Outcome at age 7 of epilepsy presenting in the first 2 years of life. A population-based study.

Objective: Existing data suggest that epilepsy presenting in the first few years of life carries a worse prognosis than later onset. However, studies are few and methods differ, making interpretations of data uncertain. This study analyzes outcome at age 7 and potential prognostic factors in a well-characterized population-based cohort with epilepsy onset during the first 2 years of life.

Childhood-onset seizures: A long-term cohort study of use of antiepileptic drugs, and drugs for neuropsychiatric conditions.

Objective: We conducted a long-term follow-up of a cohort of children with newly diagnosed unprovoked seizures to assess treatment with antiepileptic drugs (AEDs), neuroleptics, antidepressants and medication for attention deficit hyperactivity disorder (ADHD) with special attention to the impact of comorbidities on the use of such medication.

Methods: Our study cohort comprised 769 children (28 days-18 years), living in Stockholm Sweden, with a first unprovoked seizure identified between 2001 and 2006. Information on neurodevelopmental comorbidities and Cerebral Palsy (CP) at seizure onset was collected from medical records.

Epilepsy syndromes, etiologies, and the use of next-generation sequencing in epilepsy presenting in the first 2 years of life: A population-based study.

Objective: Population-based data on epilepsy syndromes and etiologies in early onset epilepsy are scarce. The use of next-generation sequencing (NGS) has hitherto not been reported in this context. The aim of this study is to describe children with epilepsy onset before 2 years of age, and to explore to what degree whole exome and whole genome sequencing (WES/WGS) can help reveal a molecular genetic diagnosis.

Neurodevelopmental comorbidities and seizure control 24 months after a first unprovoked seizure in children.

Purpose: To follow children with newly diagnosed unprovoked seizures to determine (1) whether the prevalence of neurodevelopmental comorbidities and cerebral palsy (CP) changed after the initial seizure, and (2) the association between studied comorbidities and seizures 13-24 months after seizure onset or initiation of treatment.

Methods: Analyses were based on 750 children (28 days-18 years) with a first unprovoked seizure (index) included in a population-based Incidence Registry in Stockholm between 2001 and 2006. The children were followed for two years and their medical records were examined for a priori defined neurodevelopmental/psychiatric comorbidities and CP and seizure frequency.

The incidence of unprovoked seizures and occurrence of neurodevelopmental comorbidities in children at the time of their first epileptic seizure and during the subsequent six months.

Purpose: To evaluate the incidence of unprovoked seizures in children and the prevalence of related neurodevelopmental comorbidities at the time of the presumed first seizure and six months thereafter.

Methods: The medical records of all children (0-18 years of age) seeking medical attention as the result of a first unprovoked seizure between September 1, 2001 and December 31, 2006, and registered in the population-based Stockholm Incidence Registry of Epilepsy (SIRE) were reviewed. Neurodevelopmental comorbidities were evaluated on the basis of the medical records from this first visit and from other healthcare during the following six months.

Copy number variations in children with brain malformations and refractory epilepsy.

Brain malformations are a major cause of therapy-refractory epilepsy as well as neurological and developmental disabilities in children. This study examined the frequency and the nature of copy number variations among children with structural brain malformations and refractory epilepsy. The medical records of all children born between 1990 and 2009 in the epilepsy registry at the Astrid Lindgren's Children's Hospital were reviewed and 86 patients with refractory epilepsy and various brain malformations were identified.

Presurgical language lateralization assessment by fMRI and dichotic listening of pediatric patients with intractable epilepsy.

Objective: The aim of this study was to evaluate the clinical use of a method to assess hemispheric language dominance in pediatric candidates for epilepsy surgery. The method is designed for patients but has previously been evaluated with healthy children.

Methods: Nineteen patients, 8-18 years old, with intractable epilepsy and candidates for epilepsy surgery were assessed.

Effectiveness of the ketogenic diet used to treat resistant childhood epilepsy in Scandinavia.

Background: This Scandinavian collaborative retrospective study of children treated with ketogenic diet (KD) highlights indications and effectiveness over two years follow-up.

Methods: Five centres specialised in KD collected data retrospectively on 315 patients started on KD from 1999 to 2009. Twenty-five patients who stopped the diet within four weeks because of compliance-problems and minor side-effects were excluded.

Growth charts and long-term sequelae in extreme preterm infants--from full-term age to 10 years.

Aim: To describe growth pattern from full-term age to 10 years in infants born before 26 weeks of gestation.

Method: This retrospective longitudinal cohort contained 123 children from Karolinska Hospital, Stockholm, during 1990-2002. Length/height (Ht), weight (Wt) and head circumference (HC) were recorded monthly during the first year, every 3 months until 2 years and yearly thereafter, but HC at 15 months and at median age of 8.

Medical conditions affect the outcome of early intervention in preschool children with autism spectrum disorders.

The aim was to explore the frequency of genetic and other medical conditions, including epilepsy, in a population-based group of 208 preschool children with early diagnosis of Autism spectrum disorders (ASD) and to relate outcome at a 2-year follow-up to the co-existing medical findings. They had all received early intervention. The Vineland Adaptive Behaviour Scales (VABS-II) composite score served as the primary outcome measure.

CSF levels of dopamine and serotonin, but not norepinephrine, metabolites are influenced by the ketogenic diet in children with epilepsy.

The ketogenic diet (KD) is a non-pharmacological treatment of medically refractory epilepsy in children. Its mechanisms of action are still unclear but monoamine neurotransmitters have been proposed to be involved. Norepinephrine, dopamine, and serotonin are known to modulate seizure susceptibility in many animal models.

Circulating proprotein convertase subtilisin kexin type 9 has a diurnal rhythm synchronous with cholesterol synthesis and is reduced by fasting in humans.

Objective: To gain insight into the function of proprotein convertase subtilisin kexin type 9 (PCSK9) in humans by establishing whether circulating levels are influenced by diurnal, dietary, and hormonal changes.

Methods And Results: We monitored circulating PCSK9 in a set of dynamic human experiments and could show that serum PCSK9 levels display a diurnal rhythm that closely parallels that of cholesterol synthesis, measured as serum lathosterol. In contrast to these marked diurnal changes in cholesterol metabolism, serum low-density lipoprotein (LDL) cholesterol levels remained stable during the diurnal cycle.

Postnatal cerebral infection leading to hemiplegic cerebral palsy: functional limitations and disability of 13 children in Sweden.

Purpose: To describe the motor function and disability of children with postnatal cerebral infection leading to hemiplegia, and to determine the severity of their motor disability.

Method: The disablement process was used to describe these children. Participants included 13 children (7 girls and 6 boys, mean age 9.

Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group.

The ketogenic diet (KD) is an established, effective nonpharmacologic treatment for intractable childhood epilepsy. The KD is provided differently throughout the world, with occasionally significant variations in its administration. There exists a need for more standardized protocols and management recommendations for clinical and research use.

Growth dependence on insulin-like growth factor-1 during the ketogenic diet.

Purpose: To examine the influence of the ketogenic diet (KD) on linear growth and insulin-like growth factor I (IGF-I) levels in children with pharmacotherapy-resistant epilepsy.

Methods: A prospective study was designed to evaluate growth, serum IGF-I levels, blood beta-hydroxybutyric acid (beta-OHB), and seizure frequency before and during KD in 22 children (median age 5.5 years).

Newly diagnosed single unprovoked seizures and epilepsy in Stockholm, Sweden: First report from the Stockholm Incidence Registry of Epilepsy (SIRE).

Purpose: To describe and report initial findings of a system for prospective identification and follow-up of patients with newly diagnosed single unprovoked seizures and epilepsy in Stockholm, Sweden, the Stockholm Incidence Registry of Epilepsy (SIRE).

Methods: From September 2001 through August 2004, a surveillance system has been in use to identify incident cases of first unprovoked seizures (neonatal seizures excluded) and epilepsy among residents of Northern Stockholm, an urban area with approximately 998,500 inhabitants. Potential cases are identified through multiple mechanisms: Network of health care professionals, medical record screening in specific hospital units, including outpatient clinics, emergency room services, and review of requests for electroencephalography (EEG) examination.

The circulating metabolic regulator FGF21 is induced by prolonged fasting and PPARalpha activation in man.

FGF21 is a critical metabolic regulator, pivotal for fasting adaptation and directly regulated by PPARalpha in rodents. However, the physiological role of FGF21 in man is not yet defined and was investigated in our study. Serum FGF21 varied 250-fold among 76 healthy individuals and did not relate to age, gender, body mass index (BMI), serum lipids, or plasma glucose.

Polyunsaturated fatty acids and cerebrospinal fluid from children on the ketogenic diet open a voltage-gated K channel: a putative mechanism of antiseizure action.

Purpose: Many children with epilepsy do not satisfactorily respond to conventional pharmacological therapy, but to the ketogenic diet, a high-fat, low-carbohydrate diet. This diet increases the concentrations of ketone bodies and polyunsaturated fatty acids (PUFAs) in cerebrospinal fluid (CSF) and plasma. However, its anticonvulsant mechanism is not known.

Influence of the ketogenic diet on 24-hour electroencephalogram in children with epilepsy.

The ketogenic diet is a therapeutic diet used to treat medically refractory epilepsy in children. It was found to be effective and safe. Apart from a reduced number of seizures, positive cognitive effects were described.

Participation in age-related activities and influence of cultural factors--comments from youth and parents of children with postnatal post infectious hemiplegia in Stockholm, Sweden.

Purpose: To investigate whether children with postnatal post-infectious hemiplegic cerebral palsy, and their parents, felt that participation in activities typical for the child's age and gender was affected. To identify factors, intra- and extra-personal that influenced disability with emphasis on factors related to immigration.

Method: Interviews with six youths and 15 caregivers regarding the child's ability to participate in age-related activities, the consequences of disability in their home country compared to Sweden, and whether immigration influenced being a parent to a disabled child.

Postnatal cerebral infection leading to hemiplegic cerebral palsy: clinical description of 13 children in Stockholm, Sweden.

Purpose: The aim of this research was to estimate the prevalence of postnatal cerebral infection leading to hemiplegic cerebral palsy (CP) in Stockholm County and to describe the motor impairments, associated impairments and involvement of the non hemiplegic side.

Method: Children with hemiplegic CP subsequent to a cerebral infection in the perinatal period up to the age of seven years were identified. The assessments of child psychologists and speech therapists and EEG-studies, CT-scan or MRI of the brain were extracted from the children's files.

Late onset bradyarrhythmia during vagus nerve stimulation.

Vagus nerve stimulation (VNS) is widely used to treat refractory epilepsy. It is usually safe and has few side effects. Cardiac arrhythmia has been reported during lead tests performed during implantation of the device, but never during regular treatment.

Continuous EEG monitoring in a paediatric intensive care unit.

Monitoring with continuous EEG (cEEG) has become a valuable tool in the adult neurointensive care unit. The benefits of cEEG or amplitude-integrated EEG in neonatal intensive care have also been described. The aim of the present study was to describe and evaluate the use of cEEG in a paediatric intensive care unit.