Pediatric Stroke Survivors Research Articles

Exploring the Types of Social Support Exchanged by Survivors of Pediatric Stroke and Their Families in an Online Peer Support Community: Qualitative Thematic Analysis.

Pediatric stroke is relatively rare and underresearched, and there is little awareness of its occurrence in wider society. There is a paucity of literature on the effectiveness of interventions to improve rehabilitation and the services available to survivors. Access to online health communities through the internet may be a means of support for patients with pediatric stroke and their families during recovery; however, little research has been done in this area. This study aims to identify the types of social support provided by an online peer support group to survivors of pediatric stroke and their families. This was a qualitative thematic analysis of posts from a pediatric stroke population on a UK online stroke community active between 2004 and 2011. The population was split into 2 groups based on whether stroke survivors were aged ≤18 years or aged >18 years at the time of posting. The posts were read by 2 authors who used the adapted Social Support Behavior Code to analyze the types of social support exchanged. A total of 52 participants who experienced a pediatric stroke were identified, who posted a total of 425 messages to the community. About 41 survivors were aged ≤18 years at the time of posting and were written about by others (31/35 were mothers), while 11 were aged >18 years and were writing about themselves. Survivors and their families joined together in discussion threads. Support was offered and received by all participants, regardless of age. Of all 425 posts, 193 (45.4%) contained at least 1 instance of social support. All 5 types of social support were identified: informational, emotional, network, esteem support, and tangible aid. Informational and emotional support were most commonly exchanged. Emotional support was offered more often than informational support among participants aged ≤18 years at the time of posting; this finding was reversed in the group aged >18 years. Network support and esteem support were less commonly exchanged. Notably, the access subcategory of network support was not exchanged with the community. Tangible aid was the least commonly offered type of support. The exchanged social support provided insight into rehabilitation interventions and the unmet needs of pediatric stroke survivors. We found evidence of engagement of childhood stroke survivors and their families in an online stroke community, with peer support being exchanged between both long- and short-term survivors of pediatric stroke. Engagement of long-term survivors of pediatric stroke through the online community was key, as they were able to offer informational support from lived experience. Further interventional research is needed to assess health and rehabilitation outcomes from engagement with online support groups. Research is also needed to ensure safe, nurturing online communities.

Abstract TP293: Juvenile Mice Are Susceptible to Infarct-Induced Neurodegeneration That Causes Cognitive Decline

Introduction: Over half of pediatric stroke survivors have permanent neurologic and cognitive deficits. Executive function and processing speed are two domains that are consistently affected on neuropsychiatric testing of pediatric stroke survivors. In adult mice, stroke triggers an immune-mediated, infarct-induced neurodegeneration that causes similar cognitive deficits. However, it is unknown whether similar pathophysiology causes children’s cognitive deficits after stroke. Hypothesis: We hypothesize that infarct-induced neurodegeneration occurs in a mouse model of pediatric arterial ischemic stroke. Methods: Male and female C57BL/6J mice were randomized to stroke or sham surgery at p28 to model stroke in late childhood and compared to adult 6-month-old mice. We used permanent distal MCAO followed by 60 minutes of hypoxia, an established model for focal ischemia that generates a purely cortical stroke. Mice underwent behavioral testing at 1- and 7-weeks post stroke with a standard Barnes Maze protocol used in adult mice, and with a protocol adapted for juveniles by increasing difficulty of the task. We performed immunostaining to quantify acute and chronic neuroinflammation, stroke size, myelination, and atrophy 3 days and 8 weeks after surgery. Results: At 7 weeks post-stroke, stroke mice performed significantly worse on Barnes Maze testing than sham mice (n = 7 stroke, 7 sham, p = 0.0265 2-way ANOVA with repeated measures) on the more difficult but not the standard protocol. Like adult mice, pediatric mice exhibited chronic B- and T- lymphocyte neuroinflammation in the stroke scar at 8 weeks after stroke and activated (CD68+) microglia in the scar and connected regions. Both groups had decreased subcortical myelin staining compared to age-equivalent sham mice. However, this decrease was more dramatic in pediatric mice at 65 ± 14% vs. 27% ± 9% in adults, p < 0.05). Conclusions: In a model of pediatric ischemic stroke, juvenile mice develop chronic neuroinflammation in the stroke and in white matter tracts that are connected to the stroke in conjunction with a delayed cognitive deficit. Our results suggest that infarct-induced neurodegeneration occurs after stroke in juvenile mice and may cause more severe dysmyelination than in adults.

Embracing the experiential knowledge of pediatric stroke survivors.

This commentary is on the original article by Champigny et al. on pages 1357–1365 of this issue.

Abstract TP156: Neurological Outcomes After Perinatal Stroke: A Single Center Cohort Study

Introduction: Neurological outcomes in pediatric stroke survivors have been described in observational studies. The Pediatric Stroke Outcome Measures (PSOM) was the first tool validated in children to describe outcomes specifically in stroke (Kitchen et al, 2012). Patients diagnosed with a perinatal stroke comprised 24% of this cohort, while the remainder of patients had suffered from a childhood stroke. Most patients (~ 65%) had no or mild neurological deficits after 5 years of follow-up, represented with a total PSOM score of 0-1. We examined whether perinatal stroke patients would have similar trajectories to the outcomes previously described in pediatric stroke that occurred beyond the perinatal period. Methods: We conducted a retrospective review of our pediatric stroke database at Massachusetts General Hospital. Perinatal stroke survivors were identified, and data extracted from longitudinal follow-up clinic visits. PSOM scores were acquired at: discharge, visits between 2-4 years, 5-7 years, and beyond 7 years. Results: Patients who had endured a perinatal stroke with subsequent PSOM measures recorded were identified (N= 78). Neuroimaging was obtained in the neonatal period (n = 50) or at initial clinical presentation (n = 28; presumed perinatal stroke). Preliminary analyses show that perinatal stroke patients trended towards having increased PSOM scores over extended follow-up beyond 7 years from the index clinical encounter. Discussion: Interval monitoring will unmask deficits throughout development for perinatal stroke patients and can trend towards accrual of further neurological disability. This is in contrast with prior cohorts of childhood stroke where most patients endured stroke beyond the perinatal period and had total PSOM scores of 0-1 beyond 5 years of follow up. Further analyses are underway to establish effect of stroke mechanism, size, and laterality. Separate analyses to map lesion topography to structural and functional network disruptions that occur after perinatal stroke will be performed. Understanding the relationship between these disruptions and functional outcomes will aid in deficit prediction and trajectory modelling, to guide individualized interventions for the youngest of stroke survivors.

Rehabilitation for Pediatric Stroke in Low and Middle Income Countries: A Focused Review

Rehabilitation for pediatric stroke survivors can maximize a child's potential through each developmental stage of life. Timely diagnosis and referral to a rehabilitation specialist may harness opportunities to maximize brain plasticity, to help children adapt and learn, and to participate in and enjoy daily life to their capability. The aim of this focused review is to explore current rehabilitation models and evidence-based interventions for pediatric stroke survivors in Low- and Middle- Income Countries (LMICs) and to provide recommendations for future research and focused areas of improvement.There are several published pediatric stroke guidelines from the American Heart Association (AHA), Canada, Australia, and the United Kingdom (UK) which provide specific recommendations for rehabilitation, although the suggested intervention and services vary. There are no current guidelines developed in or contextually adapted for LMICs, although the current pediatric stroke guidelines emphasize the need to develop interventions that fit the cultural and environmental contexts. The World Health Organization (WHO) Rehabilitation 2030 initiative acknowledges profound unmet rehabilitation needs around the world, especially in LMICs. According to the WHO, LMICs have less than ten skilled rehabilitation professionals per one million people. Enhancing the understanding of rehabilitation services in LMICs could lead to nationally supported workforce education initiatives targeted to expand the number of locally trained therapy providers. This could improve access to and delivery of quality rehabilitation interventions to pediatric stroke survivors in these settings.

Pediatric unilateral spatial neglect: A systematic review.

Unilateral spatial neglect (USN), an inability to attend to one side of space or one's body, is commonly reported in adult stroke survivors and is associated with poor outcomes. USN has been reported in pediatric survivors of stroke, but its impact is unclear. The purpose of this systematic review was to summarize and evaluate the literature regarding USN in pediatric stroke survivors. PRISMA guidelines, Scopus, CINAHL, PubMed, and other relevant databases were searched with terms including "children", "stroke", and "unilateral neglect", with the ages of participants limited from to birth to 18 years. Data were extracted from studies on the clinical presentation of pediatric USN, the assessment of this condition, treatment options, or USN recovery. A total of 18 articles met inclusion criteria. There were no current prevalence data available. USN presents similarly in children compared to adults. Several different USN assessments were used, however, there were little data regarding treatment options. Furthermore, the data suggest that not all children fully recover. This systematic review reveals a lack of quality research to inform the assessment and treatment of children with USN. Although the literature spans decades, there remain no guidelines for standardized assessment or treatment. Similar to adults, paper-and-pencil testing may be less able to identify USN than functional assessments. It is likely that there are children who struggle with undetected USN-related deficits years after their injuries. With a rise of pediatric stroke survivors, there is a pressing need for clinicians to be educated about USN in children, clinical presentations, assessments, and treatments.

Etiologic Profile of Childhood Stroke from North India: Is It Different from Developed World?

To describe the etiology of childhood arterial-ischemic stroke from a developing country and assess short-term neurologic outcome. Prospective observational study. Consecutive children between the age of >28 days to <12 years, admitted with the diagnosis of arterial-ischemic stroke were enrolled during the study period from January 2017 to December 2018. Short-term neurologic outcome was assessed with Pediatric Cerebral Performance Category (PCPC) scale and Pediatric Stroke Outcome Measure (PSOM). We enrolled 76 children with arterial-ischemic stroke, with a median age of 24 months (interquartile range 12-69), and 43 (57%) were boys. The most common risk factor for childhood arterial-ischemic stroke was arteriopathy in 59 (77%), followed by cardiovascular disorder in 12 (16%) children. Among 59 children with arteriopathy, 32 (42%) had infection-associated arteriopathies, 10 (13%) had mineralizing angiopathy, 10 (13%) had moyamoya disease. Pediatric stroke risk factors were classified according to Pediatric Stroke Classification and CASCADE primary classification. Short-term neurologic outcome was assessed at 3 months in 62 (82%) survivors. Among stroke survivors, 33 (61%) had sensory-motor deficits, and 24 (39%) had severe neurologic disability (PCPC ≥ 4). The presence of fever, encephalopathy, low Glasgow coma score at presentation, seizures, and infection-associated arteriopathy predicted severe neurologic disability at follow-up. The risk factors for pediatric arterial-ischemic stroke are different from developed countries in our cohort. Infection-associated arteriopathies, mineralizing angiopathy, and moyamoya disease are the most common risk factors in our cohort. Two-thirds of pediatric stroke survivors have neurologic disability at short-term follow-up.

Characteristics and functional outcomes of pediatric stroke survivors at a rehabilitation unit in Saudi Arabia

There is a lack of data regarding functional outcomes of comprehensive inpatient rehabilitation (IPR) in children with stroke in Saudi Arabia. The objective of this study was to analyze the effect of IPR on functional outcomes of pediatric stroke survivors. This retrospective cohort study included pediatric stroke survivors (n = 18) admitted to pediatric rehabilitation unit from January 2011 to December 2018. Data were obtained regarding functional status based on WeeFIM at the time of admission and discharge. Functional gain was compared within genders, age-groups, geographical regions, lesion characteristics, location of motor deficits, primary etiology, comorbidities, and risk factors. Improvement in self-care, mobility, and cognition was also compared. Results showed a delayed admission to IPR from the time of stroke; however, a significant gain was observed in overall WeeFIM score (p < 0.001), self-care, and mobility (both p = 0.001) but not in cognition (p = 0.13). WeeFIM gain was significantly better in hemorrhagic stroke survivors than ischemic stroke survivors (p = 0.027). Age-group, gender, geographical region, primary etiology, lesion characteristics, comorbidities, risk factors, and location of motor deficits did not affect the functional improvement. The association analysis of WeeFIM gain with age, duration from onset of stroke to IPR admission, WeeFIM score at admission, and length of stay did not yield any significant value. Self-care domain of WeeFIM had significantly better percentage gain than mobility and cognition (p = 0.01). In conclusion, functional outcomes among our cohort of Saudi pediatric stroke survivors improved significantly after IPR especially in self-care. Hemorrhagic strokes had better functional outcome than ischemic strokes.

Chapter 32 - Ischemic sequelae and other vascular diseases

Although pediatric stroke is associated with higher survival rates compared with adult stroke, a substantial body of evidence indicates significant neuropsychologic morbidity in pediatric stroke survivors. Neuroplasticity does not guarantee good outcome in children. The general trends observed in the literature are reviewed as is the profile observed in common causes of pediatric stroke: congenital heart disease, moyamoya disease, and sickle cell disease. The neuropsychologic profile of pediatric stroke patients is heterogeneous due to the multiplicity of associated causes. Stroke in early infancy and large strokes are associated with cognitive impairment while more limited disorders, such as phasic deficit, are observed in childhood stroke. Executive dysfunction is common in pediatric stroke, but social interaction skills are usually preserved. Congenital heart disease and sickle cell disease are associated with global neuropsychologic dysfunction while cognition is usually preserved in moyamoya. Executive dysregulation is instead more frequently reported in this population. Further study of maladaptive processes after pediatric stroke will allow identification of predictors of functional and neuropsychologic outcomes and permit personalization of care.

Remote poststroke headache in children: Characteristics and association with stroke recurrence.

New-onset headache after stroke is common among adult stroke survivors. However, pediatric data are limited. The primary aim of this study was to investigate the prevalence of new-headache after pediatric ischemic stroke. Secondary outcomes were to describe the characteristics of patients experiencing poststroke headache and the association between poststroke headache and stroke recurrence. We conducted a single-center retrospective study on children aged 30 days to 18 years with a confirmed radiographic diagnosis of arterial ischemic stroke (AIS) from January 1, 2008, to December 31, 2016. Patients were identified from an internal database, with additional data abstracted from the electronic medical record. Poststroke headache (occurring >30 days after stroke) was identified through electronic searches of the medical record and confirmed by chart review. Of 115 patients with confirmed AIS, 41 (36%) experienced poststroke headache, with headache developing a median of 6 months after stroke. Fifty-one percent of patients with poststroke headache presented to the emergency department for headache evaluation; 81% of the patients had an inpatient admission for headache. Older age at stroke (odds ratio [OR] 21.5; p = 0.0001) and arteriopathy (OR 8.65; p = 0.0029) were associated with development of poststroke headache in a multivariable analysis. Seventeen patients (15%) had a recurrent stroke during the study period. Poststroke headache was associated with greater risk for stroke recurrence (p = 0.049). Remote poststroke headache is a common morbidity among pediatric stroke survivors, particularly in older children. Headaches may increase health care utilization, including neuroimaging and hospital admissions. We identified a possible association between poststroke headache and stroke recurrence.

Prolonged or recurrent acute seizures after pediatric arterial ischemic stroke are associated with increasing epilepsy risk.

To determine epilepsy risk factors after pediatric stroke. 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. 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). 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 WP406: The Importance of Pediatric Stroke Family Support

Introduction: Insufficient social support has been linked to increased morbidity and mortality within adult medical conditions, including stroke. There is little evidence about the influence of support groups in pediatric stroke survivors and their families. Hypothesis: We hypothesized that pediatric stroke survivors and their families would report several common psychosocial themes surrounding their recovery, and would derive benefit from a structured support group. Methods: Our pediatric stroke support group - associated with a large, regional, pediatric stroke program - was established in 2011. Qualitative and quantitative data from this group were collected via standardized questionnaires during three support group sessions over a 1 year period (2013-14). Data were clustered by survivor, parent, sibling, and other family members. A concurrent triangulation mixed methods approach was used for analysis with a P-value of &lt; 0.05 considered significant. Results: Participants reported primary themes of fear, anxiety, and frustration about 1) recovery (i.e., future disabilities, regaining milestones); 2) recurrence; 3) limited resources to aid in recovery; 4) medical and family decision-making; and 5) normalcy in the community. Time interval from stroke (r= 0.49) and disability (r=0.71) were inversely correlated with fear, anxiety, and frustration (p&lt;0.01). In addition, perception of sufficient family support (r=0.56) and longer participation in rehabilitation (r=0.63) were emotionally beneficial (p&lt;0.01). All participants reported the importance of the support group; of significance to parents was reassurance and knowledge offered from families with similar experiences. Conclusion: Pediatric stroke not only impacts the patient’s life experience, but that of their family members. The stroke experience is very individualized, but common themes exist. Sharing these experiences is valuable to survivors and their families.

Age at stroke onset influences the clinical outcome and health-related quality of life in pediatric ischemic stroke survivors.

Stroke in children occurs across different phases of brain development. Age at onset may affect outcome and health-related quality of life (HRQL). We evaluated the influence of age at stroke onset on the long-term neurological outcomes and HRQL of pediatric stroke survivors. Children with ischemic stroke were recruited into three groups according to their age at onset of stroke (presumed perinatal, neonatal, and childhood). Neurological outcomes were assessed using the Pediatric Stroke Recovery and Recurrence Questionnaire. HRQL was evaluated using proxy report versions (2-18y) of the Pediatric Quality of Life Inventory (PedsQL 4.0). A χ(2) /Fisher's exact test and multivariable logistic regression analysis was performed for the neurological outcomes. HRQL scores from the different age groups were compared using linear regression. Ninety participants (presumed perinatal stroke, n=31; neonatal stroke, n=36; childhood stroke, n=23) were enrolled. Median age at the onset of stroke was 0.5days and 3.7years in neonatal and childhood participants respectively. Of the three groups, participants with presumed perinatal stroke demonstrated the worst global (p<0.002) and motor (p<0.001) outcomes and the lowest level of independence in daily activities (p<0.001). Parents reported the best global outcome and overall HRQL (p=0.007) after neonatal stroke. The age at stroke onset has important implications regarding long-term clinical outcomes and HRQL for survivors. Individuals with presumed perinatal stroke should be considered at high-risk for poor outcomes.

Costs of pediatric stroke care in the United States: a systematic and contemporary review

A substantial literature exists regarding cost-of-care outcomes in adult stroke, however less is known about pediatric stroke. The objective of this review of the literature was to examine studies of costs associated with pediatric stroke care. Six studies reporting data from individuals who experienced a pediatric stroke were included in the review. Cost data (charges and payments) were generally limited to one year and ranged from approximately US$15,000–140,000 depending upon stroke type. Pediatric stroke is linked to substantial costs but studies primarily emphasize the direct cost of care during the first year post-stroke onset. However, since many pediatric stroke survivors experience normal lifespans, they can also accumulate a significantly greater long term cost of care than strokes that occur in adulthood. Future studies are needed to examine long term direct costs, short and long term indirect costs and other economic outcomes in this population.

Abstract TMP101: Impact of Age at Stroke on Cognitive Outcome in Children: Selective Vulnerability of Younger Children

Background &amp; Purpose: Long term cognitive deficits among pediatric stroke survivors have a significant impact on quality of life and long term functional outcome. The degree to which age at stroke relates to cognitive outcome is not clear, with some literature suggesting that younger age at stroke results in worse cognitive outcome. The present study investigated the impact of age at stroke on cognitive outcome measured by IQ in a pediatric sample with mixed stroke etiology. Methods: Subjects were 47 children (62% male) with mixed stroke etiology including sickle cell disease (23%), cardiac disease (15%), vascular disease (17%), and traumatic injury (11%) who were seen for neuropsychological evaluation as part of long-term clinical care, at which time an IQ score was obtained. Age at stroke, location, and etiology were gathered from records and considered for analysis. Results: Mean IQ for the sample was 83.68, although scores ranged from 45 to 121. There was a significant correlation between IQ and age at stroke (r = .290, p = .048), with younger age at stroke associated with lower IQ. When males and females were studied separately, this correlation was not significant for males (r = .305, p = .106) but was significant for females (r = .612, p = .007). A simultaneous linear regression model including age at stroke, gender, etiology, and stroke location significantly predicted variance in IQ (R = .543, F = 4.382, p = .005). Age at stroke and gender were the only two predictors that significantly contributed to the model. Conclusions: We found a significant correlation between stroke age and IQ, with younger stroke age associated with lower IQ. Results suggest a possible gender difference, with females in our sample more vulnerable to lower IQ as a function of younger age at stroke. Combining stroke age, gender, etiology, and stroke location accounted for a significant amount of IQ variance. Further research is necessary to clarify factors associated with outcome in survivors of pediatric stroke and should include factors such as stroke severity and socioeconomic status. Improved understanding of factors associated with cognitive outcome following pediatric stroke may inform clinical management to hopefully improve cognitive outcomes and quality of life in this population.

Abstract NS15: Behavioral Outcomes in Pediatric Stroke: Emotional Changes and Depression in the Pediatric NIH Stroke Scale Cohort

Background and Purpose: Stroke affects 2-13/100,000 children annually and ranks among the top ten causes of childhood death with long-term deficits in 40-60% of survivors. Parents report behavioral changes, such as short temper, irritability, or symptoms of depression. Using data collected in the multicenter study to validate the Pediatric NIH Stroke Scale, we report the prevalence and factors associated with emotional changes and depression following childhood stroke. Methods: Children 2-18 years old with arterial ischemic stroke were prospectively enrolled over 34 months at 15 sites with daily PedNIH Stroke Scale examination. Outcomes were assessed by Pediatric Stroke Outcome Measure (PSOM) at 3 and 12 months. Parents reported the presence or absence of emotional changes or depression. Hemispheric localization and vascular distribution was determined by MRI. Associations were evaluated by logistic regression. Results: 105 children were enrolled (mean age 10.4y, 63% male). Emotional changes were reported in 39/93 (42%) at 3m and 28/78 (36%) at 12m. Depressive symptoms were reported in 13/93 (14%) and 11/80 (14%) at 3 and 12m, respectively. Among those with data at both timepoints, emotional changes present at 3m resolved at 12m for 9/30 (30%) and 6/41 (15%) of those initially without changes at 3m, developed emotional changes at 12m. Symptoms of depression resolved at 12m in 7/12 (58%) with symptoms at 3m, while 5/60 (8.3%) of those without symptoms at 3m reported depressive symptoms at 12m. In multivariate analyses, only PSOM, and not gender, hemispheric lateralization, vascular distribution, or PedNIHSS were predictive of emotional changes or depressive symptoms. Conclusions: Behavioral changes affect a large proportion of children after stroke. In multivariate analysis, parent-reported emotional changes and depression correlated with physician scored severity (PSOM). Behavioral assessment of pediatric stroke survivors and parental education is needed as children encounter emotional struggles in recovery. These potentially treatable behavioral changes could impede therapy and inhibit maximal recovery.

The Pediatric Stroke Outcome Measure

The Pediatric Stroke Outcome Measure (PSOM) is an objective, disease-specific outcome measure containing 115 test items suitable for newborn to adult ages. The PSOM measures neurological deficit and function across 5 subscales: right sensorimotor, left sensorimotor, language production, language comprehension, and cognitive/behavior yielding a final 10-point deficit score. The goal of this study was to examine PSOM construct validity in measuring neurological outcome in pediatric stroke survivors and interrater reliability (IRR) for both prospective and retrospective scoring. For construct validity, PSOM subscale scores were correlated with scores on standardized neuropsychological measures matched by functional domain. We assessed IRR by comparing same-day "live" PSOM scores from 2 independent raters in 10 children (prospective IRR) and by comparing PSOM scores estimated from medical dictations across 5 raters in another 10 children (retrospective IRR). We analyzed PSOM scores from 203 children with ischemic stroke. PSOM subscales show good construct validity (ρ=0.2-0.4; P<0.05). PSOM subscale scores of normal/abnormal demonstrate strong agreement for domain-matched neuropsychology scores (alternative chance-corrected statistic=0.4-0.8). IRR was excellent with the 2 prospective raters' scores in almost perfect agreement (intraclass correlation coefficient, 0.93; 95% CI, 0.76-0.98). Retrospective IRR demonstrated strong agreement with an intraclass correlation coefficient of 0.77 (95% CI, 0.56-0.92). The PSOM is a valid and reliable outcome measure for pediatric stroke. It is useful for retrospective scoring from health records and prospective serial longitudinal outcome assessments and is ideally suited for prospective clinical trials in pediatric stroke.

Health‐related quality of life in children and adolescents with stroke, self‐reports, and parent/proxies reports: Cross‐sectional investigation

Limited data are available on health-related quality of life (HR-QoL) in pediatric stroke survivors. The aim of the present study was to assess HR-QoL by self-assessment and parent/proxy-assessment in children and adolescents who survived a first stroke episode. We investigated HR-QoL in pediatric stroke survivors (71 preschool children [G1] and 62 school children/adolescents [G2]) and in 169 healthy controls. HR-QoL was assessed in patients and parents/proxies with the generic KINDL-R questionnaire exploring overall well-being and 6 well-being subdimensions (physical, psychological, self-esteem, family-related, friend-related, and school-related). In pediatric stroke survivors the neurological long-term outcome was measured with the standardized Pediatric Stroke Outcome Measure. Of stroke survivors, 65% exhibited at least 1 neurologic disability. Pediatric stroke survivors reported lower overall well-being compared with healthy controls. In G2 stroke patients, friend-related well-being respectively emotional well-being was significantly reduced compared with healthy controls (73.0 vs 85.0 points; p < 0.001 respectively 80.2 vs 84.5 points; p = 0.049). Parents/proxies of both stroke survivors rated the overall well-being and all subdimensions (except family-related and school-related well-being in G1 and G2 stroke survivors and physical functioning in G2 stroke survivors) lower compared with parents/proxies of healthy children/adolescents. Overall well-being was significantly reduced in children with moderate/severe neurological deficits compared with normal/mildly affected patients (75.5 vs 83.3 points, p = 0.01). Neonatal stroke survivors reported a significantly better neurological long-term outcome compared to childhood stroke survivors (82.0 vs 75.0 points; p = 0.005). Pediatric stroke survivors compared with healthy controls are strongly affected regarding their overall well-being and older children/adolescents regarding their well-being with peers.

P01-328 - Health-related quality of life in children and adolescents with stroke, self-report and parent/proxy report: Cross sectional investigation

BackgroundAim of the present study was to assess health-related quality of life (HR-QoL) by self and proxy-parent assessment in children and adolescents who survived a first stroke episode.MethodsWe investigated HR-QoL in 133 pediatric stroke survivors (71 preschool children (G1) aged 4 to &lt; 8 years and 62 school children/adolescents (G2) aged 8 to 21 years) and in 169 healthy controls aged 4 – 16 years. HR-QoL was assessed with the generic KINDL-R questionnaire exploring overall well-being and 6 sub-dimensions (physical well-being, psychological well-being, self-esteem, family-related well-being, friend-related well-being, and school-related well-being. Proxy-parent reports explored overall well-being and sub-dimensions. Results were compared within groups between cases and controls. In pediatric stroke survivors the neurological long-term outcome was measured with the standardized Pediatric Stroke Outcome Measure.Results65% of stroke survivors exhibited at least one motor-sensor/cognitive disability. G1 and G2 stroke survivors reported lower overall well-being compared with healthy controls. In G2 stroke survivors, friend-related well-being was significantly reduced compared with healthy controls, 85.0 vs. 73.0 points, p &lt; 0.001. Parents/proxys of both G1 and G2 stroke survivors rated the overall well-being and all sub-dimensions (except family-related and school-related well-being and in G1 stroke survivors physical functioning) lower compared with parent/proxys of healthy children/adolescents.InpretationOur results suggest that the KINDL-R questionnaire is a useful tool in the assessment of HR-QoL in pediatric stroke survivors. Compared with healthy controls, all pediatric/adolescent stroke survivors are strongly affected regarding their overall well-being and older children/adolescents regarding their well-being with peers.

Impact of Reduced Von Willebrand Factor-Cleaving Protease [ADAMTS13] In Pediatric Stroke: A Nested Case-Control Study

AbstractAbstract 805 Background:The interaction of von Willebrand factor [VWF] and its cleaving protease ADAMTS13 for preventing thrombosis in the microvasculature has been well described in patients with thrombotic thrombocytopenic purpura [TTP]. Recently it has been hypothesized that ADAMTS13 may protect the brain from ischemia by regulating VWF-platelet interaction after reperfusion, and that lower levels are associated with cardiovascular disease in young adults. Pediatric stroke is known as a multifactorial disease: Apart from various underlying mechanisms inherited genetic traits and intra-arterial thrombus formation constitute as major risk factors. The aim of the present nested case-control study was to determine the role of reduced ADAMTS13 levels in pediatric stroke. Methods:208 pediatric patients with confirmed stroke (6 months to 18 years) and 128 population-based control children were investigated. Patients with liver disease, sepsis or antiphospholipid antibodies were excluded. ADAMTS13 activity [AC] and antigen [Ag] levels [kit: Technoclone, Vienna, Austria] have been evaluated 6 to 12 months after first stroke onset. Non-parametric statistics was performed for descriptive analysis [median (min-max) values] and inter-group comparisons [Mann-Whitney U test]. Correlation analysis was performed using Spearman rank correlation analysis. A p-value <0.05 was considered as statistically significant. First: Age-dependent percentiles were calculated from the control cohort and the number of patients and controls <10th percentiles were compared to those >10th percentiles. Second: ADAMTS13 levels were distributed into quartiles. The number of patients versus controls in the lowest quartile [1st: 16–86%] was compared to the 2nd [87-101%], 3rd [102-112%] and 4th [113-156%] quartile using a multivariate statistical model [logistic regression] adjusted for VWF, factor VIIIC, blood group [BG] and age. Results are shown as odds ratios/95% confidence intervals [OR/CI]. Results:ADAMTS13 AC levels beyond the acute stroke onset were significantly lower in patients compared to controls [98% (16-143) vs. 103% (60-156), p=0.03], and 46 of 208 patients [22%] showed values <10th percentiles compared with 5 of 123 controls [4%: p<0.001]. The corresponding adjusted OR/CI was 7.13/2.7-19.0. In the quartile comparison adjusted for VWF, FVIIIC, BG and age the following OR/CIs were calculated: 1st vs. 2nd [2.17/1.04-4.53], 1st vs. 3rd [2.35/1.11-4.94] and 1st vs. 4th [2.45/1.15-5.22]. In addition, moderate positive correlations were found between ADAMTS13 AC and i) ADAMTS13 Ag [rho=0.30; p<0.001], ii), BG [rho=0.18; p=0.002] and iii) age [rho=0.16; p=0.004], but not between ADAMTS13 and VWF, or FVIIIC. VWF AG levels were higher in patients compared to controls [112 (34-453) vs.104% (50-451)] without reaching statistical significance [p=0.65]. Platelet count in patients and controls was within the normal age-dependent reference range. Conclusions:Compared to healthy control children pediatric stroke survivors showed significantly reduced ADAMTS13 activities with a corresponding OR of 7.13 [percentile comparison] and 2.3 [quartile comparison]. Our findings support the concept that ADAMTS13 deficiency is associated in the pathogenesis of non TTP-dependent microangiopathy in cerebro- and cardiovascular diseases. To elucidate if this deficiency state in children with stroke beyond the acute disease onset is congenital or acquired further investigations are ongoing. Disclosures:No relevant conflicts of interest to declare.