Clinical significance of ictal magnetoencephalography in patients undergoing epilepsy surgery.

OBJECTIVE: The significance of ictal magnetoencephalography (MEG) is not well appreciated. We evaluated the relationships between ictal MEG, MRI, intracranial electroencephalography (ICEEG), surgery and postoperative seizure outcome. METHODS: A total of 45 patients (46 cases) with ictal MEG who underwent epilepsy surgery was included. We examined the localization of each modality, surgical resection area and seizure freedom after surgery. RESULTS: Twenty-one (45.7%) out of 46 cases were seizure-free at more than 6 months follow-up. Median duration of postoperative follow-up was 16.5 months. The patients in whom ictal, interictal single equivalent current dipole (SECD) and MRI lesion localization were completely included in the resection had a higher chance of being seizure-free significantly (p < 0.05). Concordance between ictal and interictal SECD localizations was significantly associated with seizure-freedom. Concordance between MRI lesion and ictal SECD, concordance between ictal ICEEG and ictal and interictal SECD, as well as concordance between ictal ICEEG and MRI lesion were significantly associated with seizure freedom. CONCLUSIONS: Ictal MEG can contribute useful information for delineating the resection area in epilepsy surgery. SIGNIFICANCE: Resection should include ictal, interictal SECDs and MRI lesion localization, when feasible. Concordant ictal and interictal SECDs on MEG can be a favorable predictor of seizure freedom.

Interobserver reliability of a recently proposed semiological classification in psychogenic nonepileptic seizures in children.

BACKGROUND: The aim of this study is to examine the semiological features of Psychogenic Nonepileptic Seizures (PNES) in children and to evaluate interobserver reliability (IR) of two different classifications. to identify the sources of any variance in agreement and to estimate the IR of the classification systems METHODS: Semiological features of 137 pediatric patients with PNES with and without epilepsy were analyzed. Two different, blinded observers evaluated these semiological features according to A. Asadi-Pooya and Seneviratne et al. classifications. The interobserver reliability was measured using a kappa (κ) coefficient for each PNES classification. RESULTS: The mean age of patients with PNES was 14.3 (SD: 2.9) years. Ninety five patients were female (69.3 %), 42 were male (30.6 %). Ictal eye closure (n:109, 79.5 %), was the most common seizure semiology. Asymmetric limb movements (n: 71, 51.8 %), motor phenomenon lasting> two minutes (n:69, 50.3 %), and closed mouth (n:53, 38.6 %) were other common seizure semiologies of PNES. Kappa value was higher in A. Asadi-Pooya classification than Seneviratne classification (k = 0.697 and k = 0.433; p < 0.05). Kappa values were higher in the motor and non-motor categories of A. Asadi-Pooya classification than in the mixed category (k = 0.713, k = 0.799 and k = 0.455; p < 0.05). CONCLUSION: The added value of the new classification scheme with respect to uniform application by experienced pediatric neurologists seems to be reliable and influential, as interobserver reliability of the new classification system was higher than the early classification. Our findings suggest that a simple but comprehensive classification would be useful in the management of PNES.

Effective connectivity differs between focal cortical dysplasia types I and II.

OBJECTIVE: To determine whether brain connectivity differs between focal cortical dysplasia (FCD) types I and II. METHODS: We compared cortico-cortical evoked potentials (CCEPs) as measures of effective brain connectivity in 25 FCD patients with drug-resistant focal epilepsy who underwent intracranial evaluation with stereo-electroencephalography (SEEG). We analyzed the amplitude and latency of CCEP responses following ictal-onset single-pulse electrical stimulation (iSPES). RESULTS: In comparison to FCD type II, patients with type I demonstrated significantly larger responses in the electrodes near the ictal-onset zone (<50 mm). These findings persisted when controlling for the location of the epileptogenic zone, as noted in patients with temporal lobe epilepsies, as well as controlling for seizure type, as noted in patients with focal to bilateral tonic-clonic seizures (FBTCS). In type II, the root mean square (RMS) of CCEP responses dropped substantially from the early segment (10-60 ms) to the middle and late segments (60-600 ms). The middle and late CCEP latency segments showed the largest differences between FCD types I and II. SIGNIFICANCE: Focal cortical dysplasia type I may have a greater degree of cortical hyperexcitability as compared with FCD type II. In addition, FCD type II displays a more restrictive area of hyperexcitability in both temporal and spatial domains. In patients with FBTCS and type I FCD, the increased amplitudes of RMS in the middle and late CCEP periods appear consistent with the cortico-thalamo-cortical network involvement of FBTCS. The notable differences in degree and extent of hyperexcitability may contribute to the different postsurgical seizure outcomes noted between these two pathological substrates.

Validation of semi-automated anatomically labeled SEEG contacts in a brain atlas for mapping connectivity in focal epilepsy.

OBJECTIVE: Stereotactic electroencephalography (SEEG) has been widely used to explore the epileptic network and localize the epileptic zone in patients with medically intractable epilepsy. Accurate anatomical labeling of SEEG electrode contacts is critically important for correctly interpreting epileptic activity. We present a method for automatically assigning anatomical labels to SEEG electrode contacts using a 3D-segmented cortex and coregistered postoperative CT images. METHOD: Stereotactic electroencephalography electrode contacts were spatially localized relative to the brain volume using a standard clinical procedure. Each contact was then assigned an anatomical label by clinical epilepsy fellows. Separately, each contact was automatically labeled by coregistering the subject's MRI to the USCBrain atlas using the BrainSuite software and assigning labels from the atlas based on contact locations. The results of both labeling methods were then compared, and a subsequent vetting of the anatomical labels was performed by expert review. RESULTS: Anatomical labeling agreement between the two methods for over 17 000 SEEG contacts was 82%. This agreement was consistent in patients with and without previous surgery (P = .852). Expert review of contacts in disagreement between the two methods resulted in agreement with the atlas based over manual labels in 48% of cases, agreement with manual over atlas-based labels in 36% of cases, and disagreement with both methods in 16% of cases. Labels deemed incorrect by the expert review were then categorized as either in a region directly adjacent to the correct label or as a gross error, revealing a lower likelihood of gross error from the automated method. SIGNIFICANCE: The method for semi-automated atlas-based anatomical labeling we describe here demonstrates potential to assist clinical workflow by reducing both analysis time and the likelihood of gross anatomical error. Additionally, it provides a convenient means of intersubject analysis by standardizing the anatomical labels applied to SEEG contact locations across subjects.

How do children with drug-resistant epilepsy sleep? A clinical and video-PSG study.

AIM: The aim of this study was to assess sleep architecture and sleep problems among three homogenous groups of children including children with drug-resistant focal epilepsy, children with newly diagnosed, drug-naïve focal epilepsy, and healthy children using overnight video-polysomnography (V-PSG) and a sleep questionnaire. METHODS: We compared sleep architecture among 44 children with drug-resistant focal epilepsy, 41 children with newly diagnosed, drug naïve focal epilepsy, and 36 healthy children. All children underwent an overnight V-PSG recording, and their parents completed the Children's Sleep Habits Questionnaire (CSHQ). Sleep recordings were scored according to the American Academy of Sleep Medicine criteria. RESULTS: Compared with children with newly diagnosed epilepsy and healthy controls, children with drug-resistant epilepsy receiving antiepileptic treatment showed disturbed sleep architecture, a significant reduction in time in bed, total sleep time, sleep efficiency, NREM3%, REM%, and a significant increase in awakenings, wake after sleep onset, and periodic leg movement. Children with drug-naïve, newly diagnosed focal epilepsy showed a statistically significant increase in sleep onset latency, rapid eye movement (REM) latency, N1%, awakenings, and a significant decrease in time in bed when compared with the controls. Children with drug-resistant epilepsy had the highest CSHQ total scores, while children with drug-naïve, newly diagnosed focal epilepsy had higher scores than healthy children. CONCLUSION: This is one of the few polysomnographic studies adding to the limited research on the sleep macrostructure of children with drug-resistant epilepsy compared with children with drug-naïve, newly diagnosed focal epilepsy and healthy children by obtaining objective measurements of sleep concurrently with a validated questionnaire. Children with drug-resistant epilepsy had a greater incidence of sleep disturbance on the basis of qualitative aspects and architecture of sleep than children with newly diagnosed epilepsy, suggesting the need for referral of children with drug-resistant epilepsy for overnight sleep evaluation in order to improve the clinical management and optimize therapeutic strategies.

Two different missense mutations of PEX genes in two similar patients with severe Zellweger syndrome: an argument on the genotype-phenotype correlation.

Background Peroxisomal biogenesis disorders (PBDs) include a miscellaneous group of diseases which cause serious multisystem disease. Mutations of 13 different PEX genes lead to PBDs including Zellweger syndrome (ZS). Different types of mutations of PEX1 and PEX10 genes are correlated with broad-range phenotypes of PBDs. Case presentation Patient 1 is a 4-month-old boy who was affected by myoclonic seizures, poor oral feeding since birth. The patient was hypotonic and had hepatosplenomegaly. Patient 2 is a 2-month-old boy who presented with decreased movement, severe hypotonia and failure to thrive. The laboratory studies of the patients revealed increased plasma very-long-chain fatty acids (VLCFAs). The genetic analyses of patient 1 demonstrated the first homozygous missense mutation in the PEX10 gene. A novel homozygous missense mutation was found in the PEX1 gene in patient 2. Conclusions This report highlights that the detected homozygous missense mutations of PEX10 and PEX1 genes and the substitutions of specific amino acids lead to the severe form of PBDs.

Neonatal Seizures and Future Epilepsy: Predictive Value of Perinatal Risk Factors, Electroencephalography, and Imaging.

CONTEXT: There are limited data in the literature about the relationship between neonatal seizures and subsequent epilepsy. AIMS: This study aimed to identify the predictive value of perinatal factors, etiologies, electroencephalography (EEG), and cranial ultrasonography (USG) for future epilepsy after neonatal seizures. MATERIALS AND METHODS: A total of 92 children with epilepsy who had seizures during their neonatal period were retrospectively evaluated whether the contribution of perinatal, natal, and postnatal risk factors confining clinical, laboratory, EEG, and imaging to subsequent epilepsy. Chi-square, uni, and multivariate logistic regression were applied to find out predictive factors for subsequent epilepsy. RESULTS: The rate of epilepsy was 57.6 % during 1-6 years follow-up. Birth weight, Apgar scores at first and fifth minutes, resuscitation history, abnormal neurological examination, etiology, response to the treatment, abnormal EEG, or USG findings were the most important risk factors for future epilepsy in univariate analysis (P < 0.05). Furthermore, asphyxia, fifth minute Apgar scores, response to the treatment, USG, and EEG were independent predictors (P < 0.05) for subsequent epilepsy in multivariate logistic regression. No relationship was found between subsequent epilepsy and mode of delivery, seizure onset time, and seizure types (P > 0.05). CONCLUSION: Although there are recent promising and advanced techniques in neonatal intensive care units, asphyxia is still one of the most important risk factors for not only poor neurological conditions but also for future epilepsy after neonatal seizures. Apgar scores, treatment with multiple antiepileptic drugs, poor background EEG activity, and abnormal neuroimaging seem to have strong predictive values for developing subsequent epilepsy. Therefore, patients with a history of neonatal seizures should be closely followed up to decrease the risk of long-term outcomes and early detection of epilepsy.

Ictal pouting ('Chapeau de gendarme') in three pediatric cases with cortical dysplasia.

Ictal pouting (Chapeau de gendarme sign) can be described as an inverted smile. It consists of a turned down mouth with the contraction of the chin, wrinkling of the lips and symmetrical lowering of the labial commissures. This shape resembles the gendarme's hat during Napoléon I's time. Chapeau de gendarme sign is frequently seen in frontal and temporal lobe seizures. Focal cortical dysplasias are intrinsically epileptogenic foci and are frequently seen in patients with ictal pouting in seizure semiology. In this report, we analyzed clinical data, video EEG recordings and brain imagings of three children presenting with ictal pouting semiology in whom patients' magnetic resonans images (MRIs) or positron emission tomographies (PETs) were positive or doubtful for FCD in all. In case 1 and 2 the epileptogenic zones were temporal or temporoinsular. In these patients, with involvement of temporal lobe, dystonia and automatisms were seen in the seizure semiology after chapeau de gendarme sign. In case 3 with frontal lobe origin, hypermotor movements were seen after ictal pouting. In the patients 1 and 2, the cortical dysplasias were in temporal lobe. In patient 3, PET demonstrated hypometabolism on left inferior frontal gyrus but we couldn't verify this finding with MRIs. Ictal pouting (Chapeau de gendarme sign) is a distinct seizure semiology that can often be overlooked and coexist with focal cortical thickening. We suggest that focal cortical dysplasias should be searched in patients with ictal pouting particularly in those with refractory focal seizures.

Citrullinemia with an Atypical Presentation: Paroxysmal Hypoventilation Attacks.

Citrullinemia type 1 (CTLN1) is a rare inherited urea cycle disorder, which resulted from the deficiency of argininosuccinate synthetase enzyme. We presented an infant who was hospitalized because of acute losses of tonus and cyanotic hypoventilation attacks lasting approximately 4-5 min. The physical and neurological examinations were normal. Ammonia level was in the normal range. Citrulline levels increased in both blood and urine. The blood sample was sent to mutation analysis, which showed one novel and one known mutation on ASS1 gene sequencing: a heterozygous novel mutation p.A94V (c.281C>T) and a heterozygous mutation p.W179R (c.535C>T). Urea cycle disorders should be considered in the differential diagnosis of unexplained brief apnea or hypoventilation attacks, even though those symptoms do not lead to hyperammonemia during infancy and childhood as seen in our patient. This is the first case in terms of atypical clinical presentation with a new mutation for CTLN1.

What has changed in the utility of pediatric EEG over the last decade?

Background/aim: We evaluated the utility of electroencephalography (EEG) in children with neurological conditions and compared the results with those of our previous study on excessive uses of pediatric EEG, which was published in 2003. We also evaluated the possibility of subsequent EEGs and satisfactory duration of EEG recordings according to EEG type and admission status. We also evaluated the yield of varying durations of EEG recordings. Materials and methods: All consecutive pediatric EEG records performed at Gazi University EEG laboratory during a 1-year period were retrospectively reviewed. The indications of EEGs, the number of EEGs for each patient, condition and duration of EEG records, and activation techniques were evaluated in terms of detection of abnormalities by EEG. Results: We reviewed a total of 2045 EEGs in children aged 2 months­20 years. Of these, 776 were repeat EEGs (38%) and 485 recordings were ≥30 min (23.7%); 98% of EEG abnormalities appeared in the first 30 min. Overall, 90.5% of EEGs were ordered by a pediatric neurologist. There were similar requests for numbers of EEGs, but the rate of abnormal EEGs (43.6%) was significantly higher when compared to that of our previous study (36.2%). Conclusion: The results of this study show that the utility of EEG becomes more selective and interpretation of pediatric EEG improves depending on the increasing number of pediatric neurologists. A duration of 20­30 min of EEG recording is sufficient, on the condition of inclusion of nREM sleep records.

Aggravation of seizures and status epilepticus after vagal nerve stimulation therapy: the first pediatric case and review of the literature.

BACKGROUND: We describe the first child with drug-resistant epilepsy in whom vagus nerve stimulation aggravated seizures and emerged status epilepticus after the increase in vagal nerve stimulation current output. OBJECTIVE: A 13-year-old girl presented with refractory secondary generalized focal epilepsy. Vagal nerve stimulator was implanted because of drug-resistant epilepsy. After the increase of vagal nerve stimulator current output to a relatively high level, the patient experienced seizure aggravation and status epilepticus. CONCLUSION: We conclude that vagus nerve stimulation may induce paradoxical seizures and may lead to status epilepticus, similarly to some antiepileptic drugs.

Serial changes in the paroxysmal discharges in rolandic epilepsy may predict seizure recurrence: A retrospective 3-year follow-up study.

OBJECTIVE: The aim of this study was to assess the electrographic criteria related to seizure recurrence and determine age-related seizure recurrence in children with rolandic epilepsy under long-term follow-up. METHODS: We retrospectively analyzed the data belonging to 109 patients with rolandic epilepsy with sufficient information regarding disease course and follow-up duration longer than 3years. Patients were divided into two categories: Group A (n: 75), comprised of "patients having fewer than four seizures", and Group B (n: 34), the "recurrence group comprised of patients having more than four seizures in the first three months". The number of spikes per minute during both wakefulness and sleep, the localization of spikes other than centrotemporal region, and the duration of spike-wave activity were evaluated longitudinally, with repeated electroencephalogram (EEG) recordings every 6months. RESULTS: The appearance of rolandic spikes in awake EEGs tended to be more prevalent in Group B than Group A. In Group B, spike rates significantly increased in the 12 and 18months after onset whereas spike rates increased significantly only 6months after onset in Group A. Seizure recurrence is mostly seen at 6-8years, and improvement becomes evident by age 12. The mean number of paroxysmal rolandic discharges during sleep was significantly higher in the younger age groups (3-5, 6-8), and the mean number of spikes per minute significantly decreased at ages 9-11 and over 12. CONCLUSION: Our study demonstrates that extended periods of high frequency of paroxysmal discharges, initial frontal EEG focus, and persistence of awake interictal abnormalities are highly effective in predicting seizure recurrence in patients with rolandic epilepsy (RE).

Effects of vagus nerve stimulation on heart rate variability in children with epilepsy.

PURPOSE: The aim of this study was to evaluate the effects of vagus nerve stimulation (VNS) on heart rate variability (HRV) in children with epilepsy. METHODS: The subgroups of HRV, namely time domain (Standard deviation of NN interval (SDNN), SDNN index, Standard deviation of the averages of NN intervals (SDANN), Root mean square of successive differences (RMMSD), Adjacent NN intervals differing by more than 50 ms in the entire recording divided by the total number of all NN intervals (PNN50), triangular index) and frequency domain (Low-frequency (LF), High-frequency (HF), LF/HF), were investigated in 20 pediatric patients before and after 6 and 12months of VNS treatment during day and night by comparing their data with those of 20 control subjects. In addition, subgroups of age, epilepsy duration and localization, and antiepileptic drugs (AEDs) were also evaluated if they had further effects on basal HRV levels. RESULTS: Increased heart rates (HRs); decreased SDNN, SDANN, RMMSD, and PNN50; and increased LF/HF ratios were identified before VNS therapy (p<0.05). Even though remarkable improvement was seen after 6months of VNS treatment (p<0.05), no further changes were observed in 12-month compared with 6-month levels (p>0.05) in all parameters, still even significantly lower than those of controls (p<0.05). Longer duration of epilepsy and localization of epileptic focus, such as in the temporal lobe, were also found to further contribute to diminished basal HRV levels (p<0.05). CONCLUSION: The cardiovascular system is under deep sympathetic influence in children with epilepsy. Although VNS seems to provide a substantial improvement by achieving increased parasympathetic effects in short-term therapy, the levels were still lower than those of healthy children after either short- or long-term therapy. Therefore, impaired cardiovascular autonomic regulation may be associated with the epileptic process itself as well as with the contribution of some additional factors. Overall, different aspects such as age, epilepsy duration, epileptic focus, seizure frequency, and AEDs should also be considered for their further possible effects on HRV during VNS therapy.

Neonatal hypoglycemia: A wide range of electroclinical manifestations and seizure outcomes.

PURPOSE: We examined the various types of epilepsy in children with neonatal hypoglycemia in order to define electroclinical and prognostic features of these patients. METHOD: We retrospectively reviewed the medical records of patients with a history of symptomatic neonatal hypoglycaemia who have been followed at Gazi University Hospital Pediatric Neurology Department between 2006 and 2015. Patients with perinatal asphyxia were excluded. Details of each patient's perinatal history, neurological outcome, epilepsy details, seizure outcome and EEG and brain MRI findings were reviewed. RESULTS: Fourty five patients (range 6 mo-15 y) with a history of symptomatic neonatal hypoglycaemia were included the study. Epilepsy developed in 36 patients and 23 of them had intractable epilepsy. All patients had occipital brain injury. CONCLUSION: We observed that most of the patients, either manifesting focal or generalized seizures, further develop intractable epilepsy. This finding establishes neonatal hypoglycemia as a possible cause to be considered in any case of intractable epilepsy.

The efficacy of orbital ultrasonography and magnetic resonance imaging findings with direct measurement of intracranial pressure in distinguishing papilledema from pseudopapilledema.

INTRODUCTION: The goal of this study was to evaluate the utility of orbital ultrasonography and magnetic resonance imaging in the diagnosis of idiopathic intracranial hypertension (IIH). METHOD: We reviewed the medical records of patients referred to our department for suspected IIH. RESULTS: Seven children were diagnosed with IIH. Nine children revealed pseudopapilledema by optic coherence tomography and/or orbital ultrasonography. When the axial sequences were reexamined, patients with papilledema had optic nerve sheath (ONS) enlargement (6.62 ± 0.70 mm); patients with pseudopapilledema had ONS diameter as 4.62 ± 0.64 mm. There was a significant correlation between the CSF opening pressure and ONS diameter (p < 0.005, r = 0.661). In the papilledema group, the presence of proposed subtle markers as increased tortuosity in the optic nerve was found in six patients. Five of seven patients had a target sign, intraocular protrusion of the optic nerve, and posterior globe flattening. DISCUSSION: Ophthalmological review is important to avoid unnecessary procedures for detection of true papilledema. ONS diameter is a reliable neuroimaging marker as other subtle markers.

Comparison of ILAE 2010 and semiological seizure classification in children with epilepsy.

OBJECTIVE: The aim of this study was to compare both ILAE 2010 and semiological seizure classification (SSC) in terms of their applicability and utility and to predict epileptogenic zone in children with epilepsy. METHODS: Both ILAE 2010 classification and SSC which is a part of five dimensional classification were applied for a total 138 children by dividing into younger (≤6y/o) and older (>6y/o) age groups over the last two years as retrospectively. After the assessment of the seizures types, epilepsy syndromes, and etiologies; the data were also compared to evaluate if having correlation between epileptogenic zone and seizure subtypes in both ILAE 2010 and SSC. RESULTS: ILAE 2010 indicated that 66.7% of the patients had focal seizures, 15.9% had generalized seizures, and 14.4% had seizures of unknown origin. The SSC revealed that the most frequent seizure type was simple seizures (56.5%), second frequent one was complex motor seizures (46.4%) and dialeptic seizures (39.9%). To predict epileptogenic zone, SSC was found to be more specific than ILAE in terms of the more subgroups of SSC were related to the more subgroups of epileptogenic zone (p<0.05). Furthermore, there was a clear correlation between focal foci and specific seizure types in older ages, while many foci caused to same seizure types and tend to no clear focal foci with generalized onset in younger ages. On the other hand, the relationship between epileptogenic zone and etiology was more remarkable in the five dimensional classification. SIGNIFICANCE: Preference of seizure classification system is unique for each patient and depends on requirements. Therefore, one dimension cannot be sufficient for evaluation the nature of the seizures in some patients. Furthermore, age related evolution of the seizure types should not be ignored due to ongoing maturation state of the brain. ILAE 2010 and SSC have weak and strong points compared to each other. Semiological seizure classification is more informative in terms of identifying the epileptogenic zone which may be important in specific occasions like pre-surgical work up, while ILAE is simple and easier method which can be applied for seizure description and their characteristics in daily practice.

Outcomes of resective surgery in children and adolescents with focal lesional epilepsy: The experience of a tertiary epilepsy center.

OBJECTIVE: This study aimed to investigate the efficacy of resective surgery in children with focal lesional epilepsy by evaluating the predictive value of pre- and postsurgical factors in terms of seizure freedom. METHODS: This study included 61 children aged between 2 and 18years who were admitted to the pediatric video-EEG unit for presurgical workup. Each patient was evaluated with a detailed history, video-EEG, neuroimaging, and postsurgical outcomes according to Engel classification to predict postsurgical seizure freedom. All the possible factors including history, etiology, presurgical evaluation, surgical procedures, and postsurgical results were analyzed for their predictive value for postoperative seizure freedom. RESULTS: Of the 61 patients, 75% were diagnosed as having temporal lobe epilepsy (TLE), and 25% were diagnosed with extra-TLE. Two years after the surgery, 78.6% were seizure-free, of which 89% had TLE, and 50% had extra-TLE (p<0.05). Patients were more likely to have a favorable outcome for seizure freedom if they had rare seizure frequency, focal EEG findings, and focal seizures; had a temporal epileptogenic zone; or had TLE and hippocampal sclerosis. On the other hand, patients were more likely to have unfavorable results for seizure freedom if they had younger age of seizure onset, frequent seizures before the surgery, a frontal or multilobar epileptogenic zone, secondarily generalized seizures, extra-TLE with frontal lobe surgery, or focal cortical dysplasia. SIGNIFICANCE: Resective surgery is one of the most effective treatment methods in children with intractable epilepsy. A history of young age of seizure onset, frequent seizures before surgery, secondarily generalized seizures, a multilobar epileptogenic zone, frontal lobe surgery, and focal cortical dysplasia (FCD) are the most important predictive factors indicating that a patient would continue having seizures after surgery. On the other hand, focal seizure semiologies, temporal lobe localization, and hippocampal sclerosis indicate that a patient would have better results in terms of seizure freedom.

Sleep-wake distribution and circadian patterns of epileptic seizures in children.

BACKGROUND: Epilepsy is one of the most common chronic neurologic disorders. Daily periodicity of epileptic seizures has been known for over a century. The diurnal patterns of epileptic seizures have also been observed in studies. AIM: To investigate the sleep/wake cycle, day/night, and 24-h periodicity of various seizure subtypes and seizure onset localizations in children. METHODS: We analyzed the clinical seizures of 170 consecutive epilepsy patients who underwent video-electroencephalography (EEG) monitoring over the last 5 years. Semiology of the seizures was classified according to the semiological seizure classification. Origin of the seizures was defined by the onset of ictal activity on EEG. Seizures were evaluated in terms of occurrence during the day (06:00-18:00 h) or night (18:00-06:00 h), in wakefulness or in sleep, and within a 3-h time interval throughout 24 h. RESULTS: A total of 909 seizures were analyzed. Auras, dialeptic, myoclonic, hypomotor, atonic seizures, and epileptic spasms occurred more frequently in wakefulness; tonic, clonic, and hypermotor seizures occurred more frequently in sleep. Auras, dialeptic, and atonic seizures and epileptic spasms occurred more often during daytime; hypermotor seizures occurred more often at night. Generalized seizures were seen most frequently in wakefulness (between 12:00 and 18:00 h); frontal lobe seizures were seen at night and in sleep (between 24:00 and 03:00 h); temporal lobe seizures were seen in wakefulness (between 06:00 and 09:00 h and between 12:00 and 15:00 h); occipital seizures were seen during daytime and in wakefulness (between 09:00 and 12:00 h and between 15:00 and 18:00 h, respectively); parietal seizures were seen mostly during daytime. CONCLUSIONS: Seizures in children occur in specific circadian patterns and in specific sleep/wake distributions depending on seizure onset location and semiology.

Long term effect of vagus nerve stimulation in pediatric intractable epilepsy: an extended follow-up.

PURPOSE: Over the past two decades, vagus nerve stimulation (VNS) has become an accepted and viable treatment modality for intractable epilepsy both in children and adults. Earlier studies have demonstrated short-term seizure outcomes, usually for up to 5 years; so far, none have reported an extended outcome in children. We aimed to assess long term seizure outcome in children with intractable epilepsy for more than 5 years. METHODS: We identified patients who had VNS implantation for treatment of intractable epilepsy from March 2000 to March 2015 at our Epilepsy Center and collected data including demographic, age at epilepsy onset and VNS implantation, duration of epilepsy, seizure type, number of antiepilepsy drugs (AEDs), and monthly seizure frequency before VNS implantation and at the last clinic visit. Phone surveys were conducted with patients without recent clinic follow-up. RESULTS: Fifty-six patients (aged 4-17 at the time of implant) are the subjects of the study. Seizure reduction of >50 % was achieved in 9.8 % (6th month), 24 % (2nd year), 46.4 % (3rd year), and 54 %(5th year), and overall 35 (62.5 %) of the 56 subjects had a greater than 50 % reduction in seizure frequency at the last follow-up. Eleven patients became seizure free. The results, once obtained, were maintained steadily or even improved over time without any loss of efficacy during the follow-up. The only parameter, significantly related with clinical response, was age at seizure onset. The most frequent adverse events were hoarseness, cough, sore throat, and anorexia, experienced by 13 patients. Two patients had local wound infections and lead to the removal of the stimulator. An improvement in alertness, attention, and psychomotor activity, independent of the efficacy of vagal nerve stimulation, was observed in 8 patients. CONCLUSION: To our knowledge, this is the first pediatric study evaluating seizure outcome over more than 5 years of follow-up, and demonstrates a favorable seizure outcome of >50 % seizure frequency in 62.5 % of patients and seizure freedom in 11 patients. It is well tolerated over an extended period of time.