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The aim was to determine school performance and psychiatric comorbidity in children with childhood absence epilepsy (CAE). We reviewed the medical records in children with ICD-10 codes for idiopathic generalized epilepsy before 18 years of age, and pediatric neurologists confirmed the International League Against Epilepsy criteria for CAE were met. Control groups were the general pediatric population or children with non-neurological chronic disease. Outcomes were from nationwide and population-based registers on school performance and psychiatric comorbidity. We compared the mean grade point average using linear regression and estimated hazard ratios (HR) using Cox regression for the other outcomes. Analyses were adjusted for the child's sex, and year of birth, and parental highest education, receipt of cash benefits or early disability pension. We included 114 children with CAE with a median age at onset of 5.9 years (interquartile range = 4.5–7.3 years). Compared with both population controls and non-neurological chronically ill children, children with CAE had increased hazard of special needs education (HR = 2.7, 95% confidence interval (CI) = 1.8–4.1, p < 0.0001), lower grade point average at 9th grade by 1.7 grade points (95% CI = -2.5 to -1.0, p < 0.001), increased ADHD medicine use (HR = 4.4, 95% CI = 2.7–7.2, p < 0.001), increased sleep medicine use (HR = 2.7, 95% CI = 1.7–4.3, p < 0.001), and increased psychiatry visits (HR = 2.1, 95% CI = 1.1–4.0, p = 0.03). In conclusion, children with CAE have increased psychiatric comorbidity and a considerable proportion of these children receive special needs education in primary/secondary school, albeit insufficient to normalize their considerably lower grade point average in the 9th grade. Children with childhood absence epilepsy compared with non-neurological chronically ill children (rheumatoid arthritis, inflammatory bowel disease, and type 1 diabetes) have: • increased special needs education (hazard ratio (HR) = 2.7, 95% confidence interval (CI) = 1.8–4.1, p < 0.0001) • lower grade point average in 9th grade by 1.7 points (95% CI = -2.4 to -0.9, p < 0.001) • increased ADHD medicine use (HR = 4.4, 95% CI = 2.7–7.2, p < 0.001) • increased sleep medicine use (HR 2.7, 95% CI = 1.7–4.3, p < 0.001) • increased visits to psychiatry (HR = 2.1, 95% CI = 1.1–4.0, p = 0.03) [ABSTRACT FROM AUTHOR]

To quantify and analyse the prevalence and clinical features of cerebral palsy (CP) in Denmark for birth years 2008–2013 and compare results with previous periods. A nationwide register-based study covering all children with a confirmed diagnosis of CP born in Denmark. Information about CP subtype, aetiology and severity was collected from the Cerebral Palsy Follow-up Program and supplemented from medical files. Data from the Danish Medical Birth Register was included, and the results were compared to previous data from the Danish National Cerebral Palsy Register. Prevalence per 1000 live births and proportions were analysed using the Cochran-Armitage test for trend. The period covered 368,618 live births and 636 children with CP, making the overall prevalence for the period 1.73 per 1000 live births. This was significantly lower than the prevalence of 1.99 for the previous period 1999–2007 (p = 0.004). The decline in prevalence between the two periods was mainly due to a decrease in children with bilateral spastic and dyskinetic CP born after 37 gestational weeks. The decline in prevalence was accompanied by a smaller proportion of children with associated impairment. We found a decrease in prevalence and severity in CP among Danish children. The decline was most pronounced in children born after 37 gestational weeks with severe subtypes of CP. National guidelines that recommend induction of labour before the completion of week 42 and therapeutic hypothermia for term neonates with hypoxic-ischaemic encephalopathy, may have contributed to the decline. • The prevalence of cerebral palsy in Denmark continues to decline. • There was a decline in prevalence and a smaller proportion with comorbidities. • Very few children with CP are now born after 42 weeks of gestation. • Children born at term still represent the majority of children with CP. • Induction of labour and therapeutic hypothermia may have contributed to the decline. [ABSTRACT FROM AUTHOR]

Purpose: The recurrent seizures of pediatric drug-resistant epilepsy (DRE) are known to impair brain development and can lead to a loss in cognitive functioning. Surgery is increasingly being used to treat children with DRE. This study investigates the pre- and postoperative cognitive function in a pediatric epilepsy surgery cohort as well as predictive determinants of change in intelligence quotient (IQ) following surgery.Methods: A consecutive series of 91 Danish children who underwent focal resective epilepsy surgery between January 1996 and December 2016 were included. All underwent preoperative cognitive evaluation and were reevaluated at 1-year and/or 2-year follow-up. Single-operated and multi-operated patients were examined separately.Results: 79 of 91 patients were single-operated. Single-operated patients received less anti-epileptic drugs (AED) and experienced a decrease in seizure frequency postoperatively, p < 0.001. IQ increased postoperatively (IQ change ± standard deviation: 3.3 ± 14.0), p < 0.05. High preoperative seizure frequency was a significant predictor for decreased IQ, p < 0.01. Multi-operated patients did not experience a reduction in AED treatment. Surgery and continued AED treatment did, however, result in significantly better seizure control, p < 0.01. IQ remained unchanged in multi-operated patients.Conclusion: Epilepsy surgery allowed for IQ gains in single-operated patients. Preoperative seizure frequency was a significant predictor of IQ change following surgery. Interactions between other, not included, possible predictors remain to be examined. Single-operated patients had the best cognitive outcome. The inclusion of a non-surgical control group is needed to assess the extent of the beneficial effects of surgery on cognitive ability. [ABSTRACT FROM AUTHOR]

To compare the prevalence of mental disorders (MDs) in a cohort of children and adolescents with and without cerebral palsy (CP) and to explore whether there is an association between MDs and the Gross Motor Function Classification System (GMFCS) level. A register-linkage follow-up study of 10- to 16-year children with CP (identified in the Danish National Cerebral Palsy Registry, n = 893), and 2627 children without CP, matched by gender and age. Information on MDs was obtained from the National Patient Registry in Denmark and based on ICD-10-codes. Conditional logistic regression was performed in order to compare the prevalence of MDs. The prevalence of MDs was significantly higher in children and adolescents with CP (22.4%, CI 19.8–25.2%) compared with controls (6.3%, CI 5.5–7.3%). Intellectual disability was statistically significantly associated with motor function (odds ratio (OR) 4.55, CI 2.81–7.36 for GMFCS levels IV–V compared to GMFCS level I), but there were no statistically significant association between motor function and autism spectrum disorders, ADHD or affective disorders. Our findings emphasize that follow-up of children with CP should include screening for both cognitive dysfunction and other mental disorders. The motor function does not predict the risk of other mental disorders than intellectual disability in children and adolescents with CP. • Mental disorders are 3.5 times more prevalent in children with CP than in controls. • Autism spectrum disorders are 2.5 times more prevalent in children with CP. • Affective or anxiety disorders are 2.7 times more prevalent in children with CP. • ADHD was diagnosed twice as often in children with CP than in controls. [ABSTRACT FROM AUTHOR]

Children of immigrants in Denmark have excess risk for some of the most well-established risk factors for cerebral palsy (CP). To study differences in risk of CP between children of immigrants and children of Danish-born mothers, and explore whether socioeconomic status drives any potential association. A register-based cohort study including 1,274,616 children born in Denmark between 1981 and 2007. Of these, 2807 had a validated CP diagnosis in the Danish CP Register. We estimated the risk of CP as odds ratios (OR) using logistic regression and assessed mediation through socioeconomic status using natural effect models. In children of Danish-born mothers, 2.2/1000 had CP overall and the prevalence was similar for children of immigrants. However, children of immigrants had lower risk of unilateral spastic CP than children of Danish native-born mothers; OR = 0.59 (95% CI:0.38–0.91) for Western and OR = 0.79 (95% CI:0.61–1.03) for Non-Western immigrants. By contrast, the risk of bilateral spastic CP was higher in children of Non-Western immigrants (OR = 1.27 (95% CI:1.05–1.53)), especially from Turkey and Pakistan compared with children of Danish native-born mothers. The mediation analysis revealed an indirect effect (through maternal educational level and household income) with an OR of 1.06 (95% CI:0.99–1.14) for children of Non-Western immigrants. While children of immigrants had lower risk of unilateral spastic CP than children of Danish-born mothers, the risk of bilateral spastic CP was increased in children of Non-Western immigrants. Socioeconomic status did not appear to be a significant contributor to the increased risk of bilateral spastic CP. • Ethnic inequality exist in the risk of cerebral palsy in Denmark. • Children of immigrants have a reduced risk of unilateral spasticity. • Children of Non-Western immigrants have an increased risk of bilateral spasticity. • Socioeconomic status does not contribute to the inequality in bilateral spasticity. [ABSTRACT FROM AUTHOR]

Abstract Aim To analyse trends in prevalence and severity of cerebral palsy (CP) in Denmark in birth years 1999–2007 and compare with previous periods. Method Data has been collected uniformly in the Danish cerebral palsy national register nationwide since 1995. Rates in the time periods 1999–2001, 2002–2004 and 2005–2007 covering 585,393 births were analysed by gestational age and subtypes. Results Total number of CP cases in the period was 1165. The overall prevalence of CP decreased significantly from 2.1 in 1999–2001 to 1.8 in 2005–2007 per 1000 livebirths (p = 0.022). The decline was only significant for children born at term (p = 0.007) but not for the preterm (p = 0.44). The decline in children born at term was based on a decrease in bilateral spastic CP (n = 117 in years 1999–2001 and n = 59 in 2005–2007). Multidisciplinary obstetric skills training with neonatal resuscitation in Denmark was initiated in 2003 and timely associated with the decrease. The prevalence of unilateral spastic CP the prevalence did not change, but in the two last time periods more children had a right-sided than left-sided unilateral spastic CP. Conclusion The decline in rate of CP seen in 2005–2007 as compared to 1999–2001 was mainly based on fewer cases of severe spastic CP in term infants. We hypothesize that improved neonatal resuscitation in the delivery room may be partly responsible for the decrease. In premature children the decline was not significant in this time period, but has been dramatically decreasing in the years before the time period here analysed. Highlights • Prevalence of cerebral palsy decreased to 1.8/1000 live births in 2005–2007. • The decline was only significant for children born at term. • Less children were found to have bilateral spastic CP. • Improved neonatal resuscitation may be responsible for the decrease. • In premature children the decline was not significant in this time period. [ABSTRACT FROM AUTHOR]

Highlights • Chitinase 3-like 1 and neurofilament light chain are increased in pediatric acquired demyelinating syndromes (ADS). • Chitinase 3-like 1 and neurofilament light chain better distinguish between ADS and non-ADS than oligoclonal bands alone. • Chitinase 3-like 1 is increased in ADEM compared with MS. Abstract Background Chitinase 3-like 1 (CHI3L1), neurofilament light chain (NFL) and oligoclonal bands (OCB) in cerebrospinal fluid are associated with central nervous system demyelination in adults. CHI3L1 and OCB are markers of central nervous system inflammation, whereas NFL is a marker of white-matter axonal injury. The aim was to examine whether CHI3L1 and NFL in cerebrospinal fluid are associated with acquired demyelinating syndromes at disease onset in a pediatric population. Methods Children (<18 years) referred to hospital for possible neuroinflammatory disease were retrospectively included from 2010 to 2016. Case ascertainment was by review of medical records. NFL and CHI3L1 were measured by enzyme-linked immunosorbent assays. Endpoints were differences in concentrations of cerebrospinal fluid NFL and CHI3L1. Results We included 193 children who all underwent cerebrospinal fluid OCB examination as part of their diagnostic work-up and classified these children into 5 groups: acquired demyelinating syndromes (n = 33), normal diagnostic work-up (n = 36), inflammatory neurological disease (n = 50), other neurological disease (n = 55), and systemic inflammatory diseases (n = 19). NFL and CHI3L1 in cerebrospinal fluid differed significantly between the five groups (p = 0.0001). CHI3L1 was significantly higher in acquired demyelinating syndromes than in all other groups, and NFL was significantly higher in acquired demyelinating syndromes than in the other groups except systemic inflammatory disease. Children with acute disseminated encephalomyelitis had significantly higher concentrations of CHI3L1 than did children with multiple sclerosis. Conclusion We provide class II evidence that CHI3L1 and NFL are associated with pediatric acquired demyelinating syndromes. CHI3L1 may help distinguishing between acute disseminated encephalomyelitis and multiple sclerosis, but this needs further confirmation. [ABSTRACT FROM AUTHOR]

Background Pediatric-onset multiple sclerosis (MS) affects life at a stage vital for social and educational achievements and psychiatric co-morbidity is common after MS onset. Few studies have examined psychiatric morbidity before MS onset. Methods In this nationwide study, detailed case ascertainment was performed in all children with pediatric MS, including chart review. For each MS patient, we selected five controls using density sampling from the entire Danish population, matching controls to children with MS by sex and birthdate. We analyzed data as a nested case-control study with psychiatric morbidity as exposure and MS as outcome, and a matched cohort study with MS as exposure and psychiatric co-morbidity as outcome. Hazard ratios (HR) including 95% confidence intervals (CI) were estimated using Cox regression. Results We identified 212 children with MS and 1060 controls. No association between psychiatric morbidity and the rate of MS was found before MS onset. After MS onset, children with MS had two times higher hazard for psychiatric co-morbidity compared with children without MS (HR=2.0; 95% CI=1.3–3.1; p<0.001). Conclusion Psychiatric morbidity seems to commence after MS onset, making screening for neuropsychiatric conditions pertinent in newly-diagnosed children with MS. [ABSTRACT FROM AUTHOR]

Purpose Epilepsy surgery is performed based on the assumption that medical refractory epilepsy will continue. Rarely seizure freedom occurs before surgery is performed, while the patient is being evaluated as an epilepsy surgery candidate. The aim of this study was to describe the number of children withdrawn from an epilepsy surgery programme due to unexpected seizure improvement. Methods We retrospectively studied 173 children under 18 years with medical refractory epilepsy referred for epilepsy surgery between 1996 and 2010. Medical records were reviewed in 2012 and 2015. Results At the first evaluation point in 2012, 13 patients were withdrawn from the epilepsy surgery programme due to unexpected marked improvement. In 2015, 6 of them were still seizure free. They had unexpected seizure freedom due to change in AED treatment (n = 3) or after a febrile episode (n = 3). The mean number of years they had had seizures was 3.4 years (range 0.6–6.2 years) and the number of seizures at inclusion was 209 per month (range 6–750 per month). The duration of follow-up was 6.6 years after inclusion into the epilepsy surgery programme (range 4.0–13.0 years). The aetiology of the epilepsy for these patients was heterotopia (n = 1), focal cortical dysplasia (n = 3), infarction (n = 1) and unknown, with normal MRI (n = 1). They all had an IQ in the normal range. Two of the remaining 7 children were operated later. Conclusion Unexpected seizure control may occur during epilepsy surgery evaluation. [ABSTRACT FROM AUTHOR]

Purpose: Epilepsy surgery is performed based on the assumption that medical refractory epilepsy will continue. Rarely seizure freedom occurs before surgery is performed, while the patient is being evaluated as an epilepsy surgery candidate. The aim of this study was to describe the number of children withdrawn from an epilepsy surgery programme due to unexpected seizure improvement.Methods: We retrospectively studied 173 children under 18 years with medical refractory epilepsy referred for epilepsy surgery between 1996 and 2010. Medical records were reviewed in 2012 and 2015.Results: At the first evaluation point in 2012, 13 patients were withdrawn from the epilepsy surgery programme due to unexpected marked improvement. In 2015, 6 of them were still seizure free. They had unexpected seizure freedom due to change in AED treatment (n=3) or after a febrile episode (n=3). The mean number of years they had had seizures was 3.4 years (range 0.6-6.2 years) and the number of seizures at inclusion was 209 per month (range 6-750 per month). The duration of follow-up was 6.6 years after inclusion into the epilepsy surgery programme (range 4.0-13.0 years). The aetiology of the epilepsy for these patients was heterotopia (n=1), focal cortical dysplasia (n=3), infarction (n=1) and unknown, with normal MRI (n=1). They all had an IQ in the normal range. Two of the remaining 7 children were operated later.Conclusion: Unexpected seizure control may occur during epilepsy surgery evaluation. [ABSTRACT FROM AUTHOR]

Purpose West syndrome (WS) is a severe age-related acute epileptic encephalopathy of infancy characterized by infantile spasms, hypsarrhythmia and psychomotor delay. The aim of this study was to investigate if patients with WS had an altered autonomic output to the heart. Methods In 23 patients with WS the heart rate variability (HRV) was investigated by examining time- and frequency-domain parameters of HRV at the time of the diagnosis of hypsarrhythmia and compared to 22 age-matched controls. For the WS patients the same dataset was obtained and compared again at the end of the study period, when hypsarrhythmia was no longer present. Results Compared to controls, patients with WS during hypsarrhythmia had significantly lower SDNN (the standard deviation of the NN interval, i.e. the square root of variance) (19.2 ms; p = 0.007, Mann–Whitney's U -Test) and total power (242 ms 2 ; p = 0.044, Mann–Whitney's U -Test) in the awake state, indicating an abnormal autonomic output to the heart. Comparing the initial to the final examination demonstrated a significant increase in the HRV parameters SDNN (31.3 ms) and total power (757 ms 2 ; p = 0.001 and p = 0.013, Wilcoxon Signed Ranked Test). In addition, at the final examination the WS-patients no longer differed significantly from the controls. Conclusion Our data suggest that the initial reduction in HRV in patients at the time of onset of WS is transient and related to the presence of hypsarrhythmia. [ABSTRACT FROM AUTHOR]

Abstract: Children with cerebral palsy participate less in everyday activities than children in the general populations. During adolescence, rapid physical and psychological changes occur which may be more difficult for adolescents with impairments. Within the European SPARCLE project we measured frequency of participation of adolescents with cerebral palsy by administering the Questionnaire of Young People's Participation to 667 adolescents with cerebral palsy or their parents from nine European regions and to 4666 adolescents from the corresponding general populations. Domains and single items were analysed using respectively linear and logistic regression. Adolescents with cerebral palsy spent less time with friends and had less autonomy in their daily life than adolescents in the general populations. Adolescents with cerebral palsy participated much less in sport but played electronic games at least as often as adolescents in the general populations. Severity of motor and intellectual impairment had a significant impact on frequency of participation, the more severely impaired being more disadvantaged. Adolescents with an only slight impairment participated in some domains as often as adolescents in the general populations. Regional variation existed. For example adolescents with cerebral palsy in central Italy were most disadvantaged according to decisional autonomy, while adolescents with cerebral palsy in east Denmark and northern England played sports as often as their general populations. Participation is an important health outcome. Personal and environmental predictors of participation of adolescents with cerebral palsy need to be identified in order to design interventions directed to such predictors; and in order to inform the content of services. [Copyright &y& Elsevier]

Abstract: Background: Patients with epilepsy, including children, have an increased mortality rate when compared to the general population. Only few studies on causes of mortality in childhood epilepsy exist and pediatric SUDEP rate is under continuous discussion. Aim: To describe general mortality, incidence of sudden unexpected death in epilepsy (SUDEP), causes of death and age distribution in a pediatric epilepsy patient population. Methods: The study retrospectively examined the mortality and causes of death in 1974 patients with childhood-onset epilepsy at a tertiary epilepsy center in Denmark over a period of 9 years. Cases of death were identified through their unique civil registration number. Information from death certificates, autopsy reports and medical notes were collected. Results: 2.2% (n = 43) of the patient cohort died during the study period. This includes 9 patients with SUDEP (8 SUDEP cases per 10,000 patient years). 9 patients died in the course of neurodegenerative disease and 28 children died of various causes. Epilepsy was considered drug resistant in more than 95% of the deceased patients, 90% were diagnosed with intellectual disability. Mortality of patients that underwent dietary epilepsy treatment was slightly higher than in the general cohort. There were no epilepsy-related deaths due to drowning. Conclusions: This study confirms that SUDEP must not be disregarded in the pediatric age group. The vast majority of SUDEP cases in this study displays numerous risk factors similar to those described in adult epilepsy patients. Including SUDEP, only 30% of the mortality was directly seizure related. [Copyright &y& Elsevier]

Abstract: Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder characterized by early-onset recurrent distinctive hemiplegic episodes commonly accompanied by other paroxysmal features and developmental impairment. De novo mutations in ATP1A3 were recently identified as a genetic cause of AHC. To describe the entire Danish cohort of paediatric AHC patients we approached neuropaediatricians nationwide. All currently acknowledged Danish patients ≤16 years with AHC were genetically tested and seen by the same child neurologist (PU). Ten patients; seven girls and three boys were identified. Mean present age was 10.0 years (range 1–16). Mean age at presentation was 7.4 months (range 1–18 months). Sequencing of ATP1A3 in all ten patients revealed a pathogenic mutation in seven. Two females with moderate psychomotor impairment were heterozygous for the known p.G947R mutation, whereas one severely retarded boy was heterozygous for the common p.E815K mutation. The prevalent p.D801N mutation was identified in two moderate to severely retarded children. Interestingly, in a set of monochorionic male twins a novel p.D801E mutation was identified, underscoring that the asparagine at position 801 is a mutation hotspot. Three girls aged 5–13 years did not reveal any ATP1A3 mutations. They were rather mildly clinically affected and displayed a normal or near-normal psychomotor development. This is the first study of AHC in the Danish paediatric population. The patients harboured a wide range of psychomotor difficulties. Patients with no mutation detected tended to be less severely affected. Prevalence was approximately 1 per 100,000 children. [Copyright &y& Elsevier]

Abstract: Introduction: Refractory status epilepticus (RSE) in children is associated with a significant risk of death or neurological morbidity. Recently attention has been drawn to the ketogenic diet (KD) as an acute treatment, as it has shown promise in controlling seizures in otherwise refractory status epilepticus in several cases. We have listed these and reviewed all cases of KD used in RSE at our centre. KD was given as 4:1 fat:carbohydrate-protein solution. Results: A 3-year-old girl with RSE due to Hemiconvulsion-Hemiplegia Epilepsy syndrome. KD was instigated on day 6. Seizures stopped with ketosis on day 7. A 10-year-old boy rapidly developing RSE. After months a mitochondrial disorder was discovered. KD was tried twice with severe side-effects but no seizure control. 11-year-old healthy boy with RSE as FIRES. On KD seizures stopped for 24 h one day after reaching ketosis. He improved over 3–4 weeks. Discussion: KD was efficient in two of three cases of RSE. The non-responder had severe side-effects and proved to have a mitochondrial disorder which is arguably a contraindication for KD. More studies are needed to prove efficacy of KD in RSE, to define optimal timing of KD and possible contraindications for KD in RSE. [Copyright &y& Elsevier]

Abstract: Background: Although epilepsy is common in children with cerebral palsy (CP), no data exists on prevalence rates of CP and epilepsy. Aims: To describe epilepsy in children with CP, and to examine the association between epilepsy and neonatal characteristics, associated impairments and CP subtypes. Methods: Data on 9654 children with CP born between 1976 and 1998 and registered in 17 European registers belonging to the SCPE network (Surveillance of Cerebral Palsy in Europe) were analyzed. Results: A total of 3424 (35%) children had a history of epilepsy. Among them, seventy-two percent were on medication at time of registration. Epilepsy was more frequent in children with a dyskinetic or bilateral spastic type and with other associated impairments. The prevalence of CP with epilepsy was 0.69 (99% CI, 0.66–0.72) per 1000 live births and followed a quadratic trend with an increase from 1976 to 1983 and a decrease afterwards. Neonatal characteristics independently associated with epilepsy were the presence of a brain malformation or a syndrome, a term or moderately preterm birth compared with a very premature birth, and signs of perinatal distress including neonatal seizures, neonatal ventilation and admission to a neonatal care unit. Conclusions: The prevalence of CP with epilepsy followed a quadratic trend in 1976–1998 and mirrored that of the prevalence of CP during this period. The observed relationship between epilepsy and associated impairments was expected; however it requires longitudinal studies to be better understood. [Copyright &y& Elsevier]

Abstract: Glucocorticoids are commonly used in treatment of paediatric diseases, but evidence of associated adverse cerebral effects is accumulating. The various pharmacokinetic profiles of the exogenous glucocorticoids and the changes in pharmacodynamics during childhood, result in different exposure of nervous tissue to exogenous glucocorticoids. Glucocorticoids activate two types of intracellular receptors, the mineralocorticoid receptor and the glucocorticoid receptor. The two receptors differ in cerebral distribution, affinity and effects. Exogenous glucocorticoids favor activation of the glucocorticoid receptor, which is associated with unfavorable cellular outcomes. Prenatal treatment with glucocorticoids can compromise brain growth and is associated with periventricular leukomalacia, attentions deficits and poorer cognitive performance. In the neonatal period exposure to glucocorticoids reduces neurogenesis and cerebral volume, impairs memory and increases the incidence of cerebral palsy. Cerebral effects of glucocorticoids in later childhood have been less thoroughly studied, but apparent brain atrophy, reduced size of limbic structures and neuropsychiatric symptoms have been reported. Glucocortioids affect several cellular structures and functions, which may explain the observed adverse effects. Glucocorticoids can impair neuronal glucose uptake, decrease excitability, cause atrophy of dendrites, compromise development of myelin-producing oligodendrocytes and disturb important cellular structures involved in axonal transport, long-term potentiation and neuronal plasticity. Significant maturation of the brain continues throughout childhood and we hypothesize that exposure to exogenous glucocorticoids during preschool and school age causes adverse cerebral effects. It is our opinion that studies of associations between exposure to glucocorticoids during childhood and impaired neurodevelopment are highly relevant. [Copyright &y& Elsevier]

Abstract: The Cerebral Palsy Registry in eastern Denmark has been collecting cases using a uniform data sampling procedure since birth year 1979. Children are included by two child neurologists and an obstetrician. Information on pregnancy, birth, neonatal period, impairments and demographic data are registered. The total cerebral palsy birth prevalence has been significantly decreasing since the birth period 1983–1986 with 3.0 per 1000 live births until the period 1995–1998 with 2.1 per 1000 live births. The overall decrease was seen in preterm infants (<31 weeks) as well as in term infants and despite a simultaneous fall in perinatal and early neonatal mortality in the preterm group. Analysing the subtypes of CP we found a significant increase in the numbers as well as the rate of unilateral CP with a simultaneous fall in the numbers as well as the rate of bilateral CP. The explanation of this rise is not obvious. A change from bilateral periventricular lesions to unilateral is a possibility, but no major change in the neonatal handling could be documented. Regarding associated impairments, developmental delay/learning disabilities as well as motor function assessed by ability to walk (unassisted/assisted), both have changed toward higher percentage of children with unassisted walking and in need of special education. [Copyright &y& Elsevier]

Abstract: The aim of the present study was to evaluate the tolerability and efficacy of the modified Atkins diet given to children and adolescents with antiepileptic drug (AED) treatment resistant epilepsy. 15 children with medically intractable epilepsy were enrolled in the study. Inclusion criteria were at least one seizure a week and a trial of at least two AEDs without obtaining seizure freedom documented in a seizure calendar. At baseline subjects initiated a diet with carbohydrates restricted to make up 10 energy percent. If seizures were reduced by less than 50% after 7–14 days carbohydrates were further restricted to 10g per day. No change in AED treatment was allowed. The diet was well tolerated. After 3 months six out of the fifteen children (40%) had a seizure reduction of more than 50%, which was seen in different epileptic syndromes and different age groups. The responders reported an increase in quality of life and cognition. At 12 months follow-up 3 (20%) continued the diet with an unchanged marked seizure reduction. The present study confirms the high tolerability and effect of the modified Atkins diet on seizure control in AED treatment resistant epilepsy. Further larger prospective studies are however needed to confirm these results. [Copyright &y& Elsevier]

Abstract: Participation in home, school and community is important for all children; and little is known about the frequency of participation of disabled children. Frequency of participation is a valuable outcome measure for evaluating habilitation programmes for disabled children and for planning social and health services. We investigated how frequency of participation varied between children with cerebral palsy and the general population; and examined variation across countries to understand better how the environmental factors such as legislation, public attitudes and regulation in different countries might influence participation. We undertook a multi-centre, population-based study in children with and without cerebral palsy. Working from the Life-H instrument, we developed a questionnaire to capture frequency of participation in 8–12-year-old children. In nine regions of seven European countries, parents of 813 children with cerebral palsy and 2939 children from the general populations completed the questionnaire. Frequency of participation for each question was dichotomised about the median; multivariable logistic regressions were carried out. In the general population, frequency of participation varied between countries. Children with cerebral palsy participated less frequently in many but not all areas of everyday life, compared with children from the general population. There was regional variation in the domains with reduced participation and in the magnitude of the differences. We discuss how this regional variation might be explained by the different environments in which children live. Attending a special school or class was not associated with further reduction in participation in most areas of everyday life. [Copyright &y& Elsevier]

Introduction DYT11 is an autosomal dominant inherited movement disorder characterized by myoclonus and dystonia. Clinical presentation We present a case with atypical symptoms and with episodes of ataxia and myoclonus preceded by infections. Atypical presentation of opsoclonus myoclonus syndrome was suspected and treatment with bolus steroids and immunoglobulin were initiated with some response over 28 months. A re-evaluation gave suspicion of a dyskinetic disorder and whole exome-sequencing was performed but no causal variant was identified. Outcome A specific analysis of the SGCE gene was subsequently initiated, which revealed a pathogenic aberration confirming the diagnosis of DYT11. Conclusion A clinical DYT11 diagnosis can be difficult to establish in early childhood without a known family history. [ABSTRACT FROM AUTHOR]

• Development of a 2-step LCA framework for LIB repurposing in a circular economy. • Application to case study on real-world battery storage project at industrial scale. • Integrating energy flow modelling in LCA enables assessment of different use cases. • Climate change benefits in multi-use case are 10-22% lower than in single-use cases. • Results are compared to alternative circular business model options for LIB. In their efforts to implement a circular economy (CE) for lithium-ion batteries (LIB) in electric vehicles, automotive manufacturers need to take into account the perspective of energy consumers when assessing the environmental benefits of LIB repurposing in life cycle assessment (LCA). In response to this issue, this study presents a novel LCA framework, which allows manufacturers to assess different cases of LIB repurposing in an energy system and interpret the results in a CE context. The framework firstly uses energy flow modelling to enable the assessment of combining different battery storage applications in multi-use cases. Secondly, it includes a comparison of repurposing with alternative circular business models options for LIB. The framework is applied to an automotive manufacturer, seeking to assess a real-world project of LIB repurposing in different combinations of behind-the-meter applications at an industrial production site in Germany. As a key outcome, results reveal that from the perspective of the energy consumer, climate change benefits in multi-use cases are 10–22% lower than in single applications. Furthermore, from the perspective of the automotive manufacturer, repurposing is identified as the most beneficial option of circular business models available for LIB, taking into account additional recycling benefits resulting from the delay of end-of-life. Based on these findings, the study contributes to the application of LCA for decision-making in a CE and highlights pitfalls and potentials for a sustainable implementation of LIB repurposing in the future. [ABSTRACT FROM AUTHOR]

Muscle wasting may occur in patients with chronic renal failure (CRF). To determine whether this is due to a decrease in the synthesis or an increase in the breakdown of muscle protein, we evaluated postabsorptive whole-body protein breakdown, oxidation, and synthesis rates at steady state during a primed, continuous infusion of 13C-leucine. This was done in seven subjects on chronic maintenance hemodialysis (MHD) and in seven normal control subjects. The protein breakdown rate in MHD was not different from that in controls (103 ± 19 and 106 ± 19 µmol leucine kg-1 • h-1, respectively). In MHD, however, the protein oxidation rate was 43% greater than that in controls (20 ± 6 and 14 ± 4 µmol leucine • kg-1 • h-1, p < 0.05), whereas net protein synthesis was less (p < 0.05). Reduced net synthesis and increased oxidation rates of protein in the postabsorptive state may therefore contribute to the muscle-wasting syndrome in patients with CRF. [ABSTRACT FROM AUTHOR]

To determine whether skeletal muscle function testing (SMF) provides an index of nutritional status in patients with chronic renal failure (CRF), two groups with comparable CRF were studied. In 48 well-nourished (WN) and 17 malnourished (MN) patients with stable CRF, and in 33 WN nonazotemic controls, adductor pollicis function was assessed. The force at 10 Hz was expressed as a % offorce at 100 Hz (FlO/Fl00), and maximal relaxation rate (MRR) as % force loss/ 10 ms. Standard nutritional assessment was also performed. The WN group was not significantly different from controls for either F10/F100 or MRR. The F10/Fl00 of the MN group was significantly greater than either the WN group or controls (p <0.001), while MRR was less (p < 0.001). Significant malnutrition by conventional parameters was shown in patients with abnormal Fl0/F100, and also in patients with abnormal MRR. Hence, SMF as described is unaffected by azotemia, and provides a functional measure of nutritionalstatus in CRF. [ABSTRACT FROM AUTHOR]

Objective: The objective of the study was to investigate the association between maternal self-reported infections, fever, and smoking in the prenatal period and the subsequent risk for congenital cerebral palsy (CP).Study Design: We included the 81,066 mothers of singletons born between 1996 and 2003 who participated in the Danish National Birth Cohort. Children were followed up through December 2008. Information on maternal infections, fever, smoking, and other demographic and lifestyle factors during pregnancy were reported by mothers in computer-assisted telephone interviews in early and midgestation. We identified 139 CP cases including 121 cases of spastic CP (sCP) as confirmed by the Danish National Cerebral Palsy Register. Cox proportional hazards regression models were used to estimate adjusted hazard ratios (aHRs) and 95% confidence intervals (CIs).Results: Self-reported vaginal infections were associated with an increased risk of CP and sCP (aHR, 1.52; 95% CI, 1.04-2.24; and aHR, 1.73; 95% CI, 1.16-2.60, respectively) and particularly untreated vaginal infections were associated with an increased risk of sCP (aHR, 1.95; 95% CI, 1.16-3.26). Fever was associated with the risk of CP (aHR, 1.53; 95% CI, 1.06-2.21). Smoking 10 or more cigarettes per day during pregnancy was also associated with sCP (aHR, 1.80; 95% CI, 1.10-2.94). There was a modest excess in risk for children exposed to both heavy smoking and vaginal infections. No other self-reported infections were significantly associated with CP.Conclusion: Self-reported vaginal infections, fever, and smoking 10 or more cigarettes per day during pregnancy were associated with a higher risk of overall CP and/or sCP. [ABSTRACT FROM AUTHOR]