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Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized by generalised epileptiform activity and neurocognitive dysfunction. Causes and outcome are diverse and treatment is mainly empirical. Retrospective descriptive analysis of clinical and EEG data of children with CSWS diagnosed between 1998 and 2018 at the University Hospital Heidelberg. Ninety-five children were included with a median age at diagnosis of 5.4 years. A structural/metabolic aetiology was found in 43.2%, genetic alterations in 17.9%, while it remained unknown in 38.9%. The proportion of patients with genetic aetiology increased from 10.3% (1998–2007) to 22.8% (2008–2018). On average, each patient received 5 different treatments. CSWS was refractory in >70% of cases, steroids and neurosurgery were most effective. No difference was observed between children with CSWS or Near-CSWS (Spike-Wave-Index 40–85%). Our cohort confirms CSWS as an age-dependent epileptic encephalopathy. Structural brain abnormalities were most frequent, but genetic causes are increasingly identified. More specific criteria for the diagnosis and treatment goals should be elaborated and implemented based on evidence. This study is the largest monocentric observational study on treatment effects in children with CSWS, providing data for diagnostic and therapeutic decisions. • Age at CSWS occurrence is earlier in children with identifiable etiology. • Significant better treatment effects were found for the treatment with steroids or neurosurgery. • No difference between patients with CSWS (SWI >85%) and Near-CSWS (SWI 40–85%) apart from applied treatments was found [ABSTRACT FROM AUTHOR]

Abstract: Background: Most studies on clinical outcome of preterm born infants focus on high-risk infants, whereas little is known about the long-term prognosis of low-risk low birth weight preterm born infants (LBW). Aims: To examine physical growth, school outcome, behavioural aspects, quality of life, and intervention impact on LBW in late adolescence compared to term born controls and to analyse developmental pathways from childhood to adolescence for these groups. Study design: In late adolescence 65 LBW and 41 matched term born controls were assessed with a structured telephone interview and standardized questionnaires with regard to physical growth, school career, therapeutic interventions, behavioural aspects, and quality of life. Longitudinal analysis comprised data from birth, infancy, school age, and adolescence. Results: Growth in height of LBW was highly variable, but correlated significantly with birth parameters and was below those of controls with regard to percentiles. School enrollment of LBW was more often delayed, and there was a trend toward lower school graduation in LBW. The groups did not differ with regard to general or health-related quality of life and behavioural aspects, but LBW received significantly more therapeutical interventions. Conclusions: In general, LBW showed no major deficits in late adolescence. However, a prolonged school career and a higher demand for therapeutic interventions indicate subtle neurodevelopmental deficits in LBW. This underscores the need for professional follow-up programmes for this majority of preterm born infants. [Copyright &y& Elsevier]

Cognitive development in children and adolescents with focal lesional epilepsy is determined by the underlying epileptogenic lesion, in addition to epilepsy itself. However, the impact of lesion-related variables on intelligence quotient (IQ) and developmental quotient (DQ) remains largely unexplored. Here, we aimed to determine the effect of lesion-related predictors and their relation with epilepsy-related predictors of intellectual functioning. We retrospectively analyzed data from children with focal lesional epilepsy who underwent standardized cognitive evaluation yielding IQ/DQ in our institution. We included 50 consecutive patients aged 0.5 to 17.5 years (mean, 9.3; S.D., 4.9) at cognitive assessment. Epilepsy duration was 0 to 15.5 years (mean, 3.8; S.D., 4.1). Of the total cohort, 30 (60%) patients had unilobar lesions, seven (14%) multilobar, 10 (20%) hemispheric, and three (6%) bilateral. Etiology was congenital in 32 (64%) cases, acquired in 14 (28%), and progressive in four (8%). For patients with unilobar lesions, the mean IQ/DQ was 97.1 ± 15.7, for multilobar 98.9 ± 20.2, for hemispheric 76.1 ± 20.5, and for bilateral 76.3 ± 4.5. Larger lesion extent, earlier epilepsy onset, and longer epilepsy duration correlated with lower IQ/DQ in the univariate analysis, whereas only lesion extent and epilepsy duration contributed significantly to the explanatory model in the multivariable analysis. The present study demonstrates that lesion extent and epilepsy duration are important risk factors for intellectual impairment in pediatric patients with focal lesional epilepsy. These findings are useful for family counseling and the early consideration of interventions that may limit the duration of epilepsy. [ABSTRACT FROM AUTHOR]

Abstract: High negative reactivity in early childhood interferes with later academic and behavioral adjustment. Thus, investigating the origins of high negative affectivity in early childhood is of high relevance for understanding emotional morbidity after preterm birth. The present work explored (1) direct prematurity-related consequences for negative reactivity, (2) self-regulatory deficits as a mechanism indirectly relating prematurity to negative affectivity and (3) the implications of the interplay between procedural distress in the neonatal period and parenting stress for preterm children''s negative reactivity. The sample was comprised of 146 preterm children (very vs. moderately to late preterm) and 86 healthy full-term children, both free of major neurological impairment. Assessment involved negative affect and parenting stress (parent-report; 12, 24 months corrected age, CA), effortful control (behavioral battery, parent report; 24 months CA) and the number of potentially distressing neonatal intensive care procedures as well as severity of illness during the neonatal period (retrospective chart review). There was no direct link from prematurity to a disposition for high negative reactivity in early childhood nor was prematurity indirectly associated with higher negative reactivity through lower levels of effortful control. The relation between neonatal pain and distress and negative affectivity depended on the level of parenting stress with low parenting stress at the end of the first year of children''s life buffering the negative influence of neonatal distress. The present findings underscore the importance of complex interactions among environmental factors in processes of emotional plasticity after preterm birth thereby providing critical suggestions for follow-up care. [Copyright &y& Elsevier]

Abstract: Background: Preterm birth is thought to have an adverse impact on cognitive development and self-regulation. Aim: Examining the effect of very vs. moderately to late premature birth on cognitive development and effortful control, as well as evaluating whether effortful control explains the link between preterm birth and poorer cognitive development. Subjects: Fifty-eight very preterm children (<32weeks gestation or <1500g birth weight), 88 moderately to late preterm children (≥32weeks gestation and ≥1500 birth weight) and 86 full-term children (≥38weeks gestation and ≥2500g birth weight) were examined at the corrected age of 24months. Outcome measures: Observational and parent-report measures of effortful control as well as the Bayley Scales of Infant Development II (BSID II, Mental Scale) as a measurement of cognitive development were analyzed. Results: Very preterm and moderately to late preterm children showed significantly lower cognitive performance compared to full-term children. Lower effortful control scores (on observational measures, but not on parent-reports) were merely found for very preterm children compared to full-term children. Observational measures of effortful control partially mediated the effects of very preterm birth on cognitive performance, but did not explain the effects of moderately to late preterm birth on cognitive performance. Conclusion: Preterm birth in general is related to poorer cognitive performance in toddlerhood. In addition, effortful control mediates the effects of very preterm birth on cognitive development. Findings suggest that different mechanisms link moderately to late premature birth to poor cognitive development. [Copyright &y& Elsevier]