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OBJECTIVE: Our aim in this study is to evaluate epilepsy classification in children with epilepsy before monitoring (based on information received from the family) and after monitoring (based on video-EEG) by comparing two separate classification systems, namely the 2017 International League Against Epilepsy (ILAE) and Semiological Seizure Classification (SSC) systems. Classifications and methods were compared in terms of simplicity, intelligibility, and applicability during daily outpatient care. PATIENTS AND METHODS: The study was performed with 230 recorded seizures of 173 patients aged between 1 and 18 years who underwent video-EEG monitoring and clinical seizure recordings. Seizure types and video-EEG data of the patients were recorded. Seizures were first classified based on information obtained from the parents of the patients in interviews, recorded as "before video-EEG monitoring classification," and a second characterization, based on video EEG-monitoring, was subsequently recorded as "after video-EEG monitoring classification". The consistency of both seizure classifications was evaluated. RESULTS: For both classifications, autonomic seizures were the least congruent seizures (1=0.27, 1=-0.005). The families generally described the seizures very well; the consistency before and after video-EEG monitoring was good. Focal seizures with impaired awareness were most common in the 2017 ILAE classification (1=0.6), while for the SSC simple motor seizures were most common (1=0.84). Among subtypes, clonic-tonic seizures were the most common, and the second most common subtype was dialeptic (1=0.67). Overall, the harmony between the SSC and ILAE systems was good. The rate of good and excellent coefficients of concordance for both the SSC and 2017 ILAE was determined as 77.8% for the expanded SSC, 48% for the 2017 ILAE, 71.4% for the basic SSC, and 60% for the 2017 ILAE. CONCLUSIONS: In practice, it is difficult to determine seizure patterns reliably in cases of childhood epilepsy. Parents, however, can generally describe seizures very well. Although the SSC seems to be superior, both the SSC and 2017 ILAE systems can be applied in daily use. Such classification enables the rise of new concepts and a better understanding of disease groups. The continuing development of classification systems will lead to advancements for patients. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Benign childhood epilepsy with centro-temporal spikes (BCECTS), otherwise known as benign rolandic epilepsy, is the most common focal epilepsy in childhood. This study aimed to evaluate the development and resolution of ESES in children with BCECTS and evaluate the clinical and electroencephalography (EEG) parameters associated with prognosis. PATIENTS AND METHODS: Resolution of ESES was defined as the reduction of the spike-wave index (SWI) to <50%. The SWI short method, measurements from the first 180 s of non-rapid eye movement; and the conventional method, measurements from total NREM stage 2, SW count during the first 60 and 180 s of NREM, SW localization, and ESES type were determined. RESULTS: Of a total of 126 BCECTS patients, 33, including 13 females, 20 males, who developed ESES during follow-up, were included in the study. ESES remission was observed in 42.4% (n = 14) of the patients. The median time to remission was 10.5 months. The rate of resolution was 87.9 % for the entire population. The mean age at resolution was 9.8 ± 2.05 years and the mean time to resolution was 8.8 months. CONCLUSIONS: The data demonstrated that age at ESES diagnosis, the time between BCECTS diagnosis and the onset of ESES, time to resolution of ESES, ESES remission, and seizure freedom after ESES were significantly associated with prognosis. The early recognition of ESES evolution in children with BECTS, the better understanding of the relationship between age at ESES diagnosis and remission and prognosis, and timely intervention can prevent long-term sequelae. [ABSTRACT FROM AUTHOR]

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., Competing Interests: Conflicts of interest The authors report no competing interests., (Copyright © 2022 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

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., Competing Interests: Conflicts of interest There are no conflicts of interest., (Copyright © 2022 Elsevier B.V. All rights reserved.)

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., (© 2021 International League Against 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., (© 2021 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

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., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Pediatric Neurosciences.)

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., (Copyright © 2019 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2016 Elsevier B.V. All rights reserved.)

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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., (Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

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.

Background: Increased intracranial hypertension is defined as elevated intracranial pressure with absence of hydrocephalus, vascular or structural abnormalities, and normal cerebrospinal fluid content. Magnetic resonance imaging abnormalities of the optic nerve and sheath that have been described in adults include increased nerve tortuosity, flattening in posterior aspect of globe, intraocular protrusion of the optic nerve, and enlarged optic nerve sheath., Purpose: We evaluated accuracy of those proposed markers on magnetic resonance imaging in children with increased intracranial hypertension that are described in adults., Materials and Methods: Eleven patients between 3 and 15 years of age with intracranial hypertension were selected for re-evaluation of magnetic resonance imaging that had been previously described as normal to determine the presence of: (1) increased tortuosity and elongation of the optic nerve; (2) enlargement of the optic nerve sheath on axial and coronal T2 so called by us "target sign" and postcontrast T1 sequences; (3) flattening in posterior aspect of the globe; and (4) intraocular protrusion of the optic nerve head., Results: Of the 11 patients, tortuosity of the optic nerve (10/11, 90.9%) and enlarged optic nerve sheath--target sign (7/11, 63.6%)--were the most common findings. Flattening in the posterior aspect of globe (5/11, 45.5%) and intraocular protrusion (3/11, 27.3%) were also detected as a novel magnetic resonance imaging findings., Conclusion: Magnetic resonance imaging findings of the optic nerve and sheath include valuable signs of intracranial hypertension not only in adults but also in children. This is the first detailed analysis of the magnetic resonance imaging findings in children with increased intracranial hypertension., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to determine the long-term surgical outcomes of magnetic resonance imaging (MRI)-negative, fluorodeoxyglucose positron emission tomography (FDG-PET)-positive patients with temporal lobe epilepsy (TLE) and compare them with those of patients with mesial temporal sclerosis (MTS)., Methods: One hundred forty-one patients with TLE who underwent anterior temporal lobectomy were included in the study. The surgical outcomes of 24 patients with unilateral temporal hypometabolism on FDG-PET without an epileptogenic lesion on MRI were compared with that of patients with unilateral temporal hypometabolism on FDG-PET with MTS on MRI (n=117). The outcomes were compared using Engel's classification at 2 years after surgery. Clinical characteristics, unilateral interictal epileptiform discharges (IEDs), histopathological data and operation side were considered as probable prognostic factors., Results: Class I surgical outcomes were similar in MRI-negative patients and the patients with MTS on MRI (seizure-free rate at postoperative 2 years was 79.2% and 82% in the MRI-negative and MTS groups, respectively). In univariate analysis, history of febrile convulsions, presence of unilateral IEDs and left temporal localization were found to be significantly associated with seizure free outcome. Multivariate analysis revealed that independent predictors of a good outcome were history of febrile convulsions and presence of unilateral IEDs., Conclusion: Our results suggest that epilepsy surgery outcomes of MRI-negative, PET positive patients are similar to those of patients with MTS. This finding may aid in the selection of best candidates for epilepsy surgery., (Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

A 12-month old boy presented with intractable seizures present since 3-month of age. He had, previously, been admitted numerous times to the pediatric emergency room for intractable and prolonged seizures during the course of his disease. Differential diagnosis was made to exclude several inborn metabolic disorders, including vitamin B6 deficiency, biotinidase deficiency and nonketotic hyperglycinemia. Although the initial brain MRI revealed a mild cerebral and cerebellar white matter involvement, follow-up images showed diffuse cerebral and cerebellar white matter dysmyelination, progressive rarefaction and cystic degeneration. A genetic analysis was performed for vanishing white matter (VWM) disease and a homozygote c. 1091G>A mutation was detected at the EIF2B4 gene. This case emphasizes the fact that VWM disease may present with refractory seizures since early infancy.

Of 105 children with epilepsy who had presurgical evaluation because of solitary extratemporal focal cortical dysplasia, the 5 cases reported here had a final diagnosis of tuberous sclerosis complex without classic findings on magnetic resonance imaging (MRI). Four were infants with spasms (n = 2) or partial seizures (n = 2); the fifth was a 12-year-old boy with right motor seizures. Cranial MRI indicated solitary left frontal dysplasia in two cases (one with calcification) and right frontal, left parietal, and right parietal dysplasia in one case each. Tuberous sclerosis complex was considered based on hypopigmented macules in three cases, on cardiac rhabdomyomas on echocardiography for hypotension in one case, and on calcification in the dysplastic region and a family history of seizures in one case. Four of the patients had mutations in the TSC1 (n = 2) and TSC2 (n = 2) genes. No mutation was found in the fifth patient, who had only TSC1 sequencing. Four had epilepsy surgery, and were seizure-free on one (three cases) or two antiepileptic drugs (one case) at 1-2 year follow-up. Pathologic examination revealed cortical dysplasia without any characteristic features of tuberous sclerosis complex. These findings suggest that, in tuberous sclerosis complex, solitary focal cortical dysplasia may be the only cranial MRI finding. Tuberous sclerosis complex should be considered when focal cortical dysplasia is associated with seizure onset in infancy, family history of seizures, and peridysplastic calcification., (Copyright 2010 Elsevier Inc. All rights reserved.)

The study objective was to evaluate levels of the cytokines tumor necrosis factor alpha, interleukin-1beta, and interleukin-6 and of leptin, and then to determine the relationship between these levels and clinical responses in children with migraine after prophylactic therapy with one of four drugs. In all, 77 children who needed prophylactic drugs were treated with cyproheptadine, amitriptyline, propranolol, or flunarizine. Serum levels of the cytokines and leptin were measured before and 4 months after the treatment. Results were compared by drug for headache frequency, severity, and duration, the PedMIDAS score, and levels of each cytokine and of leptin. Each of the four drugs not only decreased the frequency and duration but also the severity of headache, and the PedMIDAS score. None of the drugs was found to be superior to others in terms of reduction in cytokine levels (P > 0.05). Both cyproheptadine and flunarizine (but not amitriptyline and propranolol) caused an increase in leptin levels (P < 0.05). These data suggest that cytokine levels are related to clinical responses, and might help in objective evaluation of clinical response in migraine. To our knowledge, the present study is the first trial to compare the effects of prophylactic drugs, cytokine levels, and leptin levels in children with migraine.

The main goal of this study was to evaluate knowledge of, perceptions of, and attitudes toward epilepsy and then to correlate knowledge with quality of life and stigmatization of children with epilepsy and their families. Specific questionnaires were administered to children aged 8 to 17 with epilepsy (n=220) and their parents (n=313). Poor school performance, less social support, less self-esteem, higher anxiety, greater stigmatization, and more depressive symptoms were documented in children who were less knowledgeable (P<0.05). Parents were found to be more knowledgeable about the antiepileptic drugs used, understanding both the effects and the side effects of the medications (P<0.05). Family activities were less restricted if they were more knowledgeable and these parents reported worrying less about their children (P<0.05). Knowledge about epilepsy is associated with less perceived stigmatization and social isolation, as well as fewer depressive symptoms and misperceptions.

The study objective was to assess the applicability and reliability of the semiological seizure classification in children with epilepsy in outpatient clinics. Ninety patients (age range, 2-16 years) who experienced clinical seizures during prolonged video-electroencephalogram (EEG) monitoring were evaluated. Semiological seizure classification was performed, first based on history obtained from parents of the patient during outpatient follow-up visits and then based on video EEG-monitoring. Kappa statistics (kappa) were used to evaluate the consistency of the two rounds of semiological seizure classification. Classification based on history yielded the following distribution: simple motor seizures (66.3%), aura (28%), complex motor seizures (15.8%), special seizures (15.8%), dialeptic seizures (9.3%), and autonomic seizures (3.7%). Classification based on video EEG-monitoring yielded a different distribution: simple motor seizures (55.7%), complex motor seizures (26.9%), automotor seizures (26.9%), aura (23%), dialeptic seizures (22.1%), special seizures (9.6%), and autonomic seizures (1.9%). Negative myoclonic seizures (kappa = 1, P = 0.000) and hypermotor seizures (kappa = 0.85, P = 0.000) had excellent consistency; somatosensory aura (kappa = 0.26, P = 0.012) and automotor seizures (kappa = 0.28, P = 0.004) had the lowest consistency. The families or doctors often defined simple motor seizures (decrease of 10.6% from before to after monitoring, kappa = 0.44); the proportion of complex motor seizures changed rather from before to after monitoring (11.1%, kappa = 0.33). Generally, parents can describe seizures quite well. We suggest that semiological seizure classification is a reliable method applicable for everyday use during outpatient visits, especially if seizure semiology is evaluated individually for each component or if the semiological seizure classification is modified or refined for some seizure components (tonic, clonic, versive, conscious, automotor seizures).

Hereditary spastic paraplegia is composed of a heterogeneous group of neurodegenerative disorders and is classified as pure or complicated due to its clinical variability. Autosomal recessive hereditary spastic paraplegia with hypoplastic corpus callosum is a rare form of complicated hereditary spastic paraplegia. In complicated hereditary spastic paraplegia, autosomal dominant, autosomal recessive, and X-linked modes of inheritance have been noted. The diagnostic criteria of autosomal recessive hereditary spastic paraplegia with hypoplastic corpus callosum are inheritance consistent with autosomal recessive trait, slowly progressive spastic paraparesis and mental detoriation, hypoplasia of corpus callosum revealed by brain computerized tomography or magnetic resonance imaging, and exclusion of other disorders by magnetic resonance imaging of the spine and brain as well as other laboratory tests. In this report, the authors present the case of 3 affected siblings in a family from Turkey, whereas 1 child and the con-sanguineous parents were healthy. To the authors' knowledge, it is the first reported case of autosomal recessive hereditary spastic paraplegia with hypoplastic corpus callosum from Turkey.