Child-to-adult transition: a survey of current practices within the European Reference Network for Rare Neurological Diseases (ERN-RND).

BACKGROUND: Transition from child-centered to adult-centered healthcare is a gradual process that addresses the medical, psychological, and educational needs of young people in the management of their autonomy in making decisions about their health and their future clinical assistance. This transfer is challenging across all chronic diseases but can be particularly arduous in rare neurological conditions. AIM: To describe the current practice on the transition process for young patients in centers participating in the European Reference Network for Rare Neurological Diseases (ERN-RND). METHODS: Members of the ERN-RND working group developed a questionnaire considering child-to-adult transition issues and procedures in current clinical practice. The questionnaire included 20 questions and was sent to members of the health care providers (HCPs) participating in the network. RESULTS: Twenty ERN-RND members (75% adult neurologists; 25% pediatricians; 5% nurses or study coordinators) responded to the survey, representing 10 European countries. Transition usually occurs between 16 and 18 years of age, but 55% of pediatric HCPs continue to care for their patients until they reach 40 years of age or older. In 5/20 ERN-RND centers, a standardized procedure managing transition is currently adopted, whereas in the remaining centers, the transition from youth to adult service is usually assisted by pediatricians as part of their clinical practice. CONCLUSIONS: This survey demonstrated significant variations in clinical practice between different centers within the ERN-RND network. It provided valuable data on existing transition programs and highlighted key challenges in managing transitions for patients with rare neurological disorders.

Positive outcome of visuospatial deficit rehabilitation in children with epilepsy using computer-based FORAMENRehab program.

Children with epilepsy often show deficits in attention and visuospatial functions. Still, very few systematically controlled evidence-based pediatric neurorehabilitation methods exist. The aim was to assess the effectiveness of a computer-based rehabilitation program for visuospatial deficit remediation in children with epilepsy. Fifty-eight children aged 8-12 years participated: 17 children with epilepsy diagnosis in intervention group, 22 patients in waiting-list control group, and 19 healthy age equivalent controls. The intervention group received guided visuospatial functions rehabilitation with FORAMENRehab software twice a week for a 5-week period. Baseline assessments were carried out before and immediately after the intervention period, and as follow-up 1.31 years later. Intervention group showed positive immediate rehabilitation effect in 3 out of 4 visuospatial components: visual organization, visual attention, and visuospatial perception. A long-term rehabilitation effect in the study group was observed in all 4 of the trained components. Also, a positive generalized effect was confirmed by the parents' and children's qualitative feedback with some of the improved skills transferring to children's everyday life. One hundred percent compliance further confirmed the children's motivation to participate and the effectiveness of FORAMENRehab for pediatric neurorehabilitation.

Neurorehabilitation with FORAMENRehab for attention impairment in children with epilepsy.

Epilepsy is a frequent neurological disorder in children and often accompanied with attention impairment. Still, few systematically controlled rehabilitation techniques for children exist. The aim of this study was to design and measure the impact of the FORAMENRehab computer-based intervention method for attention impairment rehabilitation in children with epilepsy. We chose the FORAMENRehab program because it allows separate training for different attention components based on individual needs. Forty-eight children participated in the study. At baseline, all patients underwent neuropsychological examination of attention with the NEPSY test battery. The study group consisted of 17 8- to 12-year-old children with partial epilepsy and attention impairment who received neurorehabilitation over 5weeks (10 sessions) with FORAMENRehab Attention module accompanied by a therapist. Two control groups were included: the first control group of 12 children with partial epilepsy and attention impairment (waiting-list) participated in assessments with baseline tasks before and after the five-week period and received no active training. Additionally, all patients participated in the follow-up assessment 1.31years later. The second control group consisted of 19 typically developing children who only participated in the first assessment. After the intervention, study group patients showed significant improvement in complex attention and tracking (P<0.025). To achieve the effect of intervention in children with partial epilepsy, 10 sessions tailored to individual levels of ability were the minimum. Three attention components - sustained, complex, and tracking - need selective and longer training for more effective remediation. Follow-up assessment revealed a long-term positive effect of intervention. After 1.31years, the study group had significantly improved in three out of the four attention components (P<0.025), whereas the waiting-list group showed improvement in only two aspects of one complex attention component. In conclusion, attention impairment rehabilitation with FORAMENRehab is effective for children with epilepsy. Rehabilitation should focus on training specific components of attention and follow an individual-based rehabilitation process.

Resting-State Functional Connectivity and Cognitive Impairment in Children with Perinatal Stroke.

Perinatal stroke is a leading cause of congenital hemiparesis and neurocognitive deficits in children. Dysfunctions in the large-scale resting-state functional networks may underlie cognitive and behavioral disability in these children. We studied resting-state functional connectivity in patients with perinatal stroke collected from the Estonian Pediatric Stroke Database. Neurodevelopment of children was assessed by the Pediatric Stroke Outcome Measurement and the Kaufman Assessment Battery. The study included 36 children (age range 7.6-17.9 years): 10 with periventricular venous infarction (PVI), 7 with arterial ischemic stroke (AIS), and 19 controls. There were no differences in severity of hemiparesis between the PVI and AIS groups. A significant increase in default mode network connectivity (FDR 0.1) and lower cognitive functions (p < 0.05) were found in children with AIS compared to the controls and the PVI group. The children with PVI had no significant differences in the resting-state networks compared to the controls and their cognitive functions were normal. Our findings demonstrate impairment in cognitive functions and neural network profile in hemiparetic children with AIS compared to children with PVI and controls. Changes in the resting-state networks found in children with AIS could possibly serve as the underlying derangements of cognitive brain functions in these children.

Stroke Prevalence, Mortality and Disability-Adjusted Life Years in Children and Youth Aged 0-19 Years: Data from the Global and Regional Burden of Stroke 2013.

BACKGROUND: There is increasing recognition of stroke as an important contributor to childhood morbidity and mortality. Current estimates of global childhood stroke burden and its temporal trends are sparse. Accurate and up-to-date estimates of childhood stroke burden are important for planning research and the resulting evidence-based strategies for stroke prevention and management. OBJECTIVES: To estimate the prevalence, mortality and disability-adjusted life years (DALYs) for ischemic stroke (IS), hemorrhagic stroke (HS) and all stroke types combined globally from 1990 to 2013. METHODOLOGY: Stroke prevalence, mortality and DALYs were estimated using the Global Burden of Disease 2013 methods. All available data on stroke-related incidence, prevalence, excess mortality and deaths were collected. Statistical models and country-level covariates were employed to produce comprehensive and consistent estimates of prevalence and mortality. Stroke-specific disability weights were used to estimate years lived with disability and DALYs. Means and 95% uncertainty intervals (UIs) were calculated for prevalence, mortality and DALYs. The median of the percent change and 95% UI were determined for the period from 1990 to 2013. RESULTS: In 2013, there were 97,792 (95% UI 90,564-106,016) prevalent cases of childhood IS and 67,621 (95% UI 62,899-72,214) prevalent cases of childhood HS, reflecting an increase of approximately 35% in the absolute numbers of prevalent childhood strokes since 1990. There were 33,069 (95% UI 28,627-38,998) deaths and 2,615,118 (95% UI 2,265,801-3,090,822) DALYs due to childhood stroke in 2013 globally, reflecting an approximately 200% decrease in the absolute numbers of death and DALYs in childhood stroke since 1990. Between 1990 and 2013, there were significant increases in the global prevalence rates of childhood IS, as well as significant decreases in the global death rate and DALYs rate of all strokes in those of age 0-19 years. While prevalence rates for childhood IS and HS decreased significantly in developed countries, a decline was seen only in HS, with no change in prevalence rates of IS, in developing countries. The childhood stroke DALY rates in 2013 were 13.3 (95% UI 10.6-17.1) for IS and 92.7 (95% UI 80.5-109.7) for HS per 100,000. While the prevalence of childhood IS compared to childhood HS was similar globally, the death rate and DALY rate of HS was 6- to 7-fold higher than that of IS. In 2013, the prevalence rate of both childhood IS and HS was significantly higher in developed countries than in developing countries. Conversely, both death and DALY rates for all stroke types were significantly lower in developed countries than in developing countries in 2013. Men showed a trend toward higher childhood stroke death rates (1.5 (1.3-1.8) per 100,000) than women (1.1 (0.9-1.5) per 100,000) and higher childhood stroke DALY rates (120.1 (100.8-143.4) per 100,000) than women (90.9 (74.6-122.4) per 100,000) globally in 2013. CONCLUSIONS: Globally, between 1990 and 2013, there was a significant increase in the absolute number of prevalent childhood strokes, while absolute numbers and rates of both deaths and DALYs declined significantly. The gap in childhood stroke burden between developed and developing countries is closing; however, in 2013, childhood stroke burden in terms of absolute numbers of prevalent strokes, deaths and DALYs remained much higher in developing countries. There is an urgent need to address these disparities with both global and country-level initiatives targeting prevention as well as improved access to acute and chronic stroke care.

Relationship between social competence and neurocognitive performance in children with epilepsy.

Epilepsy may affect a child's social skills and social cognition. The purpose of the study was to examine associations between sociocognitive skills and neurocognitive performance in children with epilepsy. Thirty-five children with epilepsy between the ages of 7 and 12 years (25 with partial and 10 with generalized epilepsy) and 30 controls participated. Theory of Mind (ToM) tasks, Social Cognition Questionnaire proposed by Saltzman-Benaiah and Lalonde (2007), and Social Skills Rating System were used to assess social competence and sociocognitive skills. Neurocognitive performance was assessed using the NEPSY battery. Children with epilepsy demonstrated more difficulties in understanding false belief (p<.001) and intentional lying (p<.05) and exhibited more behavioral problems (p<.05). Notably, their social skills were at the same level as typically developing peers. Children with epilepsy performed significantly worse in attention, executive, verbal, and fine motor tasks (p<.05). We found positive correlations between the understanding of false belief and in executive (r=.6, p<.05), verbal (r=.45-.49, p<.05), and visuospatial skills (r=.34-.48, p<0.01). Children with generalized epilepsy had more problems in memory tasks (p<.05) and understanding of sarcasm (p<.05) compared with children with partial epilepsy. An age of onset over 9.1 years was positively associated with ToM skills (r=.42, p<.05). In conclusion, better ToM in children with better executive functions, and language and visuospatial skills was revealed. The type of epilepsy and age of onset significantly affected ToM skills.

Copy number variations and cognitive phenotypes in unselected populations.

IMPORTANCE: The association of copy number variations (CNVs), differing numbers of copies of genetic sequence at locations in the genome, with phenotypes such as intellectual disability has been almost exclusively evaluated using clinically ascertained cohorts. The contribution of these genetic variants to cognitive phenotypes in the general population remains unclear. OBJECTIVE: To investigate the clinical features conferred by CNVs associated with known syndromes in adult carriers without clinical preselection and to assess the genome-wide consequences of rare CNVs (frequency ≤0.05%; size ≥250 kilobase pairs [kb]) on carriers' educational attainment and intellectual disability prevalence in the general population. DESIGN, SETTING, AND PARTICIPANTS: The population biobank of Estonia contains 52,000 participants enrolled from 2002 through 2010. General practitioners examined participants and filled out a questionnaire of health- and lifestyle-related questions, as well as reported diagnoses. Copy number variant analysis was conducted on a random sample of 7877 individuals and genotype-phenotype associations with education and disease traits were evaluated. Our results were replicated on a high-functioning group of 993 Estonians and 3 geographically distinct populations in the United Kingdom, the United States, and Italy. MAIN OUTCOMES AND MEASURES: Phenotypes of genomic disorders in the general population, prevalence of autosomal CNVs, and association of these variants with educational attainment (from less than primary school through scientific degree) and prevalence of intellectual disability. RESULTS: Of the 7877 in the Estonian cohort, we identified 56 carriers of CNVs associated with known syndromes. Their phenotypes, including cognitive and psychiatric problems, epilepsy, neuropathies, obesity, and congenital malformations are similar to those described for carriers of identical rearrangements ascertained in clinical cohorts. A genome-wide evaluation of rare autosomal CNVs (frequency, ≤0.05%; ≥250 kb) identified 831 carriers (10.5%) of the screened general population. Eleven of 216 (5.1%) carriers of a deletion of at least 250 kb (odds ratio [OR], 3.16; 95% CI, 1.51-5.98; P = 1.5e-03) and 6 of 102 (5.9%) carriers of a duplication of at least 1 Mb (OR, 3.67; 95% CI, 1.29-8.54; P = .008) had an intellectual disability compared with 114 of 6819 (1.7%) in the Estonian cohort. The mean education attainment was 3.81 (P = 1.06e-04) among 248 (≥250 kb) deletion carriers and 3.69 (P = 5.024e-05) among 115 duplication carriers (≥1 Mb). Of the deletion carriers, 33.5% did not graduate from high school (OR, 1.48; 95% CI, 1.12-1.95; P = .005) and 39.1% of duplication carriers did not graduate high school (OR, 1.89; 95% CI, 1.27-2.8; P = 1.6e-03). Evidence for an association between rare CNVs and lower educational attainment was supported by analyses of cohorts of adults from Italy and the United States and adolescents from the United Kingdom. CONCLUSIONS AND RELEVANCE: Known pathogenic CNVs in unselected, but assumed to be healthy, adult populations may be associated with unrecognized clinical sequelae. Additionally, individually rare but collectively common intermediate-size CNVs may be negatively associated with educational attainment. Replication of these findings in additional population groups is warranted given the potential implications of this observation for genomics research, clinical care, and public health.

Follow-up study of 2-year-olds born at very low gestational age in Estonia.

AIM: To study very low gestational age (VLGA, <32 weeks) infants at 2 years of age and to identify the predictors of adverse outcomes. METHODS: A population-based cohort of 155 surviving VLGA infants born in Estonia in 2007 was followed up and compared with a matched full-term (FT) control group. A logistic regression model was used to test associations between risk factors and adverse outcomes. RESULTS: No impairment was found in 60% of the VLGA infants. Neurodevelopmental impairment was noted in 12% of VLGA infants, with 8% of the infants affected by cerebral palsy without independent walking, 5% with cognitive delay, 10% with language delay and 1% with hearing impairment. The differences between preterm and FT infants in terms of the mean Cognitive, Language, and Motor Composite Scores assessed using the Bayley-III scales were in excess of 0.5 SD. Somatic growth delay was a significant problem among preterm infants. The existence of severe neonatal cerebral lesions was the most significant predictor of adverse outcomes. CONCLUSION: In all domains studied, adverse conditions were more prevalent among VLGA infants than among the FT control group. Efforts to reduce neonatal morbidity in preterm infants should be a key priority for health care in Estonia.

Power of combined modern technology: Multitouch-multiuser tabletops and virtual reality platforms (PowerVR) in social communication skills training for children with neurological disorders: A pilot study.

New technologies such as multitouch-multiuser tabletops (MMT) and virtual reality (VR) provide modern neurorehabilitation options. The aim was to describe the structure of acquired social communication deficits in children with neurological disorders (ND) and implement a new PowerVR method- combined MMT/VR interfaces, into social neurorehabilitation. The study was designed based on the Structured Social Rehabilitation Model. Sixty children with ND aged 8-13 years participated: 28 with epilepsy, 10 with traumatic brain injury (TBI), 3 with tic disorders, 3 with stroke, 16 with other ND. A total of 16 patients (M = 10.5 years, SD = 1.8) completed trainings with pre- and post-intervention assessments. Forty-four patients participated in base-level assessment (M = 10.2 years, SD = 1.6). Two age-matched patients practiced in pairs on MMT and individually in VR. The most impaired components in patients were theory of mind (ToM) skills. A total of 64% of children presented behavioral problems related to executive dysfunctions. Patients lacked conflict resolution (median 38% out of 100%) and empathy skills (25%). After trainings, communication and cooperation, pragmatics, social attention, conflict resolution, and empathy skills improved. Patients' general verbal ToM and understanding false beliefs (p < .005) increased. Children's ability to start conversations improved; they experienced less bullying. Findings suggest that the combined technology-based intervention PowerVR offers increased power for multicomponent training of socio-emotional skills.

Lessons learned during the process of reporting individual genomic results to participants of a population-based biobank.

The return of individual genomic results (ROR) to research participants is still in its early phase, and insight on how individuals respond to ROR is scarce. Studies contributing to the evidence base for best practices are crucial before these can be established. Here, we describe a ROR procedure conducted at a population-based biobank, followed by surveying the responses of almost 3000 participants to a range of results, and discuss lessons learned from the process, with the aim of facilitating large-scale expansion. Overall, participants perceived the information that they received with counseling as valuable, even when the reporting of high risks initially caused worry. The face-to-face delivery of results limited the number of participants who received results. Although the participants highly valued this type of communication, additional means of communication need to be considered to improve the feasibility of large-scale ROR. The feedback collected sheds light on the value judgements of the participants and on potential responses to the receipt of genetic risk information. Biobanks in other countries are planning or conducting similar projects, and the sharing of lessons learned may provide valuable insight and aid such endeavors.

Social-emotional behaviour in infants and toddlers with mild traumatic brain injury.

BACKGROUND: Paediatric head trauma is a serious health concern often leading to neurological, behavioural and cognitive impairments. In Estonia head trauma incidence is especially high in children up to 4 years. OBJECTIVE: The aim was to investigate young children's pre-injury social-emotional behaviour to discover potential risk signs for brain trauma and to study social-emotional outcome 9 months post-injury. METHODS: Thirty-five 3-65 month old children with mild traumatic brain injury (MTBI) and 70 matched controls were retrospectively assessed with child monitoring system Ages and Stages Questionnaires: Social-Emotional. During follow-up 27 patients and 54 controls were re-assessed after 9 months. RESULTS: Children with MTBI showed altered social-emotional development already before the injury. Deficits were evident in self-regulation and autonomy. Age-specific social-emotional risk signs for MTBI were found. Compared to controls more pre-injury affective problems were seen in 12-month olds, self-regulation, and communication difficulties in 30-month olds and autonomy disturbances in 60-month old children. 9 months post-injury impairments in autonomy and self-regulation were still present and new difficulties in interaction had developed. CONCLUSIONS: Childhood MTBI has a serious negative effect on further development of interaction skills. To prevent possible traumas, parents should be informed of different age-specific pre-injury social-emotional risk signs.

Attention profile in children with epilepsy compared to healthy children using computer-based ForamenRehab program.

Attention is a key component in cognitive functioning. Attention impairment is often present in children with epilepsy, which is one of the most common neurological disorders in children and adolescents. Evaluating the attention profiles is important for describing the patients' cognitive weaknesses and planning individual-based cognitive neurorehabilitation. The aim of this study was to assess the attention profiles of children with epilepsy and compare the results to neurologically healthy children. Twenty-nine children with epilepsy aged 8-12 years and 19 neurologically healthy age-matched comparison group participated in assessments with baseline tasks of the Attention module of ForamenRehab computer-program. Four separate attention components were measured - focused, sustained, complex, and tracking - to reveal the most impaired aspects of attention. At the baseline level, patients showed impairments in different attention function components compared to comparison group. Children with epilepsy had significantly lower results in sustained, complex attention, and tracking components (p < 0.05). The patients had similar results with comparison group only in one component - focused attention. All children had quicker visual reaction times compared to their auditory reaction times. The results emphasize the need for attention rehabilitation in children with epilepsy and help to focus on specific components of attention in planning the intervention process.

Structured Model of Neurorehab: A Pilot Study of Modern Multitouch Technology and Virtual Reality Platforms for Training Sociocognitive Deficit in Children with Acquired Brain Injury.

Social competence is often impaired in children with acquired brain injury (ABI), but evidence-based rehabilitation has remained undeveloped. This pilot-study aimed to create a structured model for sociocognitive rehabilitation for children with ABI. A total of 32 children aged 8-13 years participated: 22 with ABI -epilepsy, traumatic brain injury, or tic disorder and 10 healthy controls. Interactive computer-based applications were implemented into rehabilitation design using multitouch-multiuser tabletop (MMT) devices: Snowflake MultiTeach (MT) and Diamond Touch Table (DTT), plus MediqVR virtual reality (VR) platform. At baseline, patients demonstrated social incompetence compared to healthy controls. Post-training evaluations showed that rehabilitation on Snowflake MT improved children's executive and cooperation skills. DTT developed new communication and language skills, metacognitive skills, and coping with difficult social situations. This structured model for social competence rehabilitation helps therapists to understand the objectives and tools for improving social and cooperation skills in children with ABI. We provide practical recommendations using next-generation devices, which are effective and motivating for children.

The incidence of childhood traumatic brain injury in Tartu and Tartu County in Estonia.

BACKGROUND: Traumatic brain injury (TBI) is a major health problem in childhood. Estonia and other Baltic states have the highest trauma-related mortality in the European Union. There are no data on the incidence and causes of TBI for children in Estonia. The aim of this study was to estimate the incidence, structure and main causes of TBI in Tartu and Tartu County. METHODS: The study was carried out at Tartu University Hospital, between January 1, 2001, and December 31, 2005. For inclusion in the study the following criteria had to be fulfilled: age 0-14 years, documented brain trauma and neurological symptoms and residency in Tartu or Tartu County. Over the study period the inclusion criteria were fulfilled in 478 cases [272 boys (57%) and 206 girls (43%)]. RESULTS: The incidence of TBI in childhood in Tartu and Tartu County was 369:100,000 (405:100,000 for boys, 330:100,000 for girls). The incidence was highest among children from 0 to 4 years of age - 566:100,000. The main cause of TBI in all age groups was falling - 63.6%. According to severity, 82% of the cases were mild and 18% were moderate and severe. CONCLUSIONS: There is an urgent need for governmental prevention programs for TBI in children in Estonia.

Long-term neurodevelopmental outcome after perinatal arterial ischemic stroke and periventricular venous infarction.

BACKGROUND: Long-term follow-up data after different vascular types of ischemic perinatal stroke is sparse. Our aim was to study neurodevelopmental outcomes following neonatal and presumed perinatal ischemic middle cerebral artery territory stroke (arterial ischemic stroke, AIS) and periventricular venous infarction (PVI). METHODS: A prospective consecutive cohort of 40 term-born children with perinatal stroke (21 AIS, 19 PVI) was identified through the Estonian Paediatric Stroke Database. While 48% of the children with AIS were diagnosed during the neonatal period, all the children with PVI had presumed perinatal stroke. Outcomes based on the Paediatric Stroke Outcome Measure (PSOM) and Kaufman Assessment Battery for Children - Second Edition (K-ABC-II), in relation to extent and laterality of stroke, were defined. RESULTS: At a median age of 7 years 6 months (range 3.6-13y), there was a trend towards worse neurodevelopmental outcome in participants with AIS when compared to PVI (mean total PSOM scores 3.1 and 2.2, respectively; p = 0.06). Combined deficits of motor, language and cognitive/behavioural functions were significantly more common among children with AIS (90%) when compared to children with PVI (53%, p = 0.007). General cognitive ability (by K-ABC-II) was significantly lower in the AIS subgroup (mean 79.6; 95% CI 72.3-87.0), but children with PVI (91.6; 95% CI 85.5-97.8) also had poorer performance than the age-equivalent normative mean. Large extent of stroke was associated with poorer neurodevelopmental outcome and lower cognitive performance in children following AIS but not in PVI. CONCLUSION: In this national cohort, poor long-term neurodevelopmental outcome after perinatal ischemic stroke was seen irrespective of the vascular type or time of diagnosis of stroke. However, the spectrum of neurological deficits is different after perinatal AIS and PVI, with combined deficits more common among children following AIS.

Epilepsy after perinatal stroke with different vascular subtypes.

OBJECTIVE: With an incidence up to 63 per 100,000 live births, perinatal stroke is an important cause of childhood epilepsy. The aim of the study was to find the prevalence of and predictive factors for epilepsy, and to describe the course of epilepsy in children with perinatal stroke with different vascular subtypes. METHODS: Patients were retrieved from the Estonian Paediatric Stroke Database with follow-up time at least 24 months. Patients were divided into 5 perinatal stroke syndromes: neonatal arterial ischemic stroke (AIS), neonatal hemorrhagic stroke, neonatal cerebral sinovenous thrombosis, presumed AIS, and presumed periventricular venous infarction. RESULTS: The final study group included 73 children with perinatal stroke (39 boys). With a median follow-up time of 8.6 years, epilepsy was diagnosed in 21/73 (29%) children, most of whom had AIS (17/21, 81%). The 18-year cumulative poststroke epilepsy risk according to the Kaplan-Meier estimator was 40.8% (95% confidence interval [CI] 20.7-55.9%). The median age at epilepsy diagnosis was 50 months (range 1 month to 18.4 years). Children with neonatal AIS had the highest risk of epilepsy, but children with presumed AIS more often had severe epilepsy syndromes. Cortical lesions (odds ratio [OR] 19.7, 95% CI 2.9-133), and involvement of thalamus (OR 9.8, 95% CI 1.8-53.5) and temporal lobe (OR 8.3, 95% CI 1.8-39.6) were independently associated with poststroke epilepsy. SIGNIFICANCE: The risk for poststroke epilepsy after perinatal stroke depends on the vascular subtype. Patients with perinatal AIS need close follow-up to detect epilepsy and start with antiepileptic treatment on time.

Incidence of Childhood Epilepsy in Estonia.

The aim of this prospective epidemiological study was to establish the incidence rate of childhood epilepsy in Estonia, to describe the clinical spectrum and to identify etiology of childhood epilepsy. The overall incidence rate was 86.3/100 000. The incidence rate was the highest (141.9/100 000) in the age group from 5 to 9 years. Specific electroclinical syndromes were identified in 22.8% of cases. Structural or metabolic etiology was identified in 20.0% of cases, presumed genetic origin was identified in 33.9% of cases, and in 46.1% of cases the cause of epilepsy remained unknown. The incidence rate of childhood epilepsy in Estonia (86.3/100 000) is similar to the other European countries. In comparison with the results of the first epidemiological study of childhood epilepsy in Estonia (incidence rate 45/100 000; Beilmann et al), the incidence rate in this study is almost 2 times higher, what can be explained with better case collection and improved diagnostic modalities in Estonia.

Mortality After Pediatric Arterial Ischemic Stroke.

OBJECTIVES: Cerebrovascular disease is among the top 10 causes of death in US children, but risk factors for mortality are poorly understood. Within an international registry, we identify predictors of in-hospital mortality after pediatric arterial ischemic stroke (AIS). METHODS: Neonates (0-28 days) and children (29 days-<19 years) with AIS were enrolled from January 2003 to July 2014 in a multinational stroke registry. Death during hospitalization and cause of death were ascertained from medical records. Logistic regression was used to analyze associations between risk factors and in-hospital mortality. RESULTS: Fourteen of 915 neonates (1.5%) and 70 of 2273 children (3.1%) died during hospitalization. Of 48 cases with reported causes of death, 31 (64.6%) were stroke-related, with remaining deaths attributed to medical disease. In multivariable analysis, congenital heart disease (odds ratio [OR]: 3.88; 95% confidence interval [CI]: 1.23-12.29; P = .021), posterior plus anterior circulation stroke (OR: 5.36; 95% CI: 1.70-16.85; P = .004), and stroke presentation without seizures (OR: 3.95; 95% CI: 1.26-12.37; P = .019) were associated with in-hospital mortality for neonates. Hispanic ethnicity (OR: 3.12; 95% CI: 1.56-6.24; P = .001), congenital heart disease (OR: 3.14; 95% CI: 1.75-5.61; P < .001), and posterior plus anterior circulation stroke (OR: 2.71; 95% CI: 1.40-5.25; P = .003) were associated with in-hospital mortality for children. CONCLUSIONS: In-hospital mortality occurred in 2.6% of pediatric AIS cases. Most deaths were attributable to stroke. Risk factors for in-hospital mortality included congenital heart disease and posterior plus anterior circulation stroke. Presentation without seizures and Hispanic ethnicity were also associated with mortality for neonates and children, respectively. Awareness and study of risk factors for mortality represent opportunities to increase survival.

Acutely and retrospectively diagnosed perinatal stroke: a population-based study.

BACKGROUND AND PURPOSE: There are not very many epidemiological studies on perinatal stroke, and many authors suggest that this may be an underdiagnosed condition. The aim of the study was to estimate the incidence of perinatal arterial ischemic and hemorrhagic stroke in Estonia, to study the first clinical signs and to identify possible differences in predisposing factors and outcome between acutely and retrospectively diagnosed cases of perinatal stroke. METHODS: A retro- and prospective study of acutely (within the first month) and retrospectively diagnosed ischemic and hemorrhagic cases of perinatal stroke was conducted in a children population born in the eastern and southern regions of Estonia during the years 1994 to 2003. Patients were identified from a pilot study, hospital records, and an inquiry of child neurologists and general practitioners. The diagnosis was confirmed in 38 (12 were diagnosed acutely and 26 retrospectively) cases by neuroradiology (MRI or CT). RESULTS: The incidence rate of perinatal stroke in Estonia is 63 per 100,000 live births. Main clinical findings in the neonatal period were seizures, abnormalities of muscular tone, and disturbed level of alertness. Previously identified risk factors occurred in 32% of cases. Children with early diagnosis had more often adverse events during pregnancy and delivery (P<0.05) and developed more severe stage of hemiparesis compared with children with late diagnosis (P<0.05). CONCLUSIONS: The incidence rate of 63 per 100,000 live birth is higher than previously reported. Detailed analysis of the first signs of perinatal stroke may improve the early diagnostics of perinatal stroke.

Chronic paroxysmal hemicrania in a 3-year, 10-month-old female.

This is the report of a 3-year, 10-month-old female with classical symptoms of chronic paroxysmal hemicrania and favorable response to indomethacin therapy. The patient was admitted because of frequent episodes of severe unilateral headaches during the day and nighttime as well as agitation. During the episodes, she complained of severe pains on the left orbital and supraorbital region. Subsequent lacrimation from the left eye was also documented. Initially, focal epileptic attack was diagnosed and during the following 10 months several antiepileptic drugs were used without effect. After 10 months, chronic paroxysmal hemicrania was diagnosed because of the typical symptoms along with a favorable response to indomethacin therapy.