and response to treatment in 377 infants enrolled in a clinical trial of the treatment of infantile spasms between 2007 and 2014 using a systematic review of history, examination, and investigations. They were classified using the pediatric adaptation of International Classification of Diseases, Tenth Revision (ICD‐10). Results: A total of 219 of 377 (58%) had a proven etiology, of whom 128 (58%) responded, 58 of 108 (54%) were allocated hormonal treatment, and 70 of 111 (63%) had combination therapy. Fourteen of 17 (82%, 95% confidence interval [CI] 59% to 94%) infants with stroke and infarct responded (compared to 114 of 202 for the rest of the proven etiology group (56%, 95% CI 48% to 62%, chi‐square 4.3, P = .037): the better response remains when treatment allocation and lead time are taken into account (odds ratio 5.1, 95% CI 1.1 to 23.6, P = .037). Twenty of 37 (54%, 95% CI 38% to 70%) infants with Down syndrome had cessation of spasms compared to 108 of 182 (59%, 95% CI 52% to 66%, chi‐square 0.35, P = .55) for the rest of the proven etiology group. The lack of a significant difference remains after taking treatment modality and lead‐time into account (odds ratio 0.8, 95% CI 0.4 to 1.7, P = .62). In Down syndrome infants, treatment modality did not appear to affect response: 11 of 20 (55%) allocated hormonal therapy responded, compared to 9 of 17 (53%) allocated combination therapy. Significance: This classification allows easy comparison with other classifications and with our earlier reports. Stroke and infarct have a better outcome than other etiologies, whereas Down syndrome might not respond to the addition of vigabatrin to hormonal treatment. [ABSTRACT FROM AUTHOR]