Effects of sulthiame on seizure frequency and EEG in children with electrical status epilepticus during slow sleep.

OBJECTIVE: It is argued that early and adequate treatment of electrical status epilepticus in sleep (ESES) is essential to preserve cognitive functions and possibly recovering lost skills. Although antiepileptic drugs (AEDs) are effective in ESES, there is not much experience in the use of sulthiame. In this study, we aimed to examine the efficiency and tolerability of sulthiame in ESES. METHODS: The data of 39 patients diagnosed as ESES and who received sulthiame as an additional treatment between 2016 and 2020 were reviewed retrospectively. Electroencephalographic (EEG) findings and seizure rates were compared before and after the sulthiame treatment. RESULTS: The mean age was 8.5 ±â€¯4.1 (1.5-16 years). Nine out of 39 patients had benign childhood focal epilepsies. Structural causes were identified in 13 patients. The mean duration of sulthiame use was 32.5 ±â€¯13.7 months. After sulthiame treatment, 25 patients (64.1%) were seizure free, and 8 (20.5%) had more than a 50% decrease in seizure frequency. The mean seizure-free time after the sulthiame treatment was 27.8 ±â€¯17.9 months. Nineteen patients (48.7%) had complete, and nine patients (23.1%) had partial EEG improvement. Complete seizure control was significantly higher in benign focal epilepsy of childhood (p = 0.01). Significant neurocognitive and behavioral recovery, improvement in school performance was observed following sulthiame treatment (p < 0.001). CONCLUSION: Sulthiame was found to be effective in seizure control and EEG improvement in ESES. We think that the use of sulthiame alone can be a good choice with high efficacy and tolerability in ESES.

Long-term effects of vagus nerve stimulation in refractory pediatric epilepsy: A single-center experience.

INTRODUCTION: Vagus nerve stimulation (VNS) has been used as an adjunctive therapy for both children and adults with refractory epilepsy, over the last two decades. In this study, we aimed to evaluate the long-term effects and tolerability of VNS in the pediatric drug-resistant epilepsy (DRE) and to identify the predictive factors for responsiveness to VNS. METHODS: We retrospectively reviewed the medical records of pediatric patients who underwent VNS implantation between 1997 and 2018. Patients with ≥50% reduction of seizure frequency compared with the baseline were defined as "responders". The clinical characteristics of responders and nonresponders were compared. RESULTS: A total of 58 children (male/female: 40/18) with a mean follow-up duration of 5.7 years (3 months to 20 years) were included. The mean age at implantation was 12.4 years (4.5 to 18.5 years). Approximately half (45%) of our patients were responders, including 3 patients (5.8%) who achieved seizure freedom during follow-up. The age of seizure-onset, duration of epilepsy, age at implantation, and etiologies of epilepsy showed no significant difference between responders and nonresponders. Responders were more likely to have focal or multifocal epileptiform discharges (63%) on interictal electroencephalogram (EEG), when compared to nonresponders (36%) (p = .07). Vocal disturbances and paresthesias were the most common side effects, and in two patients, VNS was removed because of local reaction. CONCLUSION: Our series had a diverse etiological profile and patients with transition to adult care. Long-term follow-up showed that VNS is an effective and well-tolerated treatment modality for refractory childhood onset epilepsy. Age at implantation, duration of epilepsy and underlying etiology are not found to be predictors of responsiveness to VNS. Higher response rates were observed for a subset of patients with focal epileptiform discharges.

International consensus classification of hippocampal sclerosis and etiologic diversity in children with temporal lobectomy.

INTRODUCTION: The distribution of hippocampal sclerosis (HS) subtypes, according to the classification of the International League Against Epilepsy (ILAE), has been reported mainly in adult patients. We aimed to review the pathological findings in children who had anterior temporal lobectomy accompanied with amygdalohippocampectomy, in view of the current classification, and evaluate postsurgical outcome with respect to HS subtypes in childhood. METHODS: Seventy children who underwent temporal resections for treatment of medically refractory epilepsy, with a minimum follow-up of 2 years, were included; the surgical hippocampus specimens were re-evaluated under the HS ILAE classification. RESULTS: Neuropathological evaluations revealed HS type 1 in 38 patients (54.3%), HS type 2 in 2 (2.8%), HS type 3 in 21 patients (30%), and no HS in 9 patients (12.9%). Of 70 patients, 23 (32.9%) had dual pathology, and the most common pattern was HS type 3 with low-grade epilepsy-associated brain tumors (LEAT). The distribution of HS types with respect to age revealed that HS type 3 and no HS subgroups had significantly more patients younger than 12 years, compared with those of HS type 1 (90.5%, 77.8% vs 47.4%, respectively). History of febrile seizures was higher in HS type 1. Prolonged/recurrent febrile seizures were most common in patients 12 years and older, whereas LEAT was the most common etiology in patients under 12 years of age (p < 0.001). Patients with HS type 1 had longer duration of epilepsy and an older age at the time of surgery compared with patients with HS type 3 and no HS (p: 0.031, p: 0.007). At final visit, 74.3% of the patients were seizure-free. Seizure outcome showed no significant difference between pathological subtypes. CONCLUSIONS: Our study presents the distribution of HS ILAE subtypes in an exclusively pediatric series along with long-term seizure outcome. The study reveals that the leading pathological HS subgroup in children is HS type 1, similar with adult series. Hippocampal sclerosis type 2 is significantly less in children compared with adults; however, HS type 3 emerges as the second most predominant group because of dual pathology, particularly LEAT. Further studies are required regarding clinicopathological features of isolated HS in pediatric cohort. Seizure-free outcome was favorable and similar in all HS types in children. The proportion of HS types may be better defined in pediatric patients with temporal resections, as the current HS ILAE classification becomes more widely used, and may help reveal the surgical and cognitive outcome with respect to HS types.

Intelligence quotient improves after antiepileptic drug withdrawal following pediatric epilepsy surgery.

OBJECTIVE: Antiepileptic drugs (AEDs) have cognitive side effects that, particularly in children, may affect intellectual functioning. With the TimeToStop (TTS) study, we showed that timing of AED withdrawal does not majorly influence long-term seizure outcomes. We now aimed to evaluate the effect of AED withdrawal on postoperative intelligence quotient (IQ), and change in IQ (delta IQ) following pediatric epilepsy surgery. METHODS: We collected IQ scores of children from the TTS cohort with both pre- and postoperative neuropsychological assessments (NPAs; n = 301) and analyzed whether reduction of AEDs prior to the latest NPA was related to postoperative IQ and delta IQ, using linear regression analyses. Factors previously identified as independently relating to (delta) IQ, and currently identified predictors of (delta) IQ, were considered possible confounders and used for adjustment. Additionally, we adjusted for a compound propensity score that contained previously identified determinants of timing of AED withdrawal. RESULTS: Mean interval to the latest NPA was 19.8 ± 18.9 months. Reduction of AEDs at the latest NPA significantly improved postoperative IQ and delta IQ (adjusted regression coefficient [RC] = 3.4, 95% confidence interval [CI] = 0.6-6.2, p = 0.018 and RC = 4.5, 95% CI = 1.7-7.4, p = 0.002), as did complete withdrawal (RC = 4.8, 95% CI = 1.4-8.3, p = 0.006 and RC = 5.1, 95% CI = 1.5-8.7, p = 0.006). AED reduction also predicted ≥ 10-point IQ increase (p = 0.019). The higher the number of AEDs reduced, the higher was the IQ (gain) after surgery (RC = 2.2, 95% CI = 0.6-3.7, p = 0.007 and RC = 2.6, 95% CI = 1.0-4.2, p = 0.001, IQ points per AED reduced). INTERPRETATION: Start of AED withdrawal, number of AEDs reduced, and complete AED withdrawal were associated with improved postoperative IQ scores and gain in IQ, independent of other determinants of cognitive outcome.

Non-epileptic paroxysmal events at pediatric video-electroencephalography monitoring unit over a 15-year period.

OBJECTIVE: Non-epileptic paroxysmal events (NEPEs) are common in pediatric patients and may be misdiagnosed as epileptic seizures. We aimed to study the distribution of NEPEs across age groups and with different comorbidities, and to correlate the patients' presenting symptoms with their final diagnosis after video-EEG. METHODS: We retrospectively analyzed video-EEG recordings of children aged one month to 18 years who were admitted between March 2005 and March 2020. Patients who experienced any NEPE while under video-EEG monitorization were evaluated in this study. Subjects with concomitant epilepsy were also included. The patients were first divided into 14 groups according to the basic characteristics of symptoms they reported at admission. The events captured on video-EEG were then classified into six NEPE categories based on the nature of the events. These groups were compared according to video-EEG results. RESULTS: We retrospectively evaluated 1338 records of 1173 patients. The final diagnosis was non-epileptic paroxysmal event in 226 (19.3%) of 1173 patients. The mean age of the patients was 105.4 ± 64.4 months at the time of the monitoring. The presenting symptoms were motor in 149/226 (65.9%) patients, with jerking being the most common (n = 40, 17.7%). Based on video-EEG, the most common NEPE was psychogenic non-epileptic seizures (PNES) (n = 66, 29.2%), and the most common PNES subtype was major motor movements (n = 19/66, 28.8%). Movement disorders (n = 46, 20.4%) were the second most common NEPE and the most common NEPE (n = 21/60, 35%) in children with developmental delay (n = 60). Other common NEPEs were physiological motor movements during sleep (n = 33, 14.6%), normal behavioral events (n = 31, 13.7%), and sleep disorders (n = 15, 6.6%). Almost half of the patients had a prior diagnosis of epilepsy (n = 105, 46.5%). Following the diagnosis of NEPE, antiseizure medication (ASM) was discontinued in 56 (24.8%) patients. CONCLUSION: Non-epileptiform paroxysmal events can be difficult to distinguish from epileptic seizures in children, especially in patients with developmental delay, epilepsy, abnormal interictal EEG, or abnormal MRI findings. Correct diagnosis of NEPEs by video-EEG prevents unnecessary ASM exposure in children and guides appropriate management of NEPEs.

Clinically important intracranial abnormalities in children presenting with first focal seizure.

BACKGROUND: Management of pediatric patients presenting with first seizure is challenging, especially with regards to emergent neuroimaging. The rate of abnormal neuroimaging findings is known to be higher in focal seizures than in generalized seizures, but those intracranial abnormalities are not always clinically emergent. In this study, we aimed to determine the rate and indicators for clinically important intracranial abnormalities that change acute management in children presenting with a first focal seizure to the pediatric emergency department (PED). METHODS: This study was conducted retrospectively in the PED at a University Children`s Hospital setting. The study population consisted of patients aged between 30 days and 18 years with first focal seizure and who had emergent neuroimaging at the PED between the years 2001 and 2012. RESULTS: There were 65 eligible patients meeting the study criteria. Clinically important intracranial abnormalities requiring emergent neurosurgical or medical intervention were detected in 18 patients (27.7%) at the PED. Four patients (6.1%) underwent emergent surgical procedures. Seizure recurrence and the need for acute seizure treatment in the PED were significantly associated with clinically important intracranial abnormalities. CONCLUSIONS: Neuroimaging study yielding of 27.7% shows that first focal seizure must be evaluated meticulously. From the emergency department`s point of view; we suggest that first focal seizures in children should be evaluated with emergent neuroimaging, if possible with magnetic resonance imaging. Especially patients with recurrent seizures at presentation requires more careful evaluation.

The classification and differential diagnosis of absence seizures with short-term video-EEG monitoring during childhood.

Absence seizures are idiopathic epilepsies characterized by impairment of consciousness and generalized 2.5-4 Hz spike and slow wave discharges. This prospective study was performed to classify and define properties of subgroups of absence epilepsies. We included 31 patients, of whom seven were in the differential diagnosis group. On admission, absence epilepsy provisional diagnosis was considered in 16 patients clinically and in the other 15 patients based on routine EEG findings. Ictal EEGs were recorded by video-EEG monitoring in 23 of the patients (totally 202 ictal recordings). Patients were diagnosed as childhood absence epilepsy (n=8), juvenile absence epilepsy (n=10), juvenile myoclonic epilepsy (n=3), eyelid myoclonia with absences (n=2), and perioral myoclonia with absences (n=1). Neuroimaging, video-EEG monitoring and especially ictal recordings are important for classification of epilepsies in addition to history, physical examination and routine EEG findings. Video-EEG monitoring is required to classify, to make differential diagnosis and to determine the treatment plan and prognosis.

Epileptic encephalopathy with electrical status epilepticus during slow sleep: evaluation of treatment response from a tertiary center.

BACKGROUND: This study aimed to evaluate the clinical, electrophysiological, etiological features, and treatment response in children with epileptic encephalopathy with electrical status epilepticus during slow sleep (ESES). METHODS: Clinical data, records of electroencephalograms (EEG), and brain magnetic resonance imaging (MRI) findings of 33 patients with ESES who were treated, and followed up for at least one year were retrospectively analyzed. RESULTS: Of all patients, 57.6% were male, and 42.4% were female. The mean age was 10.45 ± 2.88 years. At first admission, 90% of patients had seizures, and 10% had only school failure. Twelve patients had childhood focal epileptic syndrome. In etiology, asphyxia (n=6), hydrocephalus (n=2), polymicrogyria (n=1), and mesial temporal sclerosis (n=1) were determined. Neurological examination was abnormal in 27.2%, and brain MRI findings were pathological in 36.3% of the patients. During the ESES phase, the spike-wave index (SWI) on the non-rapid eye movement (NREM) sleep EEG was > 85% in 16 patients and 50-85% in 17 patients. Only one patient received one, and the others had at least two antiseizure medications. Benzodiazepines were found to be the most effective treatment. In the two-year follow-up, 24 patients (72.7%) were seizure-free, and nineteen patients (57.5%) had complete recovery of SWI on their NREM sleep EEG. There was a significant correlation with reduction of the SWI on the EEG and seizure control (p < 0.001). In addition, a significant correlation was found between neurocognitive and behavioral scores scored before and after treatment, seizure control, and EEG recovery. CONCLUSIONS: ESES is an epileptic encephalopathy that can be treated safely with antiseizure medications. Neurocognitive examinations and follow-up of EEG findings are valuable in terms of the treatment response. Benzodiazepines were found to be very effective in additional treatment.

Lesional resective epilepsy surgery in childhood: Comparison of two decades and long-term seizure outcome from a single center.

OBJECTIVE: Epilepsy surgery has shown efficacy in children. We aimed to assess long-term seizure outcome in children who underwent epilepsy surgery and determine predictive factors for seizure freedom. METHODS: This is a retrospective study of 196 children who underwent epilepsy surgery between 1994 and 2015 and had a minimum postoperative follow-up of 5 years. RESULTS: The median age at the time of surgery was 9.5 (0.08-19.8) years; 110 (56.1%) had temporal, 62 (31.6%) had extratemporal resections, and 24 (12.2%) had hemispheric surgery. The duration of postsurgical follow-up was between 5 and 20 years (mean±SD: 7 ± 3.2). Overall, 129 of 196 (65.8%) patients had Engel class I outcome at final visit. Among patients who underwent temporal, extratemporal and hemispheric surgery; 84 of 110 (76.4%), 34 of 62 (54.8%), and 11 of 24 (45.8%) patients had complete seizure freedom, respectively (p: 0.016). Patients with tumors had the best outcome, with 83.1% seizure freedom. The number of preoperative antiseizure medications (OR 3.19, 95% CI 1.07-9.48), the absence of postoperative focal epileptiform discharges (OR 8.98, 95% CI 4.07-19.79) were independent predictors of seizure freedom. Across two decades, the age at surgery was decreased (p: 0.003), overall seizure freedom (61.8% vs 68%) did not differ. In the past decade, a higher proportion of malformations of cortical development was operated (14.7% vs 35.9%, p: 0.007). SIGNIFICANCE: Our findings showed favorable long-term seizure outcome in children who underwent epilepsy surgery. The results are encouraging for developing centers with limited resources to establish pediatric epilepsy programs.

Risk of recurrence after drug withdrawal in childhood epilepsy.

OBJECTIVES: After a reasonable seizure-free period, discontinuation of antiepileptic drugs (AED) is usually decided in epileptic patients despite the risk of seizure recurrence. In children, risk of recurrence after discontinuation of AED is generally 20-40%; however, there is still no general agreement on the criteria to predict safe discontinuation. This study was designed to determine the risk of recurrence and related risk factors after drug withdrawal in epileptic children. METHODS: 200 epileptic patients between 1 month and 15 years of age who were followed at least 1 year after drug withdrawal at a child neurology center between January 1993 and December 2005 formed the study population of this retrospective study. Patients were classified into groups according to defined risk factors for recurrence. RESULTS: Of 200 patients (118 boys, 82 girls), overall recurrence rate was 27%. Girls were more likely to have a seizure recurrence than boys, with the difference approaching statistical significance (p=0.058). EEG recordings after withdrawal (post-withdrawal EEG) in the follow-up were significantly different in the patients with recurrence with respect to presence of an abnormality (p=0.05). In the multivariate Cox regression analysis, female gender and abnormal post-withdrawal EEG were the risk factors influencing seizure recurrence, with female gender identified as the main risk factor. CONCLUSIONS: Although the decision to discontinue AED treatment necessitates evaluation of each patient individually, our study suggests that female patients and those with abnormal EEG after withdrawal require more cautious follow-up because of the high risk of recurrence.

Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum, seizure outcome, neuroradiology, and pathology.

INTRODUCTION: Dysembryoplastic neuroepithelial tumors (DNTs) were first described by Daumas-Duport et al. in 1988 as a typically cortical tumor affecting young patients with long-standing, drug-resistant epilepsy. METHODS: We reviewed the medical records of 29 patients with DNT between 1994 and 2007 at Hacettepe University Children's Hospital retrospectively; age at the time of surgery, age at seizure onset, electroencephalography (EEG), MRI, medical treatment, surgical procedure, seizure outcome, and pathological findings were documented. RESULTS: Male to female ratio was 15/14. Age at the time of evaluation ranged 4-24 years. Twenty-seven patients (93.1%) had complex partial seizures, one (3.44%) had simple partial seizures, and one patient had generalized seizures. Preop interictal EEG showed epileptiform discharges in 24 patients, while in five patients interictal EEG before surgery showed no epileptiform discharges. Pathologically, 24 of our patients were classified as complex type and five as simple type. MRI showed temporal lesion in 20 (68.9%) patients and nine patients had extratemporal DNT. We choose the type of surgery according to lesion and the epileptojenic zone. Finally, 27 patients had Engel Class IA and two patients had Engel Class IB outcome. CONCLUSION: Complete resection of the lesion with epileptojenic zone is important for seizure-free outcome. Timing of surgery, extent of surgery, and stopping antiepileptic drugs are still important factors.

Lamotrigine in children with refractory epilepsy.

We studied the efficacy and side effect profile oflamotrigine (LTG) in children with intractable epilepsy. We reviewed the database of our prolonged video-EEG laboratory to screen patients with intractable epilepsy who were on LTG either alone or in combination for three months or more. Of 75 patients, 28 patients (37%) had primary generalized seizures, 42 patients (56%) had partial onset seizures with or without secondary generalization, and 5 patients (7%) had two or more types of seizures. LTG reduced seizure frequency by at least 50% in 57.1% of patients with partial seizures and in 53.6% of patients with primary generalized seizures. The incidence of adverse events was relatively low (15%); the most common was skin rash. LTG should be considered in the treatment of pediatric patients with both partial onset and primary generalized seizures refractory to the major older antiepileptic drugs. LTG has a favorable side effect profile.

Neurologic outcome in patients with MRI pattern of damage typical for neonatal hypoglycemia.

Imaging features of infants who suffered from brain damage as a result of neonatal hypoglycemia were shown to have similar patterns, and to affect the parietal and occipital lobes most severely. Long term follow up regarding clinical outcome of patients with neonatal hypoglycemia and this pattern of damage are limited. We reviewed the medical records of 24 patients with typical neuroimaging features seen following neonatal hypoglycemia; we report neurological outcome in 13 of them who have documented hypoglycemia. Except for one patient, all patients had prenatal and perinatal problems including prematurity, perinatal hypoxia, intrauterine growth retardation, sepsis, indirect hyperbilirubinemia. All but one patient had symptomatic partial epilepsy, five of them were medically intractable. Other neurologic problems included developmental delays, learning and behavior problems, hyperactivity and attention difficulties, autistic features, microcephaly and cortical blindness. We conclude that early diagnosis and treatment of neonatal hypoglycemia is crucial to prevent future neurological sequelae, especially in patients with additional perinatal risk factors.

Psychiatric evaluation of children with CSWS (continuous spikes and waves during slow sleep) and BRE (benign childhood epilepsy with centrotemporal spikes/rolandic epilepsy) compared to children with absence epilepsy and healthy controls.

This investigation examined psychopathology and IQ levels in 30 children with CSWS and 42 children with BRE and compared them with 40 healthy controls and 23 children with absence epilepsy by using the Schedule for Affective Disorders and Schizophrenia for School-Age Children (K-SADS) and Wechsler Intelligence Test for Children (WISC-R). The CSWS and BRE groups had the highest rate of psychiatric diagnoses and lowest IQ scores. The BRE group had higher rates of psychopathology and lower IQ scores compared to the healthy controls. While BRE and absence groups did not differ with regard to presence of psychopathology, IQ levels and types of psychopathologies, children with CSWS had more psychiatric disorders and lower IQ scores compared to the patient group with absence epilepsy. These findings suggest that CSWS and BRE are two epileptic syndromes that lead to psychiatric disorders and lower IQ scores. Hence, psychiatric consultation should be a part of the treatment while managing these children.

Malformations of cortical development and epilepsy: evaluation of 101 cases (part II).

Malformations of cortical development (MCD) form a spectrum of lesions produced by insult to the developing neocortex. Clinical presentation and electrophysiologic findings of MCD are variable and depend on the affected cortical area. We evaluated epilepsy, EEG, and response to antiepileptic treatment in patients with MCD with respect to the neuroimaging findings. We studied 101 patients, ranging between 1 month and 19 years of age. Fifty-four patients were diagnosed with polymicrogyria (PMG), 23 patients with lissencephaly, 12 patients with schizencephaly, and 12 patients with heterotopia. With regards to epilepsy and seizure type, 72/101 (71.3%) patients had epilepsy, and 62/101 (61.4%) patients presented with seizures. Overall, 32.7% of patients had generalized seizures, and 25.7% had complex partial seizures. Mean age at the onset of seizures was 2.7 +/- 3.4 years. The onset of epilepsy tended to be younger in patients with lissencephaly and older in patients with heterotopias. Of the cases, 79.2% had abnormal EEG (56.3% with epileptiform abnormality, 22.9% with non-epileptiform abnormality). EEG was abnormal in 44.9% (13/29) of the cases without epilepsy. EEG showed bilateral synchronous and diffuse epileptiform discharges in 90% of patients with lissencephaly. Patients with schizencephaly had mostly focal epileptiform discharges. Heterotopia cases had a high rate of EEG abnormalities (72.7%). Patients with PMG had epileptiform abnormality in 59.5% of the cases. Patients with heterotopias and PMG achieved better seizure control in comparison with the other groups. In conclusion, epilepsy is the most common problem in MCD. Epilepsy and EEG findings of patients with MCD are variable and seem to be correlated with the extent of cortical involvement.

Malformations of cortical development: clinical spectrum in a series of 101 patients and review of the literature (Part I).

Patients with malformations of cortical development (MCD) present with a wide spectrum of clinical manifestations ranging from asymptomatic cases to those with epilepsy and neurodevelopmental problems. Thorough clinical delineation of patients with MCD may provide clues for future phenotype-genotype correlation studies. We studied clinical features of patients with MCD, including developmental risk factors and family history. We evaluated 10 patients with MCD at Hacettepe University Children's Hospital, Department of Pediatric Neurology. All patients underwent neurological evaluation with detailed medical and family history, and neuropsychological evaluation. Routine EEG and MRI were obtained. The patients were between 1 month and 19 years of age (mean: 6.1 +/- 4.4 years). Fifty-four patients were diagnosed with polymicrogyria (PMG), 23 patients with lissencephaly, 12 patients with schizencephaly, and 12 patients with heterotopia. Parents were relatives in 31.7% of the cases; consanguinity was most common in patients with lissencephaly and other MCDs with diffuse/bilateral involvement. Initial clinical presentation was seizures in 61.4% of the cases, developmental delays in 12.9%, and microcephaly in 9.9%. Neurological evaluation revealed most severe abnormalities in patients with lissencephaly, and relatively better outcome in patients with heterotopias. Cognitive functions were better in patients with heterotopias compared to other groups. Overall, 71.3% of patients ha epilepsy. In conclusion, initial presentation and clinical course of patients with MCD are variable and seem to be correlated with the extent of cortical involvement. Epilepsy and mental retardation are the most common problems. The most severe clinical outcome was seen in patients with lissencephaly.

Cyclic vomiting with generalized epileptiform discharges responsive to topiramate therapy.

Cyclic vomiting syndrome is a disorder characterized by recurrent attacks of vomiting and intervals of normal health between vomiting episodes averaging 2-4 weeks. It has been described by a variety of names such as abdominal migraine, abdominal epilepsy, and periodic syndrome but now has been classified in the subgroup of childhood periodic syndromes that are commonly precursors of migraine. Topiramate is an antiepileptic drug used both in the treatment of epilepsy and in migraine prophylaxis. This report presents a child with cyclic vomiting syndrome with generalized epileptiform discharges who responded to topiramate therapy. The common features of epilepsy, migraine, and cyclic vomiting syndrome are discussed.

Vigabatrin in pediatric patients with refractory epilepsy.

New generation antiepileptic medications have improved seizure outcome in patients with intractable epilepsy. We studied the efficacy and side effect profile of vigabatrin (VGB) in pediatric patients with intractable seizure disorder. We reviewed the database of our short-term video-EEG monitoring laboratory to screen patients with intractable epilepsy who were on VGB either alone or in combination for three months or more. We subsequently reviewed the medical records of these patients to abstract clinical information regarding age, sex, seizure type, epilepsy syndrome, efficacy and side effects of VGB. Of 111 patients, 75 (68%) were male and 36 (32%) female. Seizure onset was during the newborn period in 12 patients (11%), during the first year of life beyond the newborn period in 47 patients (42%), between 1-5 years in 23 patients (21%), and above five years in the remaining 29 patients (26%). Fifty-four patients (48.6%) had partial onset seizures with or without secondary generalization; 49 patients (44.1%) had primary generalized seizures; 8 patients (7.2%) had two or more types of seizure. Fifty-three percent of patients had mental retardation, and 35% had abnormal findings on physical/ neurological examination. Of 98 patients, 70 (71.4%) had abnormal magnetic resonance imaging (MRI) findings. Ninety-seven percent of patients had been on polytherapy before VGB was added to treatment. VGB reduced seizure frequency by at least 50% in 33.3% of patients with partial seizures, and in 30.6% of patients with primary generalized seizures. Six of the responders with partial seizures had complete resolution of their seizures. Most common side effects included visual field defects, increased appetite and obesity. Vigabatrin seems to be more effective in partial seizures in childhood intractable epilepsy. Patients should be closely monitored regarding side effects of VGB.

The seizure semiology consistent with frontal lobe symptomatogenic zone in children.

The aim of this study is to analyze the seizure semiology consistent with frontal lobe symptomatogenic zone in childhood. We analyzed 549 videotaped seizures from 79 patients (mean age 9.9 ± 3.8 years). Magnetic resonance imaging was normal in 30 patients. The seizures in the time interval of 10 p.m. to 6 a.m. were considered as nocturnal. The mean number of seizures per patient was 6.8 ± 7.3. The mean seizure duration was 25.7 ± 26.9 sec; postictal confusion was 27 ± 16.1 sec (7-92 seconds). The seizures were observed in sleep with a rate of 56.8%; 43.1% of them were during wakefulness. Overall 50.4% of the seizures occured during night-time sleep. Tonic seizure (77.2%) was the most frequent simple motor seizure. Versive seizures were the second most frequent type of simple motor seizure (26.7%). Clonic seizures were 17.7%, complex motor seizures were 20.5%, and dialeptic seizures were 3% of all the seizures. Epileptic spasm, myoclonic seizures, aphasia, and akinetic semiologies were not observed. Vocalization was observed in 16% of the seizures. Frontal lobe seizures in childhood have a short duration, occur frequently, especially during night time sleep, and have a brief postictal period. Tonic semiology, versive semiology are the most frequent seizure semiologies; hypermotor and secondary generalized tonic clonic seizures and vocalizations are observed less in children compared to adults.

Surgical management of temporal lobe tumor-related epilepsy in children.

OBJECT: Slow-growing, low-grade temporal lobe tumors are one of the most common causes of epilepsy in children. Although there are numerous consistent features in this patient group, consensus about the management and surgical approach is lacking. In this study the authors review the clinical, pathological, and radiological features as well as outcome data obtained in 29 pediatric patients with temporal lobe tumor-related epilepsy and discuss the surgical treatment strategies. METHODS: In patients who presented with intractable seizures secondary to mass lesions and underwent comprehensive epilepsy workup, the tumor was resected and the diagnosis confirmed by pathological examination. A minimum follow-up period of 16 months was required. Medical records were reviewed for details of seizure type and duration, electrophysiological data, imaging studies, operative notes, pathological examination reports, and follow-up data. The surgical approach was as follows. The lesionectomy with/without cortical resection was performed in all cases of lateral temporal tumors. Lesionectomy was performed with/without cortical resection in cases of basal temporal tumors if the mesial structures were radiologically normal. Mesial temporal tumors were excised, as were the remaining mesial temporal structures in the nondominant hemisphere; however, if the tumor was in the dominant hemisphere, lesionectomy was performed only if the remaining mesial structures were radiologically normal. Twenty-nine patients between 2 and 18 years of age were identified. Most tumors were located in the mesial temporal lobe. All patients underwent resection of the tumor with or without mesial and cortical structures. The most common pathological entity was dysembryoplastic neuroepithelial tumor. Sixty-nine percent of the patients remained seizure free (Engel Class I) and 14% experienced significant improvement (Engel Class II) after surgery. Outcome was better in the patients who underwent gross-total tumor resection. CONCLUSIONS: Mesially located low-grade neoplasms were the most frequently observed mass lesions in children with temporal tumor-related epilepsy in this series. Resection of the tumor with or without amygdalohippocampectomy provides a high rate of seizure-free outcome. It is the author's opinion that temporal lobe tumors should be managed based on the subgroups defined by their anatomical locations. If the tumor is located in or in proximity to eloquent cortex, we recommend functional magnetic resonance imaging and invasive monitoring techniques to map the eloquent cortex and epileptogenic zone, thereby tailoring the resection.