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BACKGROUND: Drug-resistant epilepsy (DRE) affects the development and quality of life of children and young adults. We analyzed the effectiveness and safety of purified CBD in this population. METHODS: A retrospective analysis of medical records of 139 children and young adults (54.7% female, median age 12.0 years) with DRE treated with purified CBD from 2018 to 2022 at five medical centers in Israel. RESULTS: The most common diagnosis was Lennox-Gastaut syndrome (37.4%) followed by Dravet syndrome (16.5%) and tuberous sclerosis complex (16.5%). Median purified CBD dose was 12.5 mg/kg (range 2.5 to 20.0), and median treatment duration was 9.0 months (range 0.5 to 48.0). Most patients (92.2%) had a reduced seizure frequency following treatment initiation; 41.1% had >50% reduction. Fifty-three patients (38.1%) had positive effects: improved alertness (31.7%), improved speech (10.1%), and achievement of new developmental milestones (2.2%). A multivariate linear model assessing predictive factors for seizure reduction demonstrated that patients previously treated with CBD oils, especially those with >50% seizure reduction on prior treatment, were also more likely to have a reduced seizure frequency while they were treated with purified CBD (P = 0.01, P < 0.0001). Development, diagnosis, age, purified CBD dose (0 to 10 mg/kg/day vs 10 to 20 mg/kg/day), and concomitant treatment with clobazam, valproic acid, or everolimus did not affect seizure reduction by purified CBD. The most common adverse events were irritability (20.9%) and drowsiness (12.9%). CONCLUSION: Purified CBD is well-tolerated and effective in reducing seizure frequency in children and young adults with DRE.
Subject(s)
Cannabidiol , Drug Resistant Epilepsy , Lennox Gastaut Syndrome , Child , Young Adult , Humans , Female , Male , Cannabidiol/adverse effects , Drug Resistant Epilepsy/drug therapy , Drug Resistant Epilepsy/diagnosis , Anticonvulsants/therapeutic use , Retrospective Studies , Quality of Life , Seizures/drug therapy , Lennox Gastaut Syndrome/drug therapyABSTRACT
PURPOSE: Epilepsy surgery for pediatric drug-resistant epilepsy has been shown to improve seizure control, enhance patient and family QoL, and reduce mortality. However, diagnostic tools and surgical capacity are less accessible worldwide. The International Society Pediatric Neurosurgery (ISPN) has established a Pediatric Epilepsy Surgery Interest Group (PESIG), aiming to enhance global collaboration in research and educational aspects. The goals of this manuscript are to introduce PESIG and analyze geographical differences of epilepsy surgery and technology availability. METHODS: PESIG was established (2022) following an ISPN executive board decision. Using a standardized form, we surveyed the PESIG members, collecting and analyzing data regarding geographical distribution, and availability of various epilepsy treatment-related technologies. RESULTS: Two hundred eighty-two members registered in PESIG from 70 countries, over 6 continents, were included. We categorized the countries by GDP as follows: low, lower-medium, upper-medium, and high income. The most commonly available technology was vagus nerve stimulation 68%. Stereoelectroencephalography was available for 58%. North America had statistically significant greater availability compared to other continents. Europe had greater availability compared to Africa, Asia, and South (Latin) America. Asia had greater availability compared to Africa. High-income countries had statistically significant greater availability compared to other income groups; there was no significant difference between the other income-level subgroups. CONCLUSION: There is a clear discrepancy between countries and continents regarding access to epilepsy surgery technologies. This strengthens the need for collaboration between neurologists and neurosurgeons from around the world, to enhance medical education and training, as well as to increase technological availability.
Subject(s)
Epilepsy , Neurosurgery , Humans , Child , Neurosurgery/education , Quality of Life , Public Opinion , Neurosurgical Procedures , Epilepsy/surgeryABSTRACT
OBJECTIVE: Lennox-Gastaut syndrome (LGS) is a severe form of epileptic encephalopathy, presenting during the first years of life, and is very resistant to treatment. Once medical therapy has failed, palliative surgeries such as vagus nerve stimulation (VNS) or corpus callosotomy (CC) are considered. Although CC is more effective than VNS as the primary neurosurgical treatment for LGS-associated drop attacks, there are limited data regarding the added value of CC following VNS. This study aimed to assess the effectiveness of CC preceded by VNS. METHODS: This multinational, multicenter retrospective study focuses on LGS children who underwent CC before the age of 18 years, following prior VNS, which failed to achieve satisfactory seizure control. Collected data included epilepsy characteristics, surgical details, epilepsy outcomes, and complications. The primary outcome of this study was a 50% reduction in drop attacks. RESULTS: A total of 127 cases were reviewed (80 males). The median age at epilepsy onset was 6 months (interquartile range [IQR] = 3.12-22.75). The median age at VNS surgery was 7 years (IQR = 4-10), and CC was performed at a median age of 11 years (IQR = 8.76-15). The dominant seizure type was drop attacks (tonic or atonic) in 102 patients. Eighty-six patients underwent a single-stage complete CC, and 41 an anterior callosotomy. Ten patients who did not initially have a complete CC underwent a second surgery for completion of CC due to seizure persistence. Overall, there was at least a 50% reduction in drop attacks and other seizures in 83% and 60%, respectively. Permanent morbidity occurred in 1.5%, with no mortality. SIGNIFICANCE: CC is vital in seizure control in children with LGS in whom VNS has failed. Surgical risks are low. A complete CC has a tendency toward better effectiveness than anterior CC for some seizure types.
Subject(s)
Epilepsy , Lennox Gastaut Syndrome , Vagus Nerve Stimulation , Child , Male , Humans , Infant , Child, Preschool , Adolescent , Lennox Gastaut Syndrome/surgery , Retrospective Studies , Corpus Callosum/surgery , Seizures/therapy , Syncope , Treatment Outcome , Vagus NerveABSTRACT
Epilepsy and attention-deficit/hyperactivity disorder (ADHD) are closely connected and commonly seen in both children and adults. Each of the disorders has major psychosocial and quality of life (QOL) effects, and their co-occurrence makes coping even more challenging for both the patients and their families. Moreover, an adverse effect of some anti-seizure medications can potentially induce or exacerbate symptoms of ADHD on the one hand, while some ADHD medications may increase seizure risk on the other. The combination of proper diagnosis and appropriate treatment may improve or even prevent many of the complications associated with these conditions. The objectives of this review are to present the complex relationship between epilepsy and ADHD, looking into the pathophysiological, anatomical, and functional perspectives along with the psychosocial and QOL influences and the recommended treatment approaches in accordance with the latest literature.
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INTRODUCTION: Drug-resistant epilepsy in children is associated with morbidity, developmental regression and mortality. Over recent years, there is an increase in awareness regarding the role of surgery in the treatment of refractory epilepsy, both in the diagnostic phase and for treatment, reducing the number and magnitude of seizures. Technological advancements have enabled a minimalization of surgery, with reduction in surgical associated morbidity. METHODS: In this retrospective study, we review our experience with cranial surgery for epilepsy between the years 2011-2020. Collected data included information regarding the epileptic disorder, surgery, surgical-related complications and epilepsy outcome. RESULTS: A total of 93 children underwent 110 cranial surgeries over a decade. The main etiologies included cortical dysplasia (29), Rasmussen encephalitis (10), genetic disorders (9), tumors (7) and tuberous sclerosis (7). The main surgeries included lobectomies (32), focal resections (26), hemispherotomies (25), and callosotomies (16). Two children underwent MRI-guided laser interstitial thermal treatment (LITT). The most significant improvements following surgery were following hemispherotomy or tumor resection (100% of children, each). Following resections for cortical dysplasia led to a significant improvement in 70%. In 83% of children undergoing callosotomy, there were no additional drop seizures; 14% of the entire group underwent additional epilepsy surgery; 23% of children had an unexpected complication, in the vast majority with no permanent sequela. There was not mortality. CONCLUSIONS: Epilepsy surgery may lead to significant improvement and even cure of epilepsy. There is a wide span of epilepsy surgical procedures. Ealy referral of children with refractory epilepsy for surgical evaluation may significantly reduce the developmental injury, and improve functional outcomes.
Subject(s)
Drug Resistant Epilepsy , Epilepsy , Malformations of Cortical Development , Child , Humans , Drug Resistant Epilepsy/etiology , Drug Resistant Epilepsy/surgery , Epilepsy/etiology , Epilepsy/surgery , Malformations of Cortical Development/complications , Retrospective Studies , Seizures , Treatment OutcomeABSTRACT
BACKGROUND: West syndrome is a convulsive disorder of infancy with unique seizures and a characteristic background electroencephalograph pattern. Adrenocorticotropic hormone (ACTH) is effective in spasm cessation, yet metabolic consequences of this therapeutic agent in childhood have not been published. METHODS: In this observational study we explored the cardiometabolic outcomes of 117 children with West syndrome (78 ACTH-treated and 39 non-ACTH-treated) monitored at a single medical center from 1995 to 2019 (median follow-up 7.2 years). Outcomes included the prevalence of cardiometabolic derangements (obesity, hypertension, and dyslipidemia) during infancy (< 2 years), early childhood (2-6 years), and childhood/adolescence (6-18 years). RESULTS: The rates of metabolic derangements during infancy in the West syndrome cohort were high compared to childhood/adolescence (obesity 27.3 % vs. 3.3 %, [p = 0.010], diastolic hypertension 48.8 % vs. 5.1 % [p < 0.001], hypertriglyceridemia 71 % vs. 40 % [p = 0.008], low high-density lipoprotein cholesterol [HDL-c] 54.2 % vs. 12.9 % [p = 0.001], and elevated triglycerides/HDL-c ratios 62.5 % vs. 12.9 % [p < 0.001]). The proportion of systolic and/or diastolic blood pressure levels categorized as hypertensive was 58.5 % during infancy, 48.1 % during early childhood, and 26.3 % during childhood/adolescence. ACTH-treated patients had higher weight and weight-to-length z-scores and higher triglyceride levels during infancy compared to non-ACTH-treated patients (p = 0.008, p = 0.001, and p = 0.037, respectively), and higher triglyceride levels during early childhood (p = 0.050), with no significant group differences during childhood/adolescence. CONCLUSIONS: Children with West syndrome apparently have an increased prevalence of cardiometabolic derangements more pronounced in infants and in ACTH-treated patients. These findings highlight the need to monitor these children for cardiometabolic derangements, even though these cardiometabolic abnormalities are transitory and tend to decrease with time. The health implications of cardiometabolic derangements during critical windows of growth and development warrant further investigation.
Subject(s)
Hypertension , Metabolic Syndrome , Spasms, Infantile , Adolescent , Child , Child, Preschool , Humans , Hypertension/epidemiology , Infant , Metabolic Syndrome/epidemiology , Obesity , Overweight , Risk Factors , Spasms, Infantile/drug therapy , Spasms, Infantile/epidemiologyABSTRACT
BACKGROUND: The objective of this study was to assess the prevalence of disordered eating behaviors (DEBs) in young individuals with idiopathic intracranial hypertension (IIH) and to identify predictors of DEBs in this population. METHODS: Individuals with IIH aged eight to 25 years and their matched controls responded to a self-rating survey comprised of the Eating Attitude Test-26 for assessing the presence of DEBs and the Depression, Anxiety and Stress Scale. RESULTS: Fifty-three subjects with IIH and 106 healthy controls were included. DEBs were significantly more prevalent in individuals with IIH (P < 0.001). Individuals with IIH and DEBs were more likely to have longer periods of treatment [odds ratio: 1.07, 95% CI: 1.02-1.41), P = 0.008] and to have lost a significant amount of weight during the course of treatment [odds ratio: 9.06 (95% CI: 1.30-62.9), P = 0.026]. Depression, anxiety, and stress were more prevalent in the IIH group than in the controls (P = 0.004) and were associated with DEBs in these individuals (P = 0.01). CONCLUSIONS: There is an increased prevalence of DEBs among young individuals with IIH, which persists even after disease resolution, and is associated with higher reported rates of depression, anxiety, and stress. Medical caregivers should have heightened awareness to DEBs in individuals with IIH with the aim of early identification and intervention.
Subject(s)
Anxiety/epidemiology , Depression/epidemiology , Feeding and Eating Disorders/epidemiology , Pseudotumor Cerebri/epidemiology , Stress, Psychological/epidemiology , Adolescent , Adult , Case-Control Studies , Child , Comorbidity , Female , Humans , Male , Prevalence , Young AdultABSTRACT
OBJECTIVE: Drug-resistant epilepsy (DRE) during the first few months of life is challenging and necessitates aggressive treatment, including surgery. Because the most common causes of DRE in infancy are related to extensive developmental anomalies, surgery often entails extensive tissue resections or disconnection. The literature on "ultra-early" epilepsy surgery is sparse, with limited data concerning efficacy controlling the seizures, and safety. The current study's goal is to review the safety and efficacy of ultra-early epilepsy surgery performed before the age of 3 months. METHODS: To achieve a large sample size and external validity, a multinational, multicenter retrospective study was performed, focusing on epilepsy surgery for infants younger than 3 months of age. Collected data included epilepsy characteristics, surgical details, epilepsy outcome, and complications. RESULTS: Sixty-four patients underwent 69 surgeries before the age of 3 months. The most common pathologies were cortical dysplasia (28), hemimegalencephaly (17), and tubers (5). The most common procedures were hemispheric surgeries (48 procedures). Two cases were intentionally staged, and one was unexpectedly aborted. Nearly all patients received blood products. There were no perioperative deaths and no major unexpected permanent morbidities. Twenty-five percent of patients undergoing hemispheric surgeries developed hydrocephalus. Excellent epilepsy outcome (International League Against Epilepsy [ILAE] grade I) was achieved in 66% of cases over a median follow-up of 41 months (19-104 interquartile range [IQR]). The number of antiseizure medications was significantly reduced (median 2 drugs, 1-3 IQR, p < .0001). Outcome was not significantly associated with the type of surgery (hemispheric or more limited resections). SIGNIFICANCE: Epilepsy surgery during the first few months of life is associated with excellent seizure control, and when performed by highly experienced teams, is not associated with more permanent morbidity than surgery in older infants. Thus surgical treatment should not be postponed to treat DRE in very young infants based on their age.
Subject(s)
Drug Resistant Epilepsy , Epilepsy , Malformations of Cortical Development , Aged , Drug Resistant Epilepsy/surgery , Electroencephalography , Epilepsy/surgery , Feasibility Studies , Humans , Infant , Retrospective Studies , Treatment OutcomeABSTRACT
AIM: To evaluate the long-term effectiveness of cannabidiol (CBD)-enriched oil for the treatment of refractory epilepsy and to assess the development of tolerance to its anti-seizure effect. METHODS: A prospective study of 92 consecutive patients (age 1-37 years, mean-11.8 years) with treatment resistant epilepsy who were treated with cannabis oil extract (CBD/tetrahydrocannabinol [THC] ratio of 20:1). Mean monthly seizure frequency was reported by the patients/their parents during monthly clinic visits. Tolerance was defined as either the need to increase the dose by ≥30% due to reduced treatment efficacy or as an increase of ≥30% in mean monthly seizure frequency in patients treated for at least 3 months with no change in other anti-seizure medications. RESULTS: Mean follow-up time was 19.8 ± 12.5 months (range 3-45). Mean CBD dose was 11.3 (4-38) mg/kg/day. Twenty-nine (31%) patients discontinued treatment due to lack of effect or adverse reactions, which were reported in 51% (47/87) of the patients. Overall responder rate (>50% seizures reduction) was 54%, whereas 8 patients (9%) became seizure-free. Eighty-four patients were included in the tolerance analysis. Tolerance was observed in 21 (25%) patients after a mean duration of 7.3 ± 5.4 months of CBD-enriched oil treatment. There was a negative correlation between epilepsy duration and tolerance development (p = 0.038). CONCLUSIONS: We report for the first time the plausible appearance of tolerance to cannabidiol-enriched oil. This may limit treatment efficacy in the long-term clinical management of refractory epilepsy in both pediatric and adult population. Further studies are needed to investigate potential mechanisms.
Subject(s)
Cannabidiol/therapeutic use , Drug Resistant Epilepsy/drug therapy , Drug Tolerance/physiology , Adolescent , Adult , Anticonvulsants/therapeutic use , Cannabidiol/metabolism , Child , Child, Preschool , Drug Resistant Epilepsy/metabolism , Epilepsy/drug therapy , Epilepsy/metabolism , Female , Humans , Infant , Israel , Male , Prospective Studies , Seizures/drug therapy , Seizures/metabolism , Treatment OutcomeABSTRACT
Epilepsy is one of the most frequent CNS manifestations of tuberous sclerosis, and for most patients, it is the major debilitating factor. In up to 70% of the cases, the epilepsy is refractory and usually associated with significant behavioral as well as developmental consequences. Therefore, controlling seizures is one of the biggest medical and surgical challenges. Understanding the cellular mechanism involved in the disease empowered targeted research aimed toward early intervention in the epileptogenicity process. In this review, we present an update on the pharmacological treatments in tuberous sclerosis-related epilepsy.
Subject(s)
Epilepsy , Tuberous Sclerosis , Epilepsy/drug therapy , Epilepsy/etiology , Humans , Seizures , Tuberous Sclerosis/complicationsABSTRACT
OBJECTIVE: Resection of brain tumors may lead to new-onset seizures but may also reduce seizure rates in patients presenting with seizures. Seizures are seen at presentation in about 24% of patients with brain tumors. For lesional epilepsy in general, early resection is associated with improved seizure control. However, the literature is limited regarding the occurrence of new-onset postoperative seizures, or rates of seizure control in those presenting with seizures, following resections of extratemporal low-grade gliomas (LGGs) in children. METHODS: Data were collected retrospectively from 4 large tertiary centers for children (< 18 years of age) who underwent resection of a supratentorial extratemporal (STET) LGG. The patients were divided into 4 groups based on preoperative seizure history: no seizures, up to 2 seizures, more than 2 seizures, and uncontrolled or refractory epilepsy. The authors analyzed the postoperative occurrence of seizures and the need for antiepileptic drugs (AEDs) over time for the various subgroups. RESULTS: The study included 98 children. Thirty patients had no preoperative seizures, 18 had up to 2, 16 had more than 2, and 34 had refractory or uncontrolled epilepsy. The risk for future seizures was higher if the patient had seizures within 1 month of surgery. The risk for new-onset seizures among patients with no seizures prior to surgery was low. The rate of seizures decreased over time for children with uncontrolled or refractory seizures. The need for AEDs was higher in the more active preoperative seizure groups; however, it decreased with time. CONCLUSIONS: The resection of STET LGGs in children is associated with a low rate of postoperative new-onset epilepsy. For children with preoperative seizures, even with uncontrolled epilepsy, most have a significant improvement in the seizure activity, and many may be weaned off their AEDs.
ABSTRACT
Epilepsy is estimated to exist in approximately 40% of individuals with cerebral palsy; however, the specific features that make it drug resistant are not well defined. The main aim of this study was to determine the clinical risk factors that could predict drug-resistant epilepsy, in children with cerebral palsy. The study was performed via a retrospective chart review, analyzing clinical parameters of 118 children with cerebral palsy with either drug-resistant epilepsy or controlled epilepsy, between the years 2013 and 2018. We established a predictive model for drug-resistant epilepsy in children with cerebral palsy that is simple to apply in clinical settings and composed of the additive effect of a low Apgar score at 5 minutes, neonatal seizures, focal-onset epilepsy, and focal slowing on electroencephalogram (EEG; area under the receiver operating characteristic of 0.840). Early prediction of drug-resistant epilepsy may benefit to achieve better seizure control in children with cerebral palsy.
Subject(s)
Apgar Score , Cerebral Palsy/complications , Drug Resistant Epilepsy/complications , Drug Resistant Epilepsy/diagnosis , Electroencephalography , Adolescent , Brain/physiopathology , Child , Child, Preschool , Drug Resistant Epilepsy/physiopathology , Female , Humans , Infant , Male , Retrospective Studies , Risk FactorsABSTRACT
High-dose methotrexate is used to treat a range of adult and childhood cancers including osteosarcoma. Significant neurotoxicity is reported in 1% to 4.5% of patients treated with high-dose methotrexate and can present in a wide variety of symptoms. We present a case of a 14-year-old boy with a recent diagnosis of osteosarcoma who presented to the emergency department with status epilepticus, altered mental status, and very high fever secondary to methotrexate neurotoxicity. We review current literature and discuss some controversies related to this state. We also describe high fever as one of the possible symptoms associated with this condition and suggest using specific magnetic resonance imaging sequence to uncover abnormal findings related to this state. Since high-dose methotrexate is not a rare treatment in this era, we believe that in addition to oncologists, emergency department and intensive care providers should be aware of the potential role of methotrexate in causing significant neurotoxicity and include it in the differential diagnosis when treating a patient presenting with new neurological symptoms in the setting of recent high-dose methotrexate treatment.
Subject(s)
Antimetabolites, Antineoplastic/toxicity , Brain Diseases/chemically induced , Fever/chemically induced , Methotrexate/toxicity , Status Epilepticus/chemically induced , Adolescent , Antimetabolites, Antineoplastic/therapeutic use , Bone Neoplasms/drug therapy , Emergency Service, Hospital , Humans , Male , Methotrexate/therapeutic use , Osteosarcoma/drug therapyABSTRACT
Hemimegalencephaly is a hamartomatous malformation of one hemisphere. Functional hemispherectomy, the definitive treatment, is associated with significant morbidity and mortality in early infancy. Dysregulation of the mTOR pathway can result in malformations of cortical development, and mTOR inhibitors can effectively reduce seizures in tuberous sclerosis complex. We report a 6-day-old female with hemimegalencephaly and frequent seizures despite 9 antiseizure medications. At 3 months of age, while awaiting hemispherectomy, an mTOR inhibitor, rapamycin, was initiated by the neurologist. After 1 week of treatment, there was >50% reduction in seizures and total seizure burden, and after 2 weeks, development improved, resulting in deferral of surgery by 2.5 months with an increased body weight. Pathology demonstrated cortical dysplasia with upregulation of the mTOR pathway. Deep-sequencing of brain tissue demonstrated 16% mosaicism for a pathogenic de novo MTOR gene mutation. This case exemplifies how mTOR inhibitors could be considered for seizure reduction in patients with hemimegalencephaly while awaiting surgery.
Subject(s)
Drug Resistant Epilepsy/drug therapy , Drug Resistant Epilepsy/etiology , Hemimegalencephaly/complications , TOR Serine-Threonine Kinases/therapeutic use , Anticonvulsants/therapeutic use , Drug Resistant Epilepsy/diagnostic imaging , Drug Resistant Epilepsy/genetics , Female , Hemimegalencephaly/diagnostic imaging , Hemimegalencephaly/drug therapy , Hemimegalencephaly/genetics , Humans , Infant , Seizures/diagnostic imaging , Seizures/drug therapy , Seizures/etiology , Seizures/genetics , TOR Serine-Threonine Kinases/antagonists & inhibitors , TOR Serine-Threonine Kinases/geneticsABSTRACT
BACKGROUND: It has been suggested that sleep disordered breathing (SDB) during pregnancy may adversely influence maternal as well as fetal well being. OBJECTIVES: To examine the effect of maternal SDB on neonatal neurological examination and perinatal complications. METHODS: Pregnant women of singleton uncomplicated pregnancies were prospectively recruited from a community and hospital low risk obstetric surveillance. All participants completed a sleep questionnaire in the second trimester and underwent ambulatory sleep evaluation (WatchPAT, Itamar Medical, Caesarea, Israel). They were categorized as SDB (apnea hypopnea index > 5) and non-SDB. Maternal and newborn records were reviewed and a neonatal neurologic examination was conducted during the first 48 hours. RESULTS: The study group included 44 women and full-term infants; 11 of the women (25%) had SDB. Mean maternal age of the SDB and non-SDB groups was 32.3 ± 2.8 and 32.5 ± 4.7 years, respectively (P = 0.86). Mean body mass index before the pregnancy in the SDB and non-SDB groups was 25.8 ± 4.7 and 22.0 ± 2.5 kg/m2, respectively (P = 0.028). No differences were found between infants born to mothers with SDB and non-SDB in birth weight (3353.8 ± 284.8 vs. 3379.1 ± 492.4 g), gestational age (39.5 ± 0.9 vs. 39.2 ± 1.5 weeks), 5 minute Apgar scores (9.8 ± 0.6 vs. 9.9 ± 0.3), and neurologic examination scores (95.2 ± 3.9 vs. 94.6 ± 4.1). P value for all was not significant. CONCLUSIONS: Our preliminary results suggest that maternal mild SDB during pregnancy has no adverse effect on neonatal neurologic examination or on perinatal complications.
Subject(s)
Apgar Score , Pregnancy Complications/epidemiology , Pregnancy Outcome , Sleep Apnea Syndromes/complications , Adult , Birth Weight , Body Mass Index , Female , Humans , Infant, Newborn , Israel , Male , Pregnancy , Sleep/physiology , Sleep Apnea Syndromes/epidemiology , Surveys and QuestionnairesABSTRACT
Since many of the children with BCECTSs display electrical status epilepticus during sleep and many present with different comorbidities, mainly ADHD and behavioral disturbances, clinicians are often confronted with the dilemma of how aggressive they should be with their efforts of normalizing the EEG. We conducted a retrospective study by screening medical records of all consecutive patients with BCECTSs, spike-wave index (SWI) >30%, and ADHD/ADD that were evaluated in our pediatric epilepsy service and were followed up for at least two years. Patients with neurocognitive deterioration detected by formal testing were excluded. A total of 17 patients with mean age of 6.9years at BCECTS diagnosis were identified. The patients' mean SWI was 60% and that dense electrical activity lasted 1.5years on average (range: 1-4.5years). Six children were formally diagnosed with learning disabilities in addition to ADD/ADHD. All of them were treated with an average of three antiepileptic medications, mainly for the purpose of normalizing the EEG, but none of them was treated with steroids or high-dose diazepam. The mean duration of follow-up was 5.5years. A cognitive or behavioral deterioration was not detected in any of them. Our data suggest that when treating a child with BCECTSs, high SWI, and school difficulties, the most critical parameter that determines the necessity of using second-line antiepileptic agents such as steroids or high-dose diazepam is a formal psychological evaluation that proves cognitive (I.Q.) decline. Otherwise, these agents may be avoided.
Subject(s)
Epilepsy, Rolandic/drug therapy , Sleep Wake Disorders/etiology , Status Epilepticus/drug therapy , Achievement , Adolescent , Aggression , Anticonvulsants/therapeutic use , Attention Deficit Disorder with Hyperactivity/epidemiology , Attention Deficit Disorder with Hyperactivity/psychology , Child , Comorbidity , Electroencephalography , Epilepsy, Rolandic/epidemiology , Female , Humans , Learning Disabilities/epidemiology , Learning Disabilities/psychology , Male , Retrospective Studies , Status Epilepticus/epidemiologyABSTRACT
OBJECTIVE: We sought to examine the effect of maternal sleep-disordered breathing (SDB) on infant general movements (GMs) and neurodevelopment. STUDY DESIGN: Pregnant women with uncomplicated full-term pregnancies and their offspring were prospectively recruited from a community and hospital low-risk obstetric surveillance. All participants completed a sleep questionnaire on second trimester and underwent ambulatory sleep evaluation (WatchPAT; Itamar Medical, Caesarea, Israel). They were categorized as SDB (apnea hypopnea index>5) and controls. Infant GMs were assessed in the first 48 hours and at 8-11 and 14-16 weeks of age. At 12 months of age the Infant Developmental Inventory and the Brief Infant Sleep Questionnaire were administered. RESULTS: In all, 74 women and their full-term infants were studied. Eighteen (24%) women had SDB. Mean birthweight was 3347.1±423.9 g. Median Apgar score at 5 minutes was 10 (range, 8-10). In adjusted comparisons, no differences were found between infants born to mothers with SDB and controls in GM scores in all 3 evaluations. Low social developmental score was detected at 12 months in 64% of infants born to SDB mothers compared to 25% of infants born to controls (adjusted P=.036; odds ratio, 16.7). Infant snoring was reported by 41.7% of mothers with SDB compared to 7.5% of controls (P=.004). CONCLUSION: Our preliminary results suggest that maternal SDB during pregnancy has no adverse effect on neonatal and infant neuromotor development but may affect social development at 1 year.
Subject(s)
Child Development , Developmental Disabilities/epidemiology , Language Development , Motor Skills , Pregnancy Complications/epidemiology , Prenatal Exposure Delayed Effects/epidemiology , Sleep Apnea Syndromes/epidemiology , Social Behavior , Adult , Apgar Score , Female , Humans , Infant , Infant, Newborn , Male , Polysomnography , Pregnancy , Prospective Studies , Risk Factors , Snoring/epidemiology , Surveys and QuestionnairesABSTRACT
PURPOSE: This paper summarizes our experience with surgical treatment of pediatric low-grade glial temporal lobe tumors focusing on the long-term outcome of seizures and identifying factors associated with seizure control and failure. METHODS: We reviewed all medical records of pediatric patients that underwent temporal lobe surgery due to seizures at our institution between 1997 and 2009. Only patients with temporal lobe tumors were included in this series. The files were retrospectively reviewed for seizure history. All children had undergone pre- and postoperative evaluation, neurological examination, EEG, and MRI. RESULTS: The cohort includes 48 children with mean follow-up time of 5.15 years (1-12 years). The mean age at surgery was 8.2 years (1-18.1) and the mean seizure duration until surgery was 2.6 years. All lesions in the cohort were low-grade tumors; pilocytic astrocytoma was the most common (41%). Eighty-three percent of the patients were classified as Engel class I following surgery. There was no correlation between Engel score and the preoperative epilepsy duration, age of seizure onset or type of seizures, and pathology. The surgical complication rate was 4.1% (2/48). CONCLUSIONS: Surgical treatment for seizure control in children and adolescents with low-grade temporal tumors provides excellent long-term results.