Outcome Trajectories after Primary Perinatal Hemorrhagic Stroke.

BACKGROUND: Perinatal hemorrhagic stroke in late preterm and term neonates is understudied. We describe two-month and two-year neurological outcomes in a prospective cohort. METHODS: Neonates ≥36 weeks' gestation with spontaneous hemorrhagic stroke (parenchymal and intraventricular) presenting at age ≤28 days were enrolled between March 2007 and May 2015 at three tertiary pediatric centers. Hemorrhagic transformation of arterial ischemic stroke or cerebral sinovenous thrombosis was excluded. The Pediatric Stroke Outcome Measure (PSOM) assessed outcomes. Wilcoxon signed-rank tests evaluated change over time. RESULTS: Twenty-six neonates were included (median age: 1 day, interquartile range [IQR] 0 to 16; median gestational age: 38.3 weeks, IQR 37.0 to 39.0). Hemorrhage was isolated intraventricular in seven (27%), isolated intraparenchymal in six (23%), and a combination in 10 (39%). Three neonates (12%) died during hospitalization; one died later due to cardiac disease. Among 22 survivors, outcomes were assessed at a median of 2.1 months (IQR 1.7 to 3.3) in 96% and 1.9 years (IQR 1.3 to 2.0) in 73%. Median PSOM scores were 0.0 (IQR 0.0 to 1.0) and 0.25 (IQR 0.0 to 1.3), respectively. At two years, 45% of patients had no or nonimpairing deficits (PSOM <1.0), 30% had mild deficits (PSOM 1.0 to 2.0), and 5% had moderate deficits (PSOM 2.5 to 4.5). Over time, 31% worsened and 6% improved. Although total PSOM scores did not change significantly (P = 0.08), language subscores worsened (P = 0.009). No child developed epilepsy. CONCLUSIONS: Perinatal hemorrhagic stroke survivors had favorable outcomes in early childhood; at two years moderate to severe deficits occurred in 5%. Language deficits may emerge over time, warranting close follow-up.

Neonatal seizures triple the risk of a remote seizure after perinatal ischemic stroke.

OBJECTIVES: To determine incidence rates and risk factors of remote seizure after perinatal arterial ischemic stroke. METHODS: We retrospectively identified a population-based cohort of children with perinatal arterial ischemic stroke (presenting acutely or in a delayed fashion) from a large Northern Californian integrated health care system. We determined incidence and predictors of a remote seizure (unprovoked seizure after neonatal period, defined as 28 days of life) by survival analyses, and measured epilepsy severity in those with active epilepsy (≥1 remote seizure and maintenance anticonvulsant treatment) at last follow-up. RESULTS: Among 87 children with perinatal stroke, 40 (46%) had a seizure in the neonatal period. During a median follow-up of 7.1 years (interquartile range 3.2-10.5), 37 children had ≥1 remote seizure. Remote seizure risk was highest during the first year of life, with a 20% (95% confidence interval [CI] 13%-30%) cumulative incidence by 1 year of age, 46% (CI 35%-58%) by 5 years, and 54% (CI 41%-67%) by 10 years. Neonatal seizures increased the risk of a remote seizure (hazard ratio 2.8, CI 1.3-5.8). Children with neonatal seizures had a 69% (CI 48%-87%) cumulative incidence of remote seizure by age 10 years. Among the 24 children with active epilepsy at last follow-up, 8 (33%) were having monthly seizures despite an anticonvulsant and 7 (29%) were on more than one anticonvulsant. CONCLUSIONS: Remote seizures and epilepsy, including medically refractory epilepsy, are common after perinatal stroke. Neonatal seizures are associated with nearly 3-fold increased remote seizure risk.

Hemiparesis and epilepsy are associated with worse reported health status following unilateral stroke in children.

BACKGROUND: Perinatal and childhood stroke result in neurological impairment in the majority of survivors, but less is known about patient and parent perception of function following stroke in children. Our aim was to characterize parent-proxy and child-reported health status in children following unilateral arterial ischemic stroke or intraparenchymal hemorrhage. METHODS: Fifty-nine children 2-18 years (30 girls, 29 boys) with unilateral arterial ischemic stroke or spontaneous intraparenchymal hemorrhage at least 6 months before evaluation were enrolled from a single center. The PedsQL version 4.0 Generic Short Form and PedsQL version 3.0 Cerebral Palsy Module were administered to childhood stroke subjects and parents. Generic PedsQL Inventory scores were compared between children with stroke and published data from healthy children. Reported health status scores for children with varying degrees of hemiparesis were compared. RESULTS: Children with stroke had lower reported health status scores on the Generic PedsQL Inventory than healthy children. Children with moderate-severe hemiparesis had worse scores than children without hemiparesis on several measures of the Cerebral Palsy Module as reported by both parents and children. The parents of children with epilepsy reported worse scores on several measures compared with children without epilepsy, and the parent scores were lower on several measures for children with lower intelligence quotients. Agreement between parent and child scores was better on the Cerebral Palsy Module than on the Generic Inventory. CONCLUSIONS: Children with stroke have worse reported health status than healthy controls. Degree of hemiparesis, epilepsy, and lower intelligence quotient affect reported health status on some measures. Agreement between parent-proxy and child scores ranges from slight to good which suggests that both provide useful information.

Haemorrhagic stroke in term and late preterm neonates.

OBJECTIVE: Few data regarding causes and outcomes of haemorrhagic stroke (HS) in term neonates are available. We characterised risk factors, mechanism and short-term outcomes in term and late preterm neonates with acute HS. DESIGN: Prospective cohort. SETTING: Single-centre tertiary care stroke registry. SUBJECTS: Term and late preterm neonates (≥ 34 weeks gestation), born 2004-2010, with acute HS ≤ 28 days of life were identified, and clinical information was abstracted. Short-term outcomes were assessed via standardised neurological exam and rated using the Paediatric Stroke Outcome Measure (PSOM). RESULTS: Among 42 neonates, median gestational age was 39.7 weeks (IQR 38-40.7 weeks). Diagnosis occurred at a median of 1 day (IQR 0-7 days) after delivery. Twenty-seven (64%) had intraparenchymal and intraventricular haemorrhage. Mechanism was haemorrhagic transformation of venous or arterial infarction in 22 (53%). Major risk factors included congenital heart disease (CHD), fetal distress and haemostatic abnormalities. Common presentations included seizure, apnoea, and poor feeding or vomiting. Acute hydrocephalus was common. Mortality was 12%. Follow-up occurred in 36/37 survivors at a median of 1 year (IQR 0.5-2.0 years). Among 17/36 survivors evaluated in stroke clinic, 47% demonstrated neurologic deficits. Deficits were mild (PSOM 0.5-1.5) in 9/36 (25%), and moderate-to-severe (PSOM ≥ 2.0) in 8/36 (22%). CONCLUSIONS: In our cohort with acute HS, most presented with seizures, apnoea and/or poor feeding. Fetal distress and CHD were common. Nearly two-thirds had intraparenchymal with intraventricular haemorrhage. Over half were due to haemorrhagic transformation of infarction. Short-term neurologic deficits were present in 47% of survivors.

Frequency of hematoma expansion after spontaneous intracerebral hemorrhage in children.

IMPORTANCE: Hematoma expansion is the only modifiable predictor of outcome in adult intracerebral hemorrhage; however, the frequency and clinical significance of hematoma expansion after childhood intracerebral hemorrhage are unknown. OBJECTIVE: To assess the frequency and extent of hematoma expansion in children with nontraumatic intracerebral hemorrhage. DESIGN, SETTING, AND PARTICIPANTS: Prospective observational cohort study at 3 tertiary care pediatric hospitals. Children (≥ 37 weeks' gestation to 18 years) with nontraumatic intracerebral hemorrhage were enrolled in a study from 2007 to 2012 focused on predictors of outcome. For this planned substudy of hematoma expansion, neonates 28 days or younger and participants with isolated intraventricular hemorrhage were excluded. Children with 2 head computed tomography (CT) scans within 48 hours were evaluated for hematoma expansion and were compared with children with only 1 head CT scan. Consent for the primary cohort was obtained from 73 of 87 eligible participants (84%); 41 of 73 children enrolled in the primary cohort met all inclusion/exclusion criteria for this substudy, in whom 22 had 2 head CT scans obtained within 48 hours that could be evaluated for hematoma expansion. Within our substudy cohort, 21 of 41 (51%) were male, 25 of 41 (61%) were white, 16 of 41 (39%) were black, and median age was 7.7 years (interquartile range, 2.0-13.4 years). MAIN OUTCOME AND MEASURE: Primary outcome was prevalence of hematoma expansion. RESULTS: Of 73 children, 41 (56%) met inclusion criteria, and 22 (30%) had 2 head CT scans to evaluate expansion. Among these 22 children, median time from symptom onset to first CT was 2 hours (interquartile range, 1.3-6.5 hours). Median baseline hemorrhage volume was 19.5 mL, 1.6% of brain volume. Hematoma expansion occurred in 7 of 22 (32%). Median expansion was 4 mL (interquartile range, 1-11 mL). Three children had significant (>33%) expansion; 2 required urgent hematoma evacuation. Expansion was not associated with poorer outcome. Compared with children with only 1 head CT scan within 48 hours, children with 2 head CT scans had larger baseline hemorrhage volumes (P = .05) and were more likely to receive treatment for elevated intracranial pressure (P < .001). CONCLUSIONS AND RELEVANCE: Hematoma expansion occurs in children with intracerebral hemorrhage and may require urgent treatment. Repeat CT should be considered in children with either large hemorrhage or increased intracranial pressure.

Pediatric intracerebral hemorrhage score: a simple grading scale for intracerebral hemorrhage in children.

BACKGROUND AND PURPOSE: The intracerebral hemorrhage (ICH) score is the most commonly used clinical grading scale for outcome prediction after adult ICH. We created a similar scale in children to inform clinical care and assist in clinical research. METHODS: Children, full-term newborns to 18 years, with spontaneous ICH were prospectively enrolled from 2007 to 2012 at 3 centers. The pediatric ICH score was created by identifying factors associated with poor outcome. The score's ability to detect moderate disability or worse and severe disability or death was examined with sensitivity, specificity, and area under the receiver operating characteristic curve. RESULTS: The pediatric ICH score components include ICH volume>2% to 3.99% of total brain volume (TBV): 1 point; ICH volume≥4% TBV: 2 points; acute hydrocephalus: 1 point; herniation: 1 point; and infratentorial location: 1 point. The score ranges from 0 to 5. At 3-month follow-up of 60 children, 10 were severely disabled or dead, 30 had moderate disability, and 20 had good recovery. A pediatric ICH score≥1 predicted moderate disability or worse with a sensitivity of 75% (95% confidence interval [CI], 59% to 87%) and a specificity of 70% (95% CI, 46% to 88%). A pediatric ICH score≥2 predicted severe disability or death with a sensitivity and specificity of 90% (95% CI, 55% to 99%) and 68% (95% CI, 53% to 80%), respectively. The area under the receiver operating characteristic curve for classifying outcome as severe disability or death was 0.88 (95% CI, 0.78-0.97). CONCLUSIONS: The pediatric ICH score is a simple clinical grading scale that may ultimately be used for risk stratification, clinical care, and research.

Elementary school children's attentional biases in physical and numerical space.

Numbers are conceptualized spatially along a horizontal mental line. This view is supported by mounting evidence from healthy adults and patients with unilateral spatial neglect. Little is known about children's representation of numbers with respect to space. This study investigated elementary school children's directional biases in physical and numerical space to better understand the relation between space and number. We also examined the nature of spatial organization in numerical space. In two separate tasks, children (n=57) were asked to bisect a physical line and verbally estimate the midpoint of number pairs. In general, results indicated leftward biases in both tasks, but the degree of deviation did not correlate between the tasks. In the number bisection task, leftward bias (underestimating the midpoint) increased as a function of numerical magnitude and interval between number pairs. In contrast, a rightward deviation was found for smaller number pairs. These findings suggest that different underlying spatial attentional mechanisms might be directed in physical and numerical space in young school children, which would be integrated in adulthood.

The development of organized visual search.

Visual search plays an important role in guiding behavior. Children have more difficulty performing conjunction search tasks than adults. The present research evaluates whether developmental differences in children's ability to organize serial visual search (i.e., search organization skills) contribute to performance limitations in a typical conjunction search task. We evaluated 134 children between the ages of 2 and 17 on separate tasks measuring search for targets defined by a conjunction of features or by distinct features. Our results demonstrated that children organize their visual search better as they get older. As children's skills at organizing visual search improve they become more accurate at locating targets with conjunction of features amongst distractors, but not for targets with distinct features. Developmental limitations in children's abilities to organize their visual search of the environment are an important component of poor conjunction search in young children. In addition, our findings provide preliminary evidence that, like other visuospatial tasks, exposure to reading may influence children's spatial orientation to the visual environment when performing a visual search.

Pediatric intracerebral hemorrhage: acute symptomatic seizures and epilepsy.

IMPORTANCE: Seizures are believed to be common presenting symptoms in neonates and children with spontaneous intracerebral hemorrhage (ICH). However, few data are available on the epidemiology of acute symptomatic seizures or the risk for later epilepsy. OBJECTIVE: To define the incidence of and explore risk factors for seizures and epilepsy in children with spontaneous ICH. Our a priori hypotheses were that younger age at presentation, cortical involvement of ICH, acute symptomatic seizures after presentation, ICH due to vascular malformation, and elevated intracranial pressure requiring urgent intervention would predict remote symptomatic seizures and epilepsy. DESIGN: Prospective cohort study conducted between March 1, 2007, and January 1, 2012. SETTING: Three tertiary care pediatric hospitals. PARTICIPANTS: Seventy-three pediatric subjects with spontaneous ICH including 20 perinatal (≥37 weeks' gestation to 28 days) and 53 childhood subjects (>28 days to <18 years at presentation). MAIN OUTCOME MEASURES: Acute symptomatic seizures (clinically evident and electrographic-only seizures within 7 days), remote symptomatic seizures, and epilepsy. RESULTS: Acute symptomatic seizures occurred in 35 subjects (48%). Acute symptomatic seizures as a presenting symptom of ICH occurred in 12 perinatal (60%) and 19 childhood (36%) subjects (P = .07). Acute symptomatic seizures after presentation occurred in 7 children. Electrographic-only seizures were present in 9 of 32 subjects (28%) with continuous electroencephalogram monitoring. One-year and 2-year remote symptomatic seizure-free survival rates were 82% (95% CI, 68-90) and 67% (95% CI, 46-82), respectively. One-year and 2-year epilepsy-free survival rates were 96% (95% CI, 83-99) and 87% (95% CI, 65-95), respectively. Elevated intracranial pressure requiring acute intervention was a risk factor for seizures after presentation (P = .01; Fisher exact test), remote symptomatic seizures, and epilepsy (P = .03, and P = .04, respectively; log-rank test). CONCLUSIONS AND RELEVANCE: Presenting seizures are common in perinatal and childhood ICH. Continuous electroencephalography may detect electrographic seizures in some subjects. Single remote symptomatic seizures occur in many, and development of epilepsy is estimated to occur in 13% of patients at 2 years. Elevated intracranial pressure requiring acute intervention is a risk factor for acute seizures after presentation, remote symptomatic seizures, and epilepsy.

Modified Pediatric ASPECTS Correlates with Infarct Volume in Childhood Arterial Ischemic Stroke.

BACKGROUND AND PURPOSE: Larger infarct volume as a percent of supratentorial brain volume (SBV) predicts poor outcome and hemorrhagic transformation in childhood arterial ischemic stroke (AIS). In perinatal AIS, higher scores on a modified pediatric version of the Alberta Stroke Program Early CT Score using acute MRI (modASPECTS) predict later seizure occurrence. The objectives were to establish the relationship of modASPECTS to infarct volume in perinatal and childhood AIS and to establish the interrater reliability of the score. METHODS: We performed a cross sectional study of 31 neonates and 40 children identified from a tertiary care center stroke registry with supratentorial AIS and acute MRI with diffusion weighted imaging (DWI) and T2 axial sequences. Infarct volume was expressed as a percent of SBV using computer-assisted manual segmentation tracings. ModASPECTS was performed on DWI by three independent raters. The modASPECTS were compared among raters and to infarct volume as a percent of SBV. RESULTS: ModASPECTS correlated well with infarct volume. Spearman rank correlation coefficients (ρ) for the perinatal and childhood groups were 0.76, p < 0.001 and 0.69, p < 0.001, respectively. Excluding one perinatal and two childhood subjects with multifocal punctate ischemia without large or medium sized vessel stroke, ρ for the perinatal and childhood groups were 0.87, p < 0.001 and 0.80, p < 0.001, respectively. The intraclass correlation coefficients for the three raters for the neonates and children were 0.93 [95% confidence interval (CI) 0.89-0.97, p < 0.001] and 0.94 (95% CI 0.91-0.97, p < 0.001), respectively. CONCLUSION: The modified pediatric ASPECTS on acute MRI can be used to estimate infarct volume as a percent of SBV with a high degree of validity and interrater reliability.

Cranial irradiation increases risk of stroke in pediatric brain tumor survivors.

BACKGROUND AND PURPOSE: The purposes of this study were to determine the incidence of neurovascular events as late complications in pediatric patients with brain tumor and to evaluate radiation as a risk factor. METHODS: Patients were ascertained using the tumor database of a pediatric tertiary care center. Included patients had a primary brain tumor, age birth to 21 years, initial treatment January 1, 1993, to December 31, 2002, and at least 2 visits with neuro-oncology. Radiation exposure included: whole brain, whole brain plus a focal boost, or focal brain. The primary outcome was stroke or transient ischemic attack. RESULTS: Of 431 subjects, 14 had 19 events of stroke or transient ischemic attack over a median follow-up of 6.3 years. The incidence rate was 548/100 000 person-years. Overall, 61.5% of subjects received radiation, including 13 of 14 subjects with events. Median time from first radiation to first event was 4.9 years. The stroke/transient ischemic attack hazard ratio for any brain irradiation was 8.0 (95% CI, 1.05-62; P=0.045); for the circle of Willis, radiation was 9.0 (95% CI, 1.2-70; P=0.035); and for focal noncircle of Willis, radiation was 3.4 (95% CI, 0.21-55; P=0.38). CONCLUSIONS: The incidence of neurovascular events in this population is 100-fold higher than in the general pediatric population and cranial irradiation is an important risk factor. By defining the incidence of this late effect, physicians are better able to counsel parents regarding treatment, monitor patients at risk, and target a population for primary stroke prevention in future studies.

The Pediatric Stroke Recurrence and Recovery Questionnaire: validation in a prospective cohort.

OBJECTIVE: We developed the Recurrence and Recovery Questionnaire (RRQ) by converting the Pediatric Stroke Outcome Measure (PSOM) to a questionnaire for telephone interview and sought to validate the RRQ in a large cohort. METHOD: We analyzed parents' RRQ responses and same-day PSOM assessments for 232 children who had arterial ischemic stroke, cerebral sinovenous thrombosis, or presumed perinatal ischemic stroke. We assessed the agreement and consistency of the PSOM and RRQ, and we identified conditions that contributed to differences between the 2 measures. We tested selected factors as predictors of differences between the total PSOM and total RRQ (tPSOM and tRRQ) scores. RESULTS: Median PSOM score was 1.5 and median RRQ score was 1.5. There was good agreement between tPSOM and tRRQ, and RRQ was a reliable estimator of PSOM at the total and component level. Preexisting neurologic deficits or chronic illnesses increased the difference between the tPSOM and tRRQ; the chronic illness effect was confirmed with univariate analysis. CONCLUSIONS: The RRQ can characterize poststroke function when a child cannot return for examination. While the RRQ is not identical to the PSOM, the 2 measures likely assess closely related aspects of recovery. The RRQ is particularly useful when assessing outcomes of large cohorts, and will be useful in performing long-term follow-up studies of pediatric stroke.

Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association.

BACKGROUND: The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively review the literature and create recommendations to optimize neurodevelopmental outcome in the pediatric congenital heart disease (CHD) population. METHODS AND RESULTS: A writing group appointed by the American Heart Association and American Academy of Pediatrics reviewed the available literature addressing developmental disorder and disability and developmental delay in the CHD population, with specific attention given to surveillance, screening, evaluation, and management strategies. MEDLINE and Google Scholar database searches from 1966 to 2011 were performed for English-language articles cross-referencing CHD with pertinent search terms. The reference lists of identified articles were also searched. The American College of Cardiology/American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. A management algorithm was devised that stratified children with CHD on the basis of established risk factors. For those deemed to be at high risk for developmental disorder or disabilities or for developmental delay, formal, periodic developmental and medical evaluations are recommended. A CHD algorithm for surveillance, screening, evaluation, reevaluation, and management of developmental disorder or disability has been constructed to serve as a supplement to the 2006 American Academy of Pediatrics statement on developmental surveillance and screening. The proposed algorithm is designed to be carried out within the context of the medical home. This scientific statement is meant for medical providers within the medical home who care for patients with CHD. CONCLUSIONS: Children with CHD are at increased risk of developmental disorder or disabilities or developmental delay. Periodic developmental surveillance, screening, evaluation, and reevaluation throughout childhood may enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning.

Concurrent validity and reliability of retrospective scoring of the Pediatric National Institutes of Health Stroke Scale.

BACKGROUND AND PURPOSE: The Pediatric National Institutes of Health Stroke Scale (PedNIHSS), an adaptation of the adult National Institutes of Health Stroke Scale, is a quantitative measure of stroke severity shown to be reliable when scored prospectively. The ability to calculate the PedNIHSS score retrospectively would be invaluable in the conduct of observational pediatric stroke studies. The study objective was to assess the concurrent validity and reliability of estimating the PedNIHSS score retrospectively from medical records. METHODS: Neurological examinations from medical records of 75 children enrolled in a prospective PedNIHSS validation study were photocopied. Four neurologists of varying training levels blinded to the prospective PedNIHSS scores reviewed the records and retrospectively assigned PedNIHSS scores. Retrospective scores were compared among raters and to the prospective scores. RESULTS: Total retrospective PedNIHSS scores correlated highly with total prospective scores (R(2)=0.76). Interrater reliability for the total scores was "excellent" (intraclass correlation coefficient, 0.95; 95% CI, 0.94-0.97). Interrater reliability for individual test items was "substantial" or "excellent" for 14 of 15 items. CONCLUSIONS: The PedNIHSS score can be scored retrospectively from medical records with a high degree of concurrent validity and reliability. This tool can be used to improve the quality of retrospective pediatric stroke studies.

Evaluation of intraventricular hemorrhage in pediatric intracerebral hemorrhage.

Previous studies of pediatric intracerebral hemorrhage have investigated isolated intraparenchymal hemorrhage. The authors investigated whether detailed assessment of intraventricular hemorrhage enhanced outcome prediction after intracerebral hemorrhage. They prospectively enrolled 46 children, full-term to 17 years, median age 2.7 years, with spontaneous intraparenchymal hemorrhage and/or intraventricular hemorrhage. Outcome was assessed with the King's Outcome Scale for Childhood Head Injury. Twenty-six (57%) had intraparenchymal hemorrhage, 10 (22%) had pure intraventricular hemorrhage, and 10 (22%) had both. There were 2 deaths, both with intraparenchymal hemorrhage and intraventricular hemorrhage volume ≥4% of total brain volume. Presence of intraventricular hemorrhage was not associated with poor outcome, but hydrocephalus showed a trend (P = .09) toward poor outcome. In receiver operating characteristic curve analysis, combined intraparenchymal hemorrhage and intraventricular hemorrhage volume also showed a trend toward better outcome prediction than intraparenchymal hemorrhage volume alone. Although not an independent outcome predictor, future studies should assess intraventricular hemorrhage qualitatively and quantitatively.

Risk of later seizure after perinatal arterial ischemic stroke: a prospective cohort study.

OBJECTIVE: Although acute seizures are common among neonates with arterial ischemic stroke (AIS), the incidence of subsequent seizures is unknown. The goals of this study were to determine the incidence of seizures following hospital discharge after perinatal acute AIS, and to assess lesion characteristics associated with later seizure occurrence. METHODS: Neonates with confirmed acute AIS on MRI were identified through a prospective stroke registry. Clinic visits and telephone follow-up identified occurrence of seizures after hospital discharge. MRI scans were graded for size and characteristics of infarct, and associations with seizures after stroke were analyzed. RESULTS: At a mean (SD) follow-up of 31.3 (16.1) months, 11 of 46 (23.9%) patients with perinatal AIS had at least 1 seizure. Five patients had a single episode of seizure, and 6 developed epilepsy. The Kaplan-Meier probability of remaining seizure-free at 3 years was 73%. Stroke size on MRI was significantly associated with development of later seizures, with an incidence rate of later seizures 6.2 times higher among those with larger stroke size. CONCLUSIONS: Seizures occurred in <25% of patients during initial follow-up after perinatal AIS. Of those with seizures, nearly half had a single episode of seizure and not early epilepsy. Larger stroke size was associated with higher risk of seizure. These data suggest that prolonged treatment with anticonvulsant agents may not be indicated for seizure prophylaxis after perinatal AIS. These findings may help guide clinicians in counseling families and could form the basis for much-needed future research in this area.

Seizures as a presenting symptom of acute arterial ischemic stroke in childhood.

OBJECTIVES: To define the incidence of seizures as a presenting symptom of acute arterial ischemic stroke (AIS) in children and to determine whether younger age, infarct location, or AIS etiology were risk factors for seizure at AIS presentation. STUDY DESIGN: Children aged 2 months to 18 years presenting with AIS between January 2005 and December 2008 were identified from a single center prospective pediatric stroke registry. Clinical data were abstracted, and a neuroradiologist reviewed imaging studies. RESULTS: Among the 60 children who met our inclusion criteria, 13 experienced seizure at stroke presentation (22%). Median age was significantly younger in children who presented with seizures than in those who did not (1.1 years vs 10 years; P = .0009). Seizures were accompanied by hemiparesis in all patients. Three of 4 children with clinically overt seizures at presentation also had nonconvulsive seizures on continuous electroencephalography monitoring. CONCLUSIONS: Twenty-two percent of children with acute AIS present with seizures. Seizures were always accompanied by focal neurologic deficits. Younger age was a risk factor for seizures at presentation. Seizure at presentation was not associated with infarct location or etiology. Nonconvulsive seizures may occur during the acute period.

Interrater reliability of the Pediatric National Institutes of Health Stroke Scale (PedNIHSS) in a multicenter study.

BACKGROUND AND PURPOSE: Stroke is an important cause of death and disability among children. Clinical trials for childhood stroke require a valid and reliable acute clinical stroke scale. We evaluated interrater reliability (IRR) of a pediatric adaptation of the National Institutes of Health Stroke Scale. METHODS: The pediatric adaptation of the National Institutes of Health Stroke Scale was developed by pediatric and adult stroke experts by modifying each item of the adult National Institutes of Health Stroke Scale for children, retaining all examination items and scoring ranges of the National Institutes of Health Stroke Scale. Children 2 to 18 years of age with acute arterial ischemic stroke were enrolled in a prospective cohort study from 15 North American sites from January 2007 to October 2009. Examiners were child neurologists certified in the adult National Institutes of Health Stroke Scale. Each subject was examined daily for 7 days or until discharge. A subset of patients at 3 sites was scored simultaneously and independently by 2 study neurologists. RESULTS: IRR testing was performed in 25 of 113 a median of 3 days (interquartile range, 2 to 4 days) after symptom onset. Patient demographics, total initial pediatric adaptation of the National Institutes of Health Stroke Scale scores, risk factors, and infarct characteristics in the IRR subset were similar to the non-IRR subset. The 2 raters' total scores were identical in 60% and within 1 point in 84%. IRR was excellent as measured by concordance correlation coefficient of 0.97 (95% CI, 0.94 to 0.99); intraclass correlation coefficient of 0.99 (95% CI, 0.97 to 0.99); precision measured by Pearson ρ of 0.97; and accuracy measured by the bias correction factor of 1.0. CONCLUSIONS: There was excellent IRR of the pediatric adaptation of the National Institutes of Health Stroke Scale in a multicenter prospective cohort performed by trained child neurologists.

Hemorrhagic transformation of childhood arterial ischemic stroke.

BACKGROUND AND PURPOSE: The objective of this study was to describe the occurrence of hemorrhagic transformation (HT) among children with arterial ischemic stroke within 30 days after symptom onset and to describe clinical factors associated with HT. METHODS: Sixty-three children aged 1 month to 18 years with arterial ischemic stroke between January 2005 and November 2008 were identified from a single-center prospective pediatric stroke registry. All neuroimaging studies within 30 days of stroke were reviewed by a study neuroradiologist. Hemorrhage was classified according to the European Cooperative Acute Stroke Study-1 definitions. Association of HT with clinical factors, systemic anticoagulation, stroke volume, and outcome was analyzed. RESULTS: HT occurred in 19 of 63 children (30%; 95% CI, 19% to 43%), only 2 (3%) of whom were symptomatic. Hemorrhage classification was hemorrhagic infarction (HI)1 in 14, HI2 in 2, parenchymal hematoma (PH)1 in 2, and PH2 in 1. HT was less common in children with vasculopathy (relative risk, 0.27; 95% CI, 0.07 to 1.06; P=0.04) than in those with other stroke mechanisms. HT was not significantly associated with anticoagulation versus antiplatelet therapy (relative risk, 0.6; 95% CI, 0.2 to 1.5; P=0.26) but was associated with larger infarct volumes (P=0.0084). In multivariable analysis, worse Pediatric Stroke Outcome Measure scores were associated with infarct volume ≥5% of total supratentorial brain volume (OR, 4.0; 95% CI, 1.1 to 15; P=0.04), and a trend existed toward association of worse Pediatric Stroke Outcome Measure scores with HT (OR, 4.0; 95% CI, 0.9 to 18; P=0.07). CONCLUSIONS: HT occurred in 30% of children with arterial ischemic stroke within 30 days. Most hemorrhages were petechial and asymptomatic. Infarct volume was associated with HT and worse outcome.

Outcome following decompressive craniectomy for malignant middle cerebral artery infarction in children.

AIM: Mortality from malignant middle cerebral artery infarction (MMCAI) approaches 80% in adult series. Although decompressive craniectomy decreases mortality and leads to an acceptable outcome in selected adult patients, there are few data on MMCAI in children with stroke. This study evaluated the frequency of MMCAI and the use of decompressive craniectomy in children. METHOD: We retrospectively reviewed cases of MMCAI from five pediatric tertiary care centers. RESULTS: Ten children (two females, eight males; median age 9y 10mo, range 22mo-14y) had MMCAI, with a median Glasgow Coma Scale score of 6 (range 3-9). MMCAI represented fewer than 2% of cases of pediatric arterial ischemic stroke. Three patients who did not undergo decompression, all of whom had monitoring of intracranial pressure, developed intractable intracranial hypertension, and fulfilled criteria for brain death. In contrast, seven patients underwent decompressive craniectomy and survived, with rapid improvement in their level of consciousness postoperatively. All seven survivors now walk independently with mild to moderate residual hemiparesis and speak fluently, even though four had left-sided infarcts. INTERPRETATION: Decompressive craniectomy can lead to a moderately good outcome for children with MMCAI and should be considered, even with symptomatic stroke and deep coma. Monitoring of intracranial pressure may delay life-saving treatment.