Cognitive and behavioural findings in children with frontal lobe epilepsy

BackgroundFrontal Lobe Epilepsy (FLE) is the second most frequent type of partial epilepsy and its onset is generally in childhood. Though cognitive and behavioural impairments have been described as co-morbid disorders in epilepsy, their extent in FLE, particularly in children, remains unknown.AimsIn this study, we assess cognitive skills and behaviour in a cohort of paediatric FLE patients.MethodsWe measured the performance of 71 children with cryptogenic FLE on intelligence tests, neuropsychological tests, and behavioural questionnaires. Age-dependent normative values were used for reference. Results were related to epilepsy-factors including age at epilepsy onset, duration of epilepsy, seizure frequency, localisation of the epileptic focus and drug load.ResultsPaediatric FLE patients performed worse on intellectual and neuropsychological tests compared to reference values, and had a delay in school achievement. The performance of patients was typically worse on tasks measuring visual-spatial functions, memory, psychomotor speed and alertness. High seizure frequency was associated with lower scores on the arithmetic subtest of the intelligence scale; the other epilepsy-factors had no statistically significant influence on intelligence test or neuropsychological test outcome. Behavioural problems included attention problems, anxiety and internalising behaviour. These were not significantly related to epilepsy-factors.ConclusionsChildren with cryptogenic FLE show a broad range of cognitive and behavioural impairments, compared to reference values. While high seizure frequency may affect performance on selected cognitive measures, other epilepsy-factors do not seem to influence cognition and behaviour. Study of micro-structural or functional brain abnormalities that underlie these cognitive and behavioural impairments are warranted.     

Seizure and cognitive outcomes of epilepsy surgery in infancy and early childhood

AimsTo investigate seizure and developmental outcomes following epilepsy surgery in very young children and determine their predictive factors.MethodsWe retrospectively reviewed the clinical data, surgical variables, and outcomes of 30 children under 3 years of age that underwent resection for refractory focal epilepsy in our institution in 2001–2011.ResultsSeizure onset was in the first year of life in 27 (90%) cases and mean age at surgery was 20 months (range 5–33.6). Pathology consisted of cortical malformations in 24 (80%) cases, glioneuronal tumour and infarction with or without cortical dysplasia in three (10%) cases each. Morbidity was comparable with older paediatric cohorts. At 1–11.6 year follow-up (mean 4.1) 21 of 30 (70%) children achieved seizure freedom (Engel I), six (20%) demonstrated worthwhile improvement (Engel II/III) and three (10%) did not benefit from surgery (Engel IV). Intralobar lesionectomy more often resulted in seizure freedom than multilobar or hemispheric surgery. The abundance of non-regional interictal and ictal EEG findings did not preclude seizure freedom. Presurgical developmental impairment was established in 25 of 28 (89%) children; its severity correlated with longer epilepsy duration and determined postoperative developmental outcome. Developmental progress was established in 26 out of 28 (93%) children following surgery, showing stabilized trajectories rather than catch-up.ConclusionsResective surgery in very young children is safe and effective in terms of seizure control and developmental progress. Our findings underline the importance of early intervention in order to timely stop seizures and their deleterious effects on the developing brain.     

Impairments, disabilities and handicaps in children and adolescents with epilepsy

The patterns of motor and sensory impairments, disabilities and handicap were assessed in 217 children and adolescents with epilepsy. Motor and sensory dysfunctions were found to be common even in children without major additional neurodisorders such as cerebral palsy and mental retardation. Handicap was most severe in the dimensions of physical independence and orientation and increased significantly with duration of epilepsy. It was more severe when the onset of seizures was early and when secondary generalized seizures were present. Handicap was significantly reduced after epilepsy surgery.     

Epilepsiechirurgie im Kindes- und Jugendalter

Epilepsy surgery in children and adolescents is an effective procedure, which offers a 70% chance of postoperative freedom from seizures in drug resistant focal epilepsies. The best chance of postoperative seizure freedom is after temporal resections and hemispherectomies, the least in extratemporal and multilobular resections. A single, potentially epileptogenic morphological lesion increases the chance of postoperative seizure freedom, whereas any electrophysiological or morphological sign of bilateral or multifocal aetiology decreases this chance. The plasticity of the young brain allows extensive operative procedures like functional hemispherectomies without significant cognitive impairments. In addition to a reduction of seizure frequency there is a chance to improve the cognitive and possibly the psychosocial prognosis. Mortality and morbidity of epilepsy surgery does not surpass that of medically treated severe epilepsy in a long-term course.     

Epilepsy: knowledge and attitudes of physiotherapists,occupational therapists,and speech therapists

Physiotherapists, occupational therapists, and speech therapists play a key role in the treatment of children with epilepsy. We performed a survey of therapists’ knowledge of and attitudes towards epilepsy in two regions of Germany, the city of Leipzig and the rural district of Zwickau. Therapists of 29/68 (43%) outpatient practices and 4/9 (44%) hospitals took part. In total, 195 therapists participated: 63 (32%) physiotherapists, 74 (38%) occupational therapists, and 58 (30%) speech therapist. In 65%, epilepsy was subject of vocational training. Of all therapists, 8% claimed they had not treated epilepsy patients so far. During professional life, 43% had witnessed a seizure. Of all therapists, 44% correctly assumed a seizure could result in death. During a seizure, 42% would perform the obsolete measure of placing something solid in the patient’s mouth, and 41% would administer a prescribed rescue medication. More information on epilepsy was requested by 92%.

Conclusion: Most therapists treat patients with epilepsy, and almost half have already witnessed a seizure. Often, however, epilepsy is not subject of vocational training. The risk of a fatal outcome of a seizure is underestimated, and many therapists would perform obsolete measures. Knowledge of seizure management should be transmitted to therapists especially during vocational training.

     

Long-term outcome of resective epilepsy surgery in Norwegian children

Aim: The aim of this study was to evaluate the long-term results of resective surgery on children with difficult-to-treat epilepsy in Norway. Methods: In the period 1995–2004, 64 surgical procedures (54 resections and 10 functional hemispherotomies) were performed in 54 children. The children’s medical records were retrospectively reviewed at a minimum of 2 years after surgery. We sent a questionnaire regarding their epilepsy (seizures, usage of antiepileptic drugs) and general functioning (social situation, motor, language, cognition, behavioural or emotional problems, any remedial action) to the children/parents after a mean follow-up period of 7 years. Results: 55.5% of the children were seizure-free. The success rate varied according to the type of surgery. Best results were found after functional hemispherotomies and temporal lobe resections, as nine of 10 (90%) and 10 of 19 (53%) of these patients, respectively, became seizure-free. In addition to a better seizure control, 71% of the children/parents reported of a better cognitive and psychosocial functioning. Conclusion: The results of epilepsy surgery in this paediatric cohort are very edifying, and it is our impression that this treatment option is underused in Norway.     

Vagus nerve stimulation for partial and generalized epilepsy from infancy to adolescence

Object Vagus nerve stimulation (VNS) is approved by the FDA for the treatment of partial epilepsy in patients older than 12 years. Authors of the current study performed a large retrospective analysis and comparison of VNS outcomes in children with an age ≥ and < 12 years, including those with partial and generalized epilepsy. Methods A retrospective review of the records of pediatric patients (age < 18 years) who had undergone primary VNS system implantation between 2001 and 2010 by a single pediatric neurosurgeon was undertaken. Considered data included demographics, epilepsy type (partial vs generalized), seizure frequency, seizure duration, postictal period duration, and antiepileptic medication use. Results One hundred forty-six patients (49% female) were followed up for a mean of 41 months after VNS implantation. Thirty-two percent of patients had partial epilepsy and 68% had generalized epilepsy. After VNS system implantation, seizure frequency was reduced in 91% of patients, seizure duration in 50%, postictal period in 49%, and antiepileptic medication use in 75%. There was no significant difference in age, sex, or duration of follow-up according to epilepsy type. Neither was there any significant difference in seizure frequency reduction, seizure duration, postictal period, medication use, overall clinical improvement, or improvement in quality of life based on an age ≥ or < 12 years or epilepsy type. Conclusions Vagus nerve stimulation reduced both seizure frequency and antiepileptic medication use in the majority of pediatric patients regardless of sex, age cohort, or epilepsy type. Vagus nerve stimulation also reduced seizure duration and postictal period in approximately half of the pediatric patients. Contrary to expectation, children with partial epilepsy do not benefit from VNS at higher rates than those with generalized epilepsy.     

Social and medical care of preschool children with epilepsy in Croatia: Population-based survey

BackgroundEarly detection of mental retardation and other epilepsy-associated impairments is essential for successful medical and social care of children with epilepsy; the corresponding information for children in Croatia has not yet been known.Aims of the studyTo obtain the basic information of epilepsy-associated disability in preschool children, and fundamentals of their medical and social care.MethodsData about mental retardation and other associated impairments (motor, speech, seeing, hearing), antiepileptic drug therapy and diurnal residence were collected by means of questionnaires completed by physicians working in primary health care (PHPs). Only children (0–7 years) with active epilepsy confirmed previously by neuropaediatricians were included.ResultsA total of 37 PHPs provided the required data for 116 children. One or more impairments were found in 56% children; most frequent were motor impairments (47%), speech impairments (42%) and mental retardation (40%). The regular kindergarten attendance rate of children without impairment (33%) was not different from the children without epilepsy, but high proportion (76%) of children with impairment stayed with their families during weekdays. In this subgroup monotherapy was more rarely used (64% vs. 90% in children without impairment (p < 0.01)). Valproate was predominantly used (56%) in children with and without impairment; lamotrigine was more frequently used in the former subgroup (p < 0.01).ConclusionsExistence of associated impairments has significant impact on medical and social care in preschool children with epilepsy. These children need an early diagnosis and consecutive multidisciplinary care of their intellectual and body impairments, as well as problems in social development.     

局灶运动抑制性发作5例患儿临床特征及脑电图分析

目的 探讨局灶运动抑制性癫痫儿童的临床及电生理特点,提高对此类癫痫的认识.方法 回顾性分析2015年1月至2019年4月首都医科大学宣武医院儿科住院收治的5例有局灶运动抑制性癫痫患儿脑电图特点、临床特征和治疗情况.结果 5例患儿中男4例、女1例,起病年龄从2岁3月龄至9岁不等,5例均表现为一侧肢体无力,发作期间意识清楚...     

Progression from first symptom to diagnosis in childhood brain tumours

This study was undertaken to investigate the evolution of clinical features between onset of symptoms and diagnosis in children with brain tumours and to identify ways of shortening the time to diagnosis. One hundred and thirty-nine children with a brain tumour were recruited from four UK paediatric neuro-oncology centres. Children had a median of one symptom or sign at symptom onset and six by diagnosis. The symptoms and/or signs experienced at symptom onset and at diagnosis were as follows: headache in 55 and 81 children, nausea and vomiting in 39 and 88 children, motor system abnormalities in 31 and 93 children, cranial nerve palsies in 24 and 75 children, visual system abnormalities in 23 and 96 children, endocrine or growth abnormalities in 10 and 35 children and behavioural change in 4 and 55 children. The median time between symptom onset and diagnosis (symptom interval) was 3.3 months. A longer symptom interval was associated with head tilt, cranial nerve palsies, endocrine and growth abnormalities and reduced visual acuity. More than half of children with brain tumours developed problems with vision and more than a third developed motor problems, cranial nerve palsies, behavioural change, or nausea and vomiting between symptom onset and diagnosis.     

Learning disabilities in a population-based group of children with hydrocephalus

AIM: To assess cognitive functions in a population-based group of children with hydrocephalus and to analyse differences between children with and without myelomeningocoele (MMC); with hydrocephalus already present at birth and those who developed it later; children born at full term and those born preterm. METHODS: Of 103 children with hydrocephalus born in the western-Swedish region in 1989-1993, 73 were assessed using the Wechsler Intelligence Scales or the Griffith Developmental Scales. RESULTS: One-third of the children were normally gifted (IQ >85), another 30% had a low average IQ of 70-84 and 37% had learning disabilities with an IQ of <70. The median IQ was 75, verbal IQ 90 and performance IQ 76 (p<0.001). An IQ of <70 was found in 19 of 45 (42%) children without MMC and in 8 of 28 (29%) of those with MMC. Children born preterm had a lower IQ of 68 than those born at full term with an IQ of 76, while children with hydrocephalus present at birth but without MMC had a lower IQ of 60 than the others with an IQ of 77. Children with cerebral palsy and/or epilepsy (n=22) had a lower IQ of 66 than the IQ of 78 in those without these impairments (p<0.01). CONCLUSION: Cognitive functions in children with hydrocephalus need to be carefully assessed before school age to ensure adequate support and education. Even the one-third near normally gifted children with an IQ of 70-85 require special attention, due to the profile of relatively well-preserved verbal functions but greatly impaired perceptual and non-verbal abilities.     

Vagal nerve stimulation in refractory epilepsy: the first 100 patients receiving vagal nerve stimulation at a pediatric epilepsy center

OBJECTIVE: To determine the outcome of intermittent left vagal nerve stimulation on the first 100 consecutive patients treated at our pediatric epilepsy center. METHODS: Patients were identified by means of operating room records. Data collected described the patient's epilepsy, previous and subsequent therapies, adverse events, nonepileptic changes, and outcomes. RESULTS: Average age was 10.4 years; years of epilepsy, 8.5; total number of antiepileptic therapies, 8.4; and median monthly seizure frequency, 120. Data on seizure frequency at follow-up were available for 96 of the 100 patients. Forty-five percent of patients achieved greater than 50% reduction; and 18% had had no seizures for the last 6 months. Response was similar in patients with more than 7 years of refractory epilepsy as compared with patients with a shorter history. Magnet-generated, on-demand current reduced seizure intensity in almost half of the patients with available data. Generator infections occurred in 3 patients. Twenty-four patients had their generators removed. Subsequently, 2 of these patients died. CONCLUSIONS: Seizure reduction was the same in patients younger than 12 years and 12 years or older and in patients with shorter and longer histories of refractory epilepsy. Adverse effects were few in this population, particularly in those younger than 12 years. Vagal nerve stimulation appears to be a relatively safe and potentially effective treatment for children with severely intractable epilepsy.     

Utility of brainstem auditory evoked potentials in children with spastic cerebral palsy

In a retrospective study of 75 children with spastic cerebral palsy (CP), brainstem auditory evoked potentials (BAEP) were recorded and subsequently correlated with birthweight, gestational age, aetiology and type of CP, neuroradiological findings, additional impairments and disabilities (including the inability to walk independently). Seventeen patients (22.7%) had abnormal BAEP recordings. Thirteen of these 17 patients (76.5%) had spastic tetraplegia, 16 patients (94.1%) were full-term infants, 12 patients (70.6%) had myoskeletal problems, 9 (52.9%) had epilepsy, 16 (94.1%) had visual impairment, 13 patients (76.5%) were unable to walk independently, while all 17 patients (100%) had speech impairment and mental retardation. The aetiology of CP was prenatal in 2 of these 17 patients (11.8%) and perinatal in 15 patients (88.2%). Thirteen patients (76.5%) had cortical atrophy determined by either computed tomography or magnetic resonance imaging, two patients (11.8%) had an infarct picture and two patients (11.8%) had maldevelopment of the central nervous system. There was a definite statistically significant association between abnormal BAEP recordings and full-term delivery, perinatal aetiology of CP, spastic tetraplegia, speech, visual and myoskeletal impairments, epilepsy, mental retardation, inability to walk independently and cortical atrophy on neuroimaging (p < 0.001). We conclude that abnormal BAEP recordings in children with spastic CP are indicative of poor prognosis and associated with a "multihandicap state". BAEP testing should be incorporated into the diagnostic plan of all children with spastic CP newly referred to neurodevelopmental centres.     

Antecedents of seizure disorders in early childhood

One or more nonfebrile seizures occurred between the ages of 1 month and 7 years in eight per 1000 white and in nine per 1000 black children enrolled in a large prospective study. We examined hundreds of prenatal and perinatal factors as predictors of childhood seizure disorders. Congenital malformations of the fetus (cerebral and noncerebral), family history of certain neurologic disorders, and neonatal seizures were the major predictors identified. Forty percent of children with postneonatal seizures and 68% of children with minor motor seizures had one or more of these risk factors compared with 21% of the seizure-free population. More than half of the children with minor motor seizures and a third of the infants with neonatal seizures had congenital malformations. Based on the prenatal and perinatal factors examined, prediction of postneonatal seizures carried a high rate of false-positive identification, indicating that our knowledge of the etiology of childhood seizure disorders is still very limited.     

Efficacy and safety of lamotrigine monotherapy in children and adolescents with epilepsy

Lamotrigine (LTG) has shown to confer broad-spectrum, well-tolerated control of epilepsy. Monotherapy is preferable over polytherapy because of better compliance, fewer adverse events, less interactions, lower teratogenicity and lower cost. The aim of this study is to evaluate the efficacy and safety of LTG monotherapy on seizure control in a cohort of children and adolescents with epilepsy.We retrospectively reviewed the records of children and adolescents treated with LTG monotherapy at our institution between 2001 and 2006. Data collected included demographics, seizure type, etiology of seizures, age at onset of seizures and at initiation of LTG treatment, number of antiepileptic drugs (AEDs) prior to LTG, dose of LTG, length of follow-up, treatment response, and adverse events.Seventy-two children and adolescents were identified (mean age 12.1 years); 37.5% had mental retardation. Age at onset of epilepsy was 5.7 years (0–16). Twenty three percent had symptomatic focal epilepsy, 15.5% idiopathic focal epilepsy, 19.4% symptomatic generalized epilepsy and 41.6% idiopathic generalized epilepsy. LTG was used as first-line monotherapy in 26.4% of patients and as a second-line monotherapy in 73.6%. Age at initiation of LTG therapy was 10 years (2.8–19). Mean number of AEDs tried prior to LTG was 1.3 (0–6). Mean dose of LTG was 5.5 mg/kg/day (1.1–13.7). Mean follow-up period was 33 months (3 weeks to 11.5 years).The degree of seizure reduction was as follows: seizure free in 42%, 75–90% reduction in 17.4%, 50–74% in 11.6%, 25–49% in 10%. Sixteen percent had no change in seizure control and 3% became worse. The most common adverse event was rash (6.9%). Six (8.3%) patients discontinued LTG because of the adverse events. No patient had Stevens–Johnson syndrome.In conclusion, LTG was effective and well-tolerated as monotherapy in children and adolescents for both focal and generalized epilepsies.     

SOCIAL ADJUSTMENT AND FUNCTIONING OF CHRONICALLY ILL AND IMPARED CHILDREN AND ADOLESCENTS

A cluster sample of 532 children aged 8 to 19 years with certain impairments were compared to a matched sample of 316 healthy children of the same age. Almost half (46 %) of the impaired had a disability, which was mostly in the domain of body disposition ability (in 88 %), behaviour (63 %) and situational functioning (54 %). In the controls, where 12 % had some kind of disability, the corresponding figures were 13 %, 11 % and 15 %. The most disabling conditions were CP (67 % handicapped), MBD (52 %) and epilepsy (42 %). The most frequent non-compènsated handicaps were those of occupation (44 % of impaired), mobility (31 %) and social integration (22 %). More than one handicap was associated most frequently with CP (76 %) and with epilepsy (36 %). Neurological impairments are more disabling and handicapping than non-neurological ones, with the exception of mobility and occupation handicaps in asthmatics.     

Prediction of outcome at 5 years from assessments at 2 years among extremely preterm children: a Norwegian national cohort study

Aim: To examine the predictive value of early assessments on developmental outcome at 5 years in children born extremely preterm. Methods: This is a prospective observational study of all infants born in Norway in 1999–2000 with gestational age (GA) <28 weeks or birth weight (BW) <1000 g. At 2 years of age, paediatricians assessed mental and motor development from milestones. At 5 years, parents completed questionnaires on development and professional support before cognitive function was assessed with Wechsler Preschool and Primary Scale of Intelligence‐Revised (WPPSI‐R) and motor function with the Movement Assessment Battery for children (ABC test). Results: Twenty‐six of 373 (7%) children had cerebral palsy at 2 and 29 of 306 (9%) children at 5 years. Of children without major impairments, 51% (95% CI 35–67) of those with and 22% (95% CI 16–28) without mental delay at 2 years had IQ <85 at 5 years, and 36% (95% CI 20–53 with and 16% (95% CI 11–21) without motor delay at 2 years had an ABC score >95th percentile (poor function). Approximately half of those without major impairments but IQ <85 or ABC score >95th percentile had received support or follow‐up beyond routine primary care. Conclusion: Previous assessments had limited value in predicting cognitive and motor function at 5 years in these extremely preterm children without major impairments.     

Role of surgery in pediatric epilepsy

Twenty five percent of patients with intractable epilepsy have surgically remediable epilepsy syndromes. This article reviews the treatment paradigm for pediatric epilepsy and also the indications, methods, and surgical options for the subgroup of patients with surgically remediable epileptic disorders based on our experience in the management of these children. The article also discusses the rationale for offering surgery and the timing of surgery in these patients. The study of surgically remediable epilepsy can best be divided into focal, sub hemispheric, hemispheric and multifocal epileptic syndromes. These syndromes have both acquired and congenital etiologies and can be treated by resective or disconnective surgery. The surgical management of these conditions (with the exception of multifocal epilepsy) provides Engel's Class 1 outcome(complete seizure freedom) in approximately 80% of children. The consequences of seizure freedom leads to a marked improvement in the quality of life of these children.The benefits to society, of allowing a child to grow to adulthood with normal cognition to earn a livelihood and contribute actively to society, cannot be understated.     

Corpus callosotomy for treatment of pediatric epilepsy in the modern era

OBJECTIVE: The purpose of this study was to evaluate seizure outcome in children with intractable secondary generalized epilepsy without a resectable focus who underwent complete corpus callosotomy and compare these results to those of anterior two-third callosotomy. METHOD: Data were obtained for all patients who underwent a corpus callosotomy from 2000 to 2005. The study involved 37 patients. Eleven patients had anterior two-third corpus callosotomy compared with 28 patients who underwent complete corpus callosotomy. Two of these patients had completion of their callosotomy following initial partial callosotomy. Seizure type, seizure frequency, and family satisfaction were evaluated for all patients pre- and postoperatively. RESULTS: A reduction of >or=75% in seizures occurred in 75% of the total-callosotomy patients compared to 55% of the partial-callosotomy patients. Family satisfaction for complete and partial callosotomy was 89 and 73%, respectively. No prolonged neurologic deficits were observed in either group. CONCLUSION: Complete corpus callosotomy is the most effective treatment for secondary generalized intractable seizures not amenable to focal resection in children.     

Resective epilepsy surgery in childhood: the Dutch experience 1992-2002.

PURPOSE: We present the outcome of resective epilepsy surgery in 69 pediatric patients who participated in the Dutch Collaborative Epilepsy Surgery Program (DCESP) between 1992 and 2002 with special emphasis on long-term follow-up. METHODS: Sixty-nine children (aged 3 months to 17 years) operated on before 2003 were included in this study (34 temporal resections (49%), 17 extra-temporal resections (24%) and 19 hemispherectomies (27%)). Engel classification was used to assess seizure outcome annually. Cognitive outcome was assessed if possible. Two telephone surveys were carried out with an interval of 2(1/2) years to obtain data on seizure frequency, use of AEDs and on aspects op psychosocial development. Kaplan-Meier survival curves were constructed to assess recurrence of seizures after initial postsurgical seizure freedom, based on both telephone surveys. RESULTS: Seventy percent scored Engel 1, 18% Engel 2, 6% Engel 3 and 6% Engel 4 at the time of the first telephone survey (2(1/2) years later: 77% Engel 1, 8% Engel 2, 12% Engel 3 and 3% Engel 4). Temporal resections were associated with the best seizure outcome (Engel 1 74% and 82%). AEDs could be withdrawn successfully in 53% of patients at time of the last follow-up. No negative impact on cognition was found. The first long-term follow-up (mean 4.5 years after surgery) measurement showed recurrence of seizures after initial seizure freedom in 17%. At time of the second long-term follow-up measurement (mean 7.5 years after surgery) this percentage had increased to 21%. CONCLUSIONS: Our results support previous reports that surgery for intractable epilepsy in pediatric patients can be safely performed with satisfactory long-term results. Best results are attained in temporal resections.