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This literature review on social functioning of children after epilepsy surgery is based on 24 papers addressing two categories of social functioning: social cognition (n = 4) and general social functioning (n = 20). Overall, studies that compared with healthy peers revealed children who had undergone epilepsy surgery to have more problems in both social cognition and general social functioning. Half of the studies found some improvement in social functioning in the first year(s) after epilepsy surgery, but this pertained to general social functioning, not to social cognition. The persistence of the problems in social cognition after surgery may be related to the critical period of brain maturation, lacking improvement of impairments in related cognitive domains or to a defective underlying brain condition — rather than to the epilepsy. Problems in general social functioning may be explained by the adjustments the children and their families had to make because of the child's drug-resistant epilepsy and difficulties to adjust to the new situation after surgery. The neurological and behavioral explanations are likely to be interrelated in light of the multifaceted and complex nature of social functioning. Epilepsy surgery does not appear to solve the problems in social functioning associated with having had drug-resistant epilepsy. As social functioning is an important aspect of healthy development, it should be assessed comprehensively in order to obtain a knowledge base that allows 1) proper treatment of children with epilepsy (CwE) and 2) counseling patients and families prior to and after epilepsy surgery.

Objectives The objective of this study was to explore whether parents experience problems in their own psychological wellbeing and their family functioning two to four years after their child's epilepsy surgery and whether these problems are associated with epilepsy variables, demographic and cognitive variables, and parent-observed behavior problems of the child. Methods Of the 65 approached families, parents of 31 children participated by completing the Brief Symptom Inventory (BSI), the Family Questionnaire, and the Child Behavior Checklist (CBCL). High scores indicating clinically relevant problems were reported and called ‘problem scores’. Correlations between results of questionnaires and demographic and illness variables (abstracted from medical files) were computed for fathers and mothers separately. By comparing the group with at least one problem score with the group without problem scores, risk factors for parent-perceived problems in their own psychological functioning and in family functioning were explored. Results Thirty percent of the mothers had problem scores on hostility and on communication within their family. Only a few fathers obtained problem scores, most of these pertaining to their family's organization. Not one parent had a problem score regarding their partner relationship. Many parents had problem scores on behavior problems in their child. Brain area of surgery was the only epilepsy variable related to parents' wellbeing and family functioning, with lowest problem scores for the hemispherotomy group. Scores on behavior problems in the child were also lowest for children after hemispherotomy and for those who had achieved freedom of seizures and antiepileptic drugs (AEDs). Fathers of older children experienced more problems than those of young children. Conclusions Parent's wellbeing and family functioning cannot be understood from epilepsy or epilepsy surgery variables only but are related to the child's age and behavior. Having epilepsy is associated with emotional and behavior problems and limits children in developing age-appropriate self-dependence. These problems are not resolved after achieving seizure freedom and have great influence on the family. Professionals should set realistic expectations of epilepsy surgery and should assess, acknowledge, and follow up problems of parental psychological wellbeing and family functioning, regardless of the outcome.

Mutations in the gene encoding of the catalytic subunit of mtDNA polymerase gamma (POLG1) can cause typical Alpers' syndrome. Recently, a new POLG1 mutation phenotype was described, the so-called juvenile-onset Alpers' syndrome. This POLG1 mutation phenotype is characterized by refractory epilepsy with recurrent status epilepticus and episodes of epilepsia partialis continua, which often necessitate admission to the intensive care unit (ICU) and pose an important mortality risk. We describe two previously healthy unrelated teenage girls, who both were admitted with generalized tonic-clonic seizures and visual symptoms leading to a DNA-supported diagnosis of juvenile-onset Alpers' syndrome. Despite combined treatment with anti-epileptic drugs, both patients developed status epilepticus requiring admission to the ICU. Intravenous magnesium as anti-convulsant therapy was initiated, resulting in clinical and neurophysiological improvement and rapid extubation of both patients. Treating status epilepticus in juvenile-onset Alpers' syndrome with magnesium has not been described previously. Given the difficulties encountered while treating epilepsy in patients with this syndrome, magnesium therapy might be considered.

Febrile seizures are the most prevalent type of seizures among children up to 5 years of age (2-4% of Western-European children). Complex febrile seizures are associated with an increased risk to develop temporal lobe epilepsy. To investigate short- and long-term effects of experimental febrile seizures (eFS), we induced eFS in highly febrile convulsion-susceptible C57BL/6J mice at post-natal day 10 by exposure to hyperthermia (HT) and compared them to normotherm-exposed (NT) mice. We detected structural re-organization in the hippocampus 14 days after eFS. To identify molecular candidates, which entrain this structural re-organization, we investigated temporal changes in mRNA expression profiles eFS 1 hour to 56 days after eFS. We identified 931 regulated genes and profiled several candidates using in situ hybridization and histology at 3 and 14 days after eFS. This is the first study to report genome-wide transcriptome analysis after eFS in mice. We identify temporal regulation of multiple processes, such as stress-, immune- and inflammatory responses, glia activation, glutamate-glutamine cycle and myelination. Identification of the short- and long-term changes after eFS is important to elucidate the mechanisms contributing to epileptogenesis.

We studied how children with epilepsy (CWE) who are candidates for epilepsy surgery, perceive themselves with respect to overarching personality traits and whether the traits change after surgery. We explored influences of demographic and illness variables. A total of 23 CWE [mean age at inclusion 12.8 (sd 2.3); 12 girls] participated. Using the Dutch Personality Questionnaire Juniors (DPQ-J), we assessed 20 of the CWE shortly before epilepsy surgery and compared the results to those of 39 age- and gender-matched healthy controls. Furthermore, we obtained follow-up scores 6, 12 and 24 months after epilepsy surgery from the clinical group. CWE who were candidates for epilepsy surgery scored above average in inadequacy, perseverance, social inadequacy and recalcitrance, whereas healthy peers scored average. Over the two years' period after epilepsy surgery we found no changes, apart from a decrease of social inadequacy. Sporadic illness and demographic variables were related to some personality traits. However, neither post-surgical seizure freedom nor cessation of AEDs did noticeably change the self-evaluations of CWE. Asking CWE to evaluate their personality themselves may offer a shared basis for individually tailored behavior intervention in order to help them adapting to their ameliorated circumstances after surgery.

Purpose To evaluate cognition, behavior, daily functioning and health-related quality of life (HrQoL) five years to more than a decade after hemispherectomy (HE) in childhood. Methods This countrywide Dutch cohort study of 31 patients, who underwent HE between 1994 and 2009, included a semi-structured interview with parents, an assessment of cognition, and screening of behavioral problems and HrQoL. Results Twenty-two school-age children and young adults [median age 13.8 years (0.5 at epilepsy onset, 5.3 at HE)] were assessed with age-appropriate cognitive tests. IQ ranged from 45 to 82 (median 61). Despite performing below mean norm scores, these participants could learn and remember, sustain attention, inhibit irrelevant responses, read and write. Nine more children [median age 9.7 years (0.25 at epilepsy onset, 1.4 at HE)] were so mentally retarded that age-appropriate testing was impossible. This group was almost totally dependent on others in daily activities, had the highest proportion of pre-existing contralateral MRI-abnormalities and after HE the highest rates of seizure recurrence and behavioral problems. Parents in both groups rated HrQoL surprisingly positively (mean VAS-score 72.5), with a scarce low rating (40). All parents reported problems with respect to their children's self-care, daily activities and mobility. Conclusion At least five years after HE, cognitive, behavioral and daily functioning encompasses a broad spectrum that varies from profound retardation and almost total dependence to low normal cognition and a reasonably independent existence. Pre-existing contralateral MRI abnormalities reflect a more generally affected brain with a limited ability to mediate development after HE.

Objective To determine the prevalence of motor impairments and activity limitations and their inter-relationships in Dutch children with spastic cerebral palsy. Patients and methods In a population-based survey 119 children, age range 6-19 years, with spastic cerebral palsy were examined. Anthropometry, muscle tone, abnormal posture, joint range of motion, major orthopaedic impairments and gross motor functioning and manual ability were assessed or classified, in addition to limitations in mobility and self-care activities. Spearman's correlation coefficients, bivariate post hoc analyses and univariate and multivariate logistic regression analyses were used. Results Children with spastic cerebral palsy had a lower body height and weight compared with typically developing peers. Forty percent had no range of motion deficits. Hip dislocations were rarely encountered. Motor impairments were associated with gross motor functioning and manual ability levels. Close to sixty-five percent walked independently. Children with diplegia and tetraplegia differed in activity limitations. Motor impairments and limitations in mobility and self-care activities were only modestly related in multivariate analyses. Conclusion Distribution of cerebral palsy-related characteristics is consistent with that found in representative studies of other countries. The distinction between diplegia and tetraplegia is relevant from an activity point of view. The child's activity limitations are not a mirror of the motor impairments, which suggests multifactorial influences. An activity-oriented rehabilitation approach goes beyond treating specific impairments.

Temporal lobe epilepsy (TLE) is one of the most common focal epilepsy syndromes. In a genome-wide expression study of the human TLE hippocampus we previously showed up-regulation of genes involved in chemokine signalling. Here we investigate in the rat pilocarpine model for TLE, whether changes in chemokine signalling occur during epileptogenesis and are persistent. Therefore we analysed hippocampal protein expression and cellular localisation of CCL2, CCL4, CCR1 and CCR5 after status epilepticus. We found increased CCL4 (but not CCL2) expression in specific populations of hilar astrocytes at 2 and 19weeks after SE concomitant with a persistent up-regulation of its receptor CCR5. Our results show an early and persistent up-regulation of CCL4/CCR5 signalling during epileptogenesis and suggest that CCL4 signalling, rather than CCL2 signalling, could have a role in the epileptogenic process.