Search

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]

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]

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.

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]

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: To describe 5-year motor and functional outcomes after paediatric arterial ischaemic stroke (AIS) and to explore factors associated with poorer long-term outcome.Method: Thirty-three children (21 males, 12 females) with AIS were recruited to a single-site, cross-sectional study, from a previously reported prospective longitudinal stroke outcome study. Children were stratified according to age at diagnosis: neonates (≤30d), preschool (>30d-5y), and school age (≥5y). Motor and functional outcomes were measured at 5 years after stroke. Neurological outcomes were evaluated using the Pediatric Stroke Outcome Measure (PSOM) at 1 month and more than 4 years after stroke.Results: At 5 years after stroke, motor function, quality of life, fatigue, adaptive behaviour, activities of daily living, and handwriting speed were significantly poorer than age expectations. The preschool group had the highest percentage of fine and gross motor impairment. Poorer fine motor skills were associated with subcortical-only lesions and large lesion size. Poorer gross motor outcomes correlated with preschool age, bilateral lesions, and PSOM impairment at 1 month.Interpretation: Children are at elevated risk for motor and functional impairments after AIS, with the preschool age group most vulnerable. Identifying early predictors of poorer outcomes facilitates targeted early intervention and long-term rehabilitation.What This Paper Adds: Following paediatric stroke, children are at elevated risk of motor and functional difficulties. Stroke occurring between 30 days and 5 years of age may result in poorer motor and functional outcomes. [ABSTRACT FROM AUTHOR]

Aim: Childhood stroke disrupts brain development and emerging neural networks. Motor, cognitive, and language deficits are well recognized, yet little is known about psychosocial function after childhood stroke. This study aims to describe psychosocial function within the first year after childhood stroke, and to identify factors associated with outcome.Method: Thirty-seven children were involved in a prospective, longitudinal study investigating recovery over the first year after childhood stroke. Children's social functioning was assessed at 6-months and 12-months poststroke and psychological function at 12-months poststroke, using standardized measures.Results: Mean social function was poorer at both 6-months and 12-months poststroke, compared to prestroke. Psychological problems were more common than expected, with emotional difficulties and hyperactivity-inattention most significantly affected. Poorer social function was associated with older age at onset, acute neurological impairment, and prestroke social impairment. Social and psychological problems were associated with parent mental health.Interpretation: While not all children are affected, psychosocial impairment affects a significant minority after childhood stroke. Older age at onset, acute neurological impairment, prestroke social problems, and poorer parent mental health were associated with deficits. Identifying early predictors of poorer outcome will facilitate early intervention. Of particular importance is parent mental health, suggesting support for families may improve child outcome. [ABSTRACT FROM AUTHOR]

Aim: Little is known about psychological and social outcomes after paediatric stroke. This study aimed to evaluate psychosocial outcomes in children 5 years after paediatric stroke and explore the contribution of early presenting factors.Method: Thirty-one children (19 males, 12 females) with arterial ischemic stroke were involved in this prospective, longitudinal study. Children underwent intellectual assessment at 12 months poststroke and parents completed questionnaires rating their own mental health and their child's functioning at 12 months and 5 years poststroke.Results: At 5-year follow-up, psychological and social function were significantly poorer than normative expectations. Exploration of early predictive factors showed poorer cognitive and psychological function at 12 months poststroke and older age at stroke onset was associated with poorer psychosocial function at 5 years. Larger lesion size was also associated with poorer psychological function at 5 years poststroke.Interpretation: These early predictors of poorer psychosocial outcome suggest that screening children within the first year after stroke may identify children most at risk of later problems and facilitate early intervention. [ABSTRACT FROM AUTHOR]

Aim: Loss-of-function mutations in SLC2A1, encoding glucose transporter-1 (GLUT-1), lead to dysfunction of glucose transport across the blood-brain barrier. Ten percent of cases with hypoglycorrhachia (fasting cerebrospinal fluid [CSF] glucose <2.2mmol/L) do not have mutations. We hypothesized that GLUT1 deficiency could be due to non-coding SLC2A1 variants.Method: We performed whole exome sequencing of one proband with a GLUT1 phenotype and hypoglycorrhachia negative for SLC2A1 sequencing and copy number variants. We studied a further 55 patients with different epilepsies and low CSF glucose who did not have exonic mutations or copy number variants. We sequenced non-coding promoter and intronic regions. We performed mRNA studies for the recurrent intronic variant.Results: The proband had a de novo splice site mutation five base pairs from the intron-exon boundary. Three of 55 patients had deep intronic SLC2A1 variants, including a recurrent variant in two. The recurrent variant produced less SLC2A1 mRNA transcript.Interpretation: Fasting CSF glucose levels show an age-dependent correlation, which makes the definition of hypoglycorrhachia challenging. Low CSF glucose levels may be associated with pathogenic SLC2A1 mutations including deep intronic SLC2A1 variants. Extending genetic screening to non-coding regions will enable diagnosis of more patients with GLUT1 deficiency, allowing implementation of the ketogenic diet to improve outcomes. [ABSTRACT FROM AUTHOR]

Aim: We aimed to determine whether response to ketogenic dietary therapies (KDT) was due to undiagnosed glucose transporter type 1 deficiency syndrome (GLUT1-DS).Method: Targeted resequencing of the SLC2A1 gene was completed in individuals without previously known GLUT1-DS who received KDT for their epilepsy. Hospital records were used to obtain demographic and clinical data. Response to KDT at various follow-up points was defined as seizure reduction of at least 50%. Seizure freedom achieved at any follow-up point was also documented. Fisher's exact and gene-burden association tests were conducted using the PLINK/SEQ open-source genetics library.Results: Of the 246 participants, one was shown to have a novel variant in SLC2A1 that was predicted to be deleterious. This individual was seizure-free on KDT. Rates of seizure freedom in cases without GLUT1-DS were below 8% at each follow-up point. Two cases without SLC2A1 mutations were seizure-free at every follow-up point recorded. No significant results were obtained from Fisher's exact or gene-burden association tests.Interpretation: A favourable response to KDT is not solely explained by mutations in SLC2A1. Other genetic factors should be sought to identify those who are most likely to benefit from dietary treatment for epilepsy, particularly those who may achieve seizure freedom. [ABSTRACT FROM AUTHOR]