Sleep Spindle Alterations in Children With Migraine.

BACKGROUND: The modulation of thalamocortical activity is the most important site of several levels of interference between sleep spindles and migraine. Thalamocortical circuits are responsible for the electrophysiological phenomenon of sleep spindles. Spindle alterations may be used as a beneficial marker in the diagnosis and follow-up of children with migraine. We aimed to formulate the hypothesis that there is a shared mechanism that underlies migraine and sleep spindle activity. METHODS: We analyzed the amplitude, frequency, duration, density, and activity of sleep spindles in non-rapid eye movement stage 2 sleep in patients with migraine without aura when compared with healthy control subjects. RESULTS: The amplitudes of average, slow, and fast sleep spindles were higher in children with migraine without aura (P = 0.020, 0.013, and 0.033, respectively). The frequency of fast spindles was lower in children with migraines without aura when compared with the control group (P = 0.03). Although not statistically significant, the fast sleep spindle duration in the migraine group was shorter (P = 0.055). Multivariate analysis revealed an increased risk of migraine associated with increased mean spindle amplitude and decreased fast spindle frequency and duration. CONCLUSIONS: Our data suggest that spindle alterations may correlate with the vulnerability to develop migraine and may be used as a model for future research about the association between the thalamocortical networks and migraine.

A Multicenter Study of Self-Limited Epilepsy With Centrotemporal Spikes: Effectiveness of Antiseizure Medication With Respect to Spike-Wave Index.

BACKGROUND: There is no certain validated electroencephalographic (EEG) parameters for outcome prediction in children with self-limited epilepsy with centrotemporal spikes. To assess the effectiveness of antiseizure medication (ASM) for seizure outcome with respect to the spike-wave index (SWI) on serial EEG recordings. METHODS: In this multicenter study, the study cohort consisted of 604 children with self-limited epilepsy with centrotemporal spikes. A data set of epilepsy centers follow-up between 2010 and 2022. The cohort was divided into 4 groups as those receiving 3 different monotherapy (carbamazepine [CBZ]/valproic acid [VPA]/levetiracetam [LEV]) and dual therapy. SWI analysis was performed with the percent of spikes in the 2-minute epoch in the 5th 6th minutes of the nonrapid eye movement sleep EEG record. The study group were also categorized according to seizure burden with seizure frequency (I) >2 seizures and (II) >5 seizures. Seizure outcome was evaluated based on the reduction in seizure frequency over 6-month periods: (1) 50% reduction and (2) seizure-free (complete response). RESULTS: ASM monotherapy was achieved in 74.5% children with VPA, CBZ, and LEV with similar rates of 85.8%, 85.7%, and 77.9%. Dual therapy was need in the 25.5% of children with SeLECT. More dual therapy was administered in children aged below 5 years with a rate of 46.2%. Earlier seizure-free achievement time was seen in children with LEV monotherapy with more complete-response rate (86.7%) compared the VPA and CBZ. CONCLUSIONS: We also determined that the children on dual therapy had more SWI clearance in the subsequent EEG recordings. The ROC curve analyses were performed to predict initial drug selection with using the SWI% might be used for the prediction of ASM type and drug selection in children.

Electroencephalogram abnormalities in children have rotated error on block design performance: An university hospital child and adolescent psychiatry clinic sample.

OBJECTIVE: Neuropsychiatric assessment is essential part of child and adolescent psychiatry clinic practice, also provides important information about central nervous system dysfunctions. In studies conducted to date, it has been known that both the high frequency of psychiatric comorbidity in epileptic patients and that epilepsy comorbidity is quite common in neurodevelopmental disorders. In fact, considering the high comorbidity of epileptic abnormalities and psychiatric disorders, it has been very important to determine predictors for epileptic abnormalities in a clinical sample of child and adolescent psychiatry. In this retrospective study, we aim to determine possible predictive factors for epileptic abnormalities in a clinical sample of child and adolescent psychiatry according to Weschler Intelligence Scale for Children (WISC-R) results. METHODS: We identified patients who had two or more rotation errors in the block design subtest of WISC-R by retrospectively scanning the system records of 2609 cases who were applied WISC-R with different prediagnoses at Gazi University Child and Adolescent Psychiatry Outpatient Clinic between January 2013 and December 2020 (n=71). After the first step identification, we selected the ones who had a previous electroencephalography (EEG) recording available for our own re-review (n=60). RESULTS: We found 15% EEG abnormalities and ADHD is the most common diagnosis in both normal and abnormal EEG groups. Due to correlation analysis, there was a positive-mild correlation between presence of EEG abnormality and WISC-R performance (r=0.56) in intellectual disability (ID) group and a positive-strong correlation between presence of EEG abnormality and WISC-R performance-verbal scores (r=0.74) in ID group. CONCLUSION: This study has shown that many different abnormal EEG patterns can be found in patients who have rotation errors in the block design test of WISC-R, suggesting diagnoses of ID, and having notable performance-verbal subtests scores difference and rotation errors in the block design subtest of WISC-R should be predicitive factors for epileptic abnormalities.

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.

The effectiveness and tolerability of clobazam in the pediatric population: Adjunctive therapy and monotherapy in a large-cohort multicenter study.

OBJECTIVE: To evaluate the effectiveness and tolerability of clobazam therapy in the pediatric population in terms of seizure semiology, epileptic syndromes, and etiological subgroups. METHODS: A retrospective cohort study was conducted consisting of 1710 epileptic children from eight centers in seven geographic regions of Turkey. The initial efficacy of clobazam therapy was evaluated after three months of treatment. The long-term effectiveness of the drug, overall seizure outcomes, and overall therapeutic outcomes were evaluated during 12 months of therapy. RESULTS: Analysis of initial efficacy after the first three months of clobazam therapy showed that 320 (18.7 %) patients were seizure-free, 683 (39.9 %) had > 50 % seizure reductions, and 297 (17.4 %) had < 50 % seizure reductions. A positive response (seizure-free and >50 % seizure reduction) was determined for focal-onset (62.3 %) seizures, epileptic spasms (61.5 %), and generalized onset seisures (57.4). The highest positive response rate among the epileptic syndromes was for self-limited epilepsy with centrotemporal spikes (SeLECTS). The highest negative response rate was for developmental and/or epileptic encephalopathies (DEEs). Magnetic resonance imaging (MRI) revealed a structural etiological diagnosis in 25.8 % of the cohort. A higher positive response rate was observed at MRI in patients with sequelae lesions than in those with congenital lesions. The seizure recurrence rate was higher in the patient group with epilepsy with genetic and metabolic causes, in individuals with more than one seizure type, and in those using three or more antiseizure drugs. CONCLUSIONS: This cohort study provides additional evidence that clobazam is an effective and well-tolerable drug with a high seizure-free rate (18.7 %), a significant seizure reduction rate (57.3 %), and with excellent overall therapeutic outcomes with a low seizure relapse rate and considerable reversible benefits in the pediatric population.

Trends in the choice of antiseizure medications in juvenile myoclonic epilepsy: A retrospective multi-center study from Turkey between 2010 and 2020.

PURPOSE: Valproic acid (VPA) is frequently used and effective in juvenile myoclonic epilepsy (JME). Recently, levetiracetam (LEV) has been suggested as a monotherapy in JME. This study aimed to evaluate antiseizure medication (ASM) use in patients with JME. METHODS: Treatment choices in a total of 257 patients (age range 8-18 years, 152 girls, 105 boys) with JME diagnosed and treated between 2010 and 2020 were evaluated retrospectively. Seizure remission was defined as complete seizure control for at least 12 months. RESULTS: Across the study period and entire patient group, VPA was most commonly chosen as the initial ASM (50.9%), followed by LEV (44.4%), and lamotrigine (4.7%). VPA was also the most frequent first choice in the subgroup of boys (73.3%), while LEV was the commonest first choice in girls (57.9%). The sex difference regarding the ASM of the first choice was statistically significant (p<0.001). While VPA was the most frequent initial ASM in the first 5 years of the study period (2010-2015,n = 66, 64%), LEV had taken over as the most popular first ASM in the last 5 years (n = 83, 53.9%, p = 0.005). The most frequent reasons for discontinuation were inefficacy for LEV and adverse effects for VPA (p = 0.001). During follow-up, 237 patients (92.2%) were seizure-free for at least 12 months, and 159 (61.9%) were also in electrographic remission. Seizure remission occurred earlier than electroencephalographic remission (p<0.001). CONCLUSION: This study revealed that LEV has become the most frequently chosen initial ASM in the treatment of JME. Although LEV appears to have a better adverse effect profile, VPA seems more likely to be effective in achieving seizure control.

Ketogenic diet therapy map of Turkey.

BACKGROUND: Although the ketogenic diet (KD) is a well-established non-pharmacologic treatment for intractable epilepsy in pediatric patients, it is still perceived as theoretical information contained within textbooks rather than implementation in daily clinical practice. The aim of the present study was to primarily determine KD implementation frequency in daily clinical practice, the number of pediatric patients with intractable epilepsy, the conditions that hindered or facilitated KD implementation, and to provide a roadmap to improve patient outcomes. METHODS: A total of 27 pediatric neurologists, who were experienced in intractable epileptic pediatric patients and the implementation of KDs, responded to a 24-question survey. The survey was structured to outline patient selection criteria for KDs, prevalent treatment approaches in daily clinical practice for intractable epilepsy, level of physician awareness and impediments in KD implementation. RESULTS: Intractable epilepsy was diagnosed predominantly in children within the 7 to 12-year age group (44%). KD implementation was hindered mainly by lack of an adequate number of personnel (53.8%), lack of a dietitian (52%), inadequate training of patients (24%), and inadequate experience of healthcare professionals (23.1%). Lack of guidance in treatment, physician`s hesitations due to probable problems, inadequate time spent for each patient, lack of awareness for KD therapy, and loss of appetite in these patients were also emphasized by the participants (each 16.7%). Additional drawbacks were non-appealing taste (76.9%), need for continuous supervision (76.9%), and low patient motivation (73.1%). The treatment failure causes for KDs were ranked as imprecise cooking of recipes (94%), inadequate family support (92.3%), inadequate consumption of meals (73%), incorrect indication (53.9%), and inefficiency of KD despite correct application (42.3%). CONCLUSION: The panoramic view of KDs in Turkey indicates that a National Guideline would increase both physician awareness level for KD, and the rate of structured therapy implementation in pediatric patients, who suffer from inadequate treatment.

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.

Magnetic resonance imaging patterns of subacute sclerosing panencephalitis may mimic metabolic disorders: clinical, electroencephalographic and imaging features of six cases.

Subacute sclerosing panencephalitis is a rare, devastating neurodegenerative encephalitis whose diagnosis and therapy are still in question. Atypical clinical presentation and heterogeneity of neuroimaging findings that have been initially confused with metabolic disorders have hampered early diagnosis. To describe a series of patients with subacute sclerosing panencephalitis with imaging findings mimicking metabolic disorders. A total of six patients with subacute sclerosing panencephalitis were diagnosed from January 2012 to December 2016 in whom a metabolic disorder was suspected on initial clinical and MRI findings. Detailed laboratory investigation was performed in all patients. All patients presented with atypical neurologic manifestations, including dystonia, syncopal attacks, involuntary limb movements, meaningless speech and ataxia. Magnetic resonance imaging abnormalities included bilateral putaminal, bilateral posterior periventricular white matter and diffuse or splenial corpus callosum involvement which are particularly unusual in SSPE and mostly observed in metabolic disorders. All patients had elevated cerebrospinal fluid Ig G measles antibodies. The diagnosis of subacute sclerosing panencephalitis through clinical and imaging features can be considerably challenging. It is crucial to differentiate it from metabolic disorders, since the management and clinical outcome are different.

MRI in CLN2 disease patients: Subtle features that support an early diagnosis.

Neuronal ceroid lipofuscinosis type 2 (CLN2) disease is a rare, paediatric-onset, neurodegenerative disorder characterised in its early stages by language delay, seizures and loss of motor function. It is rapidly progressive and ultimately results in the premature death of patients. We aim to highlight common magnetic resonance imaging (MRI) features seen in early CLN2 disease and increase disease awareness among clinicians in order to facilitate early diagnosis and treatment of patients with disease-modifying enzyme replacement therapy. We obtained MRI scans from 12 Turkish children with CLN2 disease, at symptom onset or time of diagnosis, and at various times during disease progression. Patient details including age at onset of symptoms, age at diagnosis and clinical presentation were collected. MRIs were analysed to identify common features present in patients with CLN2 disease. The median diagnostic delay in this cohort was 2 years, highlighting the need for increased disease awareness among clinicians. Key MRI features suggestive of CLN2 disease that were identified included cerebellar atrophy in 11 patients, linear hyperintensity of central white matter in 10 patients, cerebral atrophy in 8 patients and thinning of the corpus callosum in 6 patients. Thalamic hypointensity was seen in 1 patient and may also indicate CLN2 disease. It is important to consider the presenting symptoms alongside clinical test results in order to support early diagnosis of CLN2 disease. Clinical suspicion of CLN2 disease accompanied by the detection of any of the above-mentioned features on MRI should encourage healthcare professionals to test for CLN2 disease.

Evaluation of state and trait anxiety levels of parents and children before electroencephalography procedures: A prospective study from a tertiary epilepsy center.

BACKGROUND: Inadequate or misinformation about electroencephalography (EEG) and epilepsy may lead to anxiety in children and their parents. The purpose of this study was to make a simultaneous evaluation of the anxiety levels of children and parents before EEG procedures and to make a brief assessment of their knowledge about EEG. METHODS AND MATERIALS: Children aged between 8 and 18 years who were referred for EEG tests at Department of Pediatric Neurology, Gazi University Faculty of Medicine, Ankara, Turkey and their parents were included in the study, prospectively. Data were collected through Personal Information Forms; an EEG questionnaire form, which questioned the knowledge of the participants about EEG; the Spielberger's State-Trait Anxiety Inventory (STAI) to determine anxiety levels of the parents; and the State-Trait Anxiety Inventory for Children-State form (STAIC) to determine the anxiety levels of the children. The following parameters were collected in a database: demographic data about children and parents (sex, age), indication of suspected diagnosis on EEG request (i.e., the referral diagnosis), history of epilepsy, number of EEG recordings, and results of previous EEG recordings. The state and trait anxiety test results of the children were compared between the girls and boys, between age groups, and their parents' results in terms of both trait and state anxiety in terms of EEG, sex, ages, educational levels, and working. RESULTS: Eighty-five children (mean age: 13.25 ±â€¯3.02 years) and 85 parents (mean age: 41.16 ±â€¯7.65 years) were included in the study. The children's mean trait anxiety score was 32.51 ±â€¯8.09, and the mean state anxiety score was 34.97 ±â€¯7.62. Half of the children who had a trait anxiety score of ≤30 points had increased state anxiety levels because they received more than 30 points in the state anxiety evaluation score. No significant differences were found between the boys and girls in terms of the state and trait anxiety scores (p > 0.05). The parents' mean trait anxiety score was 39.16 ±â€¯7.74, and the mean state anxiety score was 42.74 ±â€¯6.22. Forty (47%) parents were found to have trait anxiety, and 52 (61.2%) parents had state anxiety before the EEG. The trait anxiety score of the mothers was statistically significantly higher than that of the fathers (p < 0.01). The investigation of the knowledge level of both parents and children about EEG demonstrated some misunderstandings or points of insufficiency. CONCLUSION: The present study revealed that both parents and children had insufficient knowledge about EEG, and the procedure caused anxiety for both the parents and children. When EEG procedures are requested, parents and children should be given brief information about EEG and epilepsy. We think that in this way, the knowledge of both parents and children about this issue may be increased and their anxiety may be decreased.

Acute necrotizing encephalopathy with organic psychosis: a pediatric case report.

BACKGROUND: Enteroviruses-associated acute necrotizing encephalopathy (ANE) of childhood is rarely reported in the literature. Clinico-radiological features of ANE are well-recognized and bilateral thalamic nuclei are frequently affected by ANE. Neuropsychiatric symptoms are rarely seen. Thalamic damage can cause psychosis. CASE: Herein, we present a pediatric case of enterovirus-associated ANE presenting with psychosis related to thalamus damage in whom a favorable response to treatment was achieved. CONCLUSION: Thalamic damage occurs during the Enteroviruses-associated acute necrotizing encephalopathy and it can be related psychiatric sympthoms. Clinicians should be aware of uncommon presentations of ANE, and patients with thalamic damage should be followed for neuropsychiatric manifestations. Early recognition and appropriate treatment of ANE is important to obtain favorable outcomes.

Benign epilepsy with centrotemporal spikes: Is there a thalamocortical network dysfunction present?

INTRODUCTION: Benign epilepsy of childhood with centrotemporal spikes (BECTS) is one of the most frequently seen epileptic syndromes in childhood. It is characterized by centrotemporal spikes (CTS) on electroencephalography (EEG) that are typically activated by drowsiness and stage N2 sleep. The location, frequency, and amplitude of the spikes may vary in different EEG records of the same patient, supporting the presence of a global pathology rather than a focal one. Despite the well-known relation between BECTS and stage N2 sleep, the results of sleep studies have been diverse and have mainly focused on sleep cycles. The characteristics of sleep spindles in the interictal periods have not been studied well. METHODS: A retrospective study involving patients with BECTS who were admitted to the Gazi University, Faculty of Medicine, Department of Pediatric Neurology from January 2017 to October 2018 was conducted herein. Patients with BECTS and age-matched controls who had stage N2 sleep records of 10 min were enrolled for spindle amplitude (peak-to-peak difference in spindle voltage), frequency (number of waveforms per second), and duration and density (number of spindle bursts/minute of stage N2 sleep). RESULTS: A total of 30 children with BECTS and 20 age-matched healthy peers were enrolled in the study. There were no significant differences between the age and sex of the patients. Statistically significant lower mean values of the amplitude, and duration and density of the spindle activity were observed in patients with BECTS when compared to the controls (P: 0.034, P: 0.016, and 0.020, respectively). Additionally, the risk of epilepsy was found to increase by 1.9 %, by the decrease of the mean amplitude of the spindles by 1 mV when compared to control group. CONCLUSION: The interictal records of stage N2 sleep differed in the patients with BECTS when compared to the controls. Findings related to the stage N2 sleep of the present study may suggest a network problem involving the thalamus and thalamocortical pathways in patients with BECTS.

Utility of brain fluorodeoxyglucose PET in children with possible autoimmune encephalitis.

PURPOSE: We aimed to explore the utility and additional clinical contribution of brain fluorodeoxyglucose (FDG) PET imaging for the assessment of children with possible autoimmune encephalitis in comparison to brain MRI. MATERIALS AND METHODS: We conducted a retrospective analysis of six pediatric patients (all seronegative) between 2014 and 2019 with the initial diagnosis of possible autoimmune encephalitis who had brain FDG PET/CT or PET/MRI and brain MRI during the diagnostic period. Diagnosis of possible autoimmune encephalitis was based on clinical consensus criteria defined by Graus et al. Brain FDG PET images were visually evaluated. Semiquantitative evaluation was also performed by using the statistical parametric mapping (SPM) method. RESULTS: Cerebrospinal fluid pleiocytosis and electroencephalography abnormality were present in all patients. Mean duration between the onset of symptoms and brain FDG PET imaging was 33 ± 16 days (range: 18-62 days). There were a total of eight brain FDG PET scans (six of PET/MRI and two of PET/CT). In two patients, FDG PET imaging was performed at diagnosis and follow-up. Initial FDG PET and SPM analysis findings were abnormal in all patients (100%), with four demonstrating only hypometabolism. Only a hypermetabolic pattern was seen in one patient, and mixed the hypohypermetabolic pattern was seen in one patient. All patients had metabolic abnormalities in temporal lobes. Additionally, visual and semiquantitative FDG PET findings revealed hypometabolism in extratemporal regions. Brain MRI was abnormal in two patients (33.3%) who had also FDG hypermetabolism in mesial temporal lobes. CONCLUSIONS: Our findings support the usage of fluorine-18-FDG PET/computed tomography (CT)/MRI with quantitative analysis early in the diagnostic work-up of possible autoimmune encephalitis, particularly in those with normal or nonspecific MRI findings. However, it remains a purpose of further studies, if and to what extent FDG PET/CT or integrated FDG PET/MRI with quantitative analysis can improve the diagnostic workup of children with possible autoimmune encephalitis.

Optic nerve ultrasonography in monitoring treatment efficacy in pediatric idiopathic intracranial hypertension.

PURPOSE: Measurement of optic nerve sheath diameter (ONSD) with ocular ultrasonography (USG) is a noninvasive technique that can be readily used to determine clues of increased intracranial pressure. In this study, we aimed to determine the role of optic nerve sheath diameter measurements in the diagnosis and follow-up of pediatric patients with idiopathic intracranial hypertension (IIH). METHODS: Eight patients with a diagnosis of IIH with a median age of 11.7 (range 4.5-17) years were examined prospectively. During follow-up, orbital ultrasonography (USG) was performed immediately prior to lumbar puncture (LP) and at 24 h, at 1 week, and between 1 and 18 months after LP. Cranial MRI examinations and automated visual field assessments were performed at baseline and at 3 months, and both measurements were compared with each other. RESULTS: The mean cerebrospinal fluid opening pressure (37.75 ± 12.64 cm H2O) and the mean ONSD (5.94 ± 0.46 mm) were correlated. The median follow-up was 16 (range, 12-18 months), and ONSD regressed gradually consistent with clinical and radiologic improvement during follow-up. CONCLUSIONS: To the best of our knowledge, this is the first prospective pilot study performed on pediatric patients with IIH using orbital USG for ONSD measurements. Despite the small sample size, the present study indicated that orbital USG may be used as a promising noninvasive tool to diagnose increased intracranial pressure and for monitoring treatment efficacy in this special patient population.

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.

Effects of antiepileptic drugs on dynamic thiol/disulphide homeostasis in children with idiopathic epilepsy.

PURPOSE: Anti-epileptic drugs have been widely used in children with epilepsy. Although several studies have investigated the role of oxidative stress and the effects of antiepileptic drugs on several oxidative markers in epilepsy, adequate information is not available on this issue. This study aimed to investigate the changes in thiol disulphide homeostasis in children with epilepsy under two commonly prescribed AED monotherapies, carbamazepine and valproic acid. METHODS: A hundred and one children with epilepsy using valproic acid or carbamazepine and 58 healthy children were included in this study. Of the 101 patients with idiopathic epilepsy, 58 were on valproic acid monotherapy and 43 patients were on carbamazepine monotherapy. The total thiol, native thiol, and disulphide levels were measured and the disulphide/native thiol, disulphide/total thiol and native thiol/total thiol ratios were calculated in both groups. RESULTS: The total thiol and native thiol levels of the valproic acid treated group were significantly lower than the control group (p < 0.05). The native thiol level of carbamazepine treated group was lower than the control group without a significance (p = 0.123). Disulphide level, disulphide/native thiol and disulphide/total thiol ratios were significantly higher and native thiol/total thiol ratio was significantly lower in both valproic acid and carbamazepine treated group compared with the control group. CONCLUSION: Thiol/disulphide homeostasis is impaired in children with idiopathic epilepsy using valproic acid or carbamazepine. Valproic acid which is frequently used in childhood epilepsy may modify this balance more than carbamazepine monotherapy. More importantly, the new method used in our study proposes a promising, practical and daily applicable test for evaluating oxidative stress in these patien.