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Aim: to discuss seizure outcomes of patients with invasive electroencephalography (EEG) monitorization (IEM) following their epilepsy surgery at our centre. Material and Methods: forty-seven patients suffering from refractory epilepsy and who were evaluated by invasive EEG were included in this retrospective study at Istanbul Faculty of Medicine from 2003 to 2017. We examined the Video EEG and invasive EEG monitorization, cranial MRI, SPECT, PET and neuropsychological tests of all patients. Postoperative seizure outcome results were evaluated according to Engel classification. The factors affecting seizure outcomes were discussed. Results: twenty-six of the patients were female (55.3%), 21 were male (44.7). The average age was 32.0 (+/- 12.4). Forty-three patients had surgery and the average age of these patients was 26,6 (+/- 11.15). 38.3% of the patients had hippocampal sclerosis (HS), 23.4% had focal cortical dysplasia (FCD), 8.5% had a tumor, 14.9% had sequela lesion and 14.9% had unknown etiology. Postoperative seizure status according to the Engel classification showed that 81.6% of the patients were class I, 10.5% were class II, 2.6% were class III and 5.3% were class IV. Conclusion: a significant relation was statistically determined between structural MRI lesion and favorable seizure outcome (p0.05). We argue that IEM is an essential examination for favorable outcomes for determining the epileptogenic zone and/or the proximity of the functional structures., NA

PURPOSE Electroencephalography (EEG) findings related to the eye-closing motion can be defined in two ways: 'sensitivity to eyes closed' (SEC) and 'eye closure sensitivity (ECS).' Fixation-off sensitivity (FOS) is a different phenomenon induced by the elimination of central vision/fixation. The purpose of our study was to determine the frequencies of SEC, ESC, and FOS, and to analyze the relationship between eyes closure and the fixation-off phenomenon and clinical importance in an unselected population. METHODS We prospectively evaluated 200 routine interictal EEGs by adding a standardized FOS examination protocol between June and September 2015. Goggles covered with semitransparent tape were used to evaluate FOS. We determined SEC when the epileptiform discharges appeared during eye closure and continued during the eye closed state, whereas ECS was defined as transient epileptic abnormalities following the closure of the eyes lasting for 1-4 sec. The patients were evaluated in terms of demographic characteristics, clinical features, and the relationship between SEC, ECS, and FOS. RESULTS We detected SEC in 9 (4.4 %) and ECS in 11 (5.4 %) patients. FOS was detected in four (44.4 %) of the patients who showed SEC, all of whom had occipital epileptiform discharges. A statistically significant correlation was found between FOS and treatment resistance in the SEC group (p < 0.001). In logistic regression analysis, occipital lobe epilepsy (p < 0.001) and age under 20 years (p = 0.004) were found as risk factors for SEC. Another interesting finding was the suppression of epileptic discharges with fixation-off in three of 11 patients with ECS. CONCLUSIONS According to the results of our study, FOS is related to treatment resistance. Therefore, FOS should be evaluated in patients with SEC.

Objective Phenylketonuria (PKU) is the most common form of amino acid metabolism disorders with autosomal recessive inheritance. The brain damage can be prevented by early diagnosis and a phenylalanine-restricted diet. Untreated or late-treated patients may show mental retardation and other cognitive dysfunctions, as well as motor disability and/or epilepsy. Methods Three patients with PKU and epilepsy were recognized to have reflex epileptic features, and there were ten consecutive adult patients with PKU and epilepsy who were evaluated retrospectively. Medical history, ages at diagnosis and therapy onset, age at seizure onset, seizure types and reflex features, neurological findings, cranial imaging, electroencephalography (EEG) findings, and final clinical condition were evaluated. Reflex epilepsy features were examined in detail. Results The cases (6 females, 4 males) were diagnosed at ages between 3.5 months and 12 years. All patients had various degrees of mental–motor retardation and focal or generalized seizures with age at seizure onset varied between neonatal period and 15 years. Three patients had febrile seizure, 3 patients had myoclonia, and 3 patients had status epilepticus. All patients had abnormal EEG findings except one. There was a slowing of background activity, and generalized discharges were observed in 7 patients; 3 of them had asymmetrical discharges. One patient had right hippocampal sclerosis (HS), and another patient had hypointensities in the basal ganglia and corpus callosum. Reflex features were clinically observed in 3 of the patients; however, EEG results did not show any related findings. One patient had reflex seizures triggered by photic stimuli, hot water, and startling; one by photic stimuli; and the other one by startling. Conclusion Reports on the clinical and electrophysiological features of adult patients with PKU were scant. We emphasized that reflex clinical features may be observed in this metabolic disease, and focal epileptiform abnormalities and asymmetry may be present in electrophysiological evaluation besides the rare association with HS.

Objective The objective of this study was to determine the predictors of outcomes and refractoriness in status epilepticus (SE). Methods This is a prospective study of 59 adult patients with SE who were admitted to the Emergency Department between February 2012 and December 2013. The effects of clinical, demographic, and electrophysiologic features of patients with SE were evaluated. To evaluate outcome in SE, STESS, mSTESS, and EMSE scales were used. Results Logistic regression analysis showed that being aged ≥ 65 years (p = 0.02, OR: 17.68, 95% CI: [1.6–198.4]) for the short term and having potentially fatal etiology (p = 0.027, OR: 11.7, 95% CI: [1.3–103]) for the long term were the only independent predictors of poor outcomes; whereas, the presence of periodic epileptiform discharges (PEDs) in EEG was the only independent predictor of refractoriness (p = 0.032, OR: 13.7, 95% CI: [1.3–148.5]). The patients with ≥ 3 Status Epilepticus Severity Score (STESS) did not have poorer outcomes in the short- (p = 0.157) and long term (p = 0.065). There was no difference between patients with 0–2, 3–4, and ≥ 4 mSTESS in the short- and long term in terms of outcome (p = 0.28 and 0.063, respectively). Also, there was no difference between subgroups (convulsive SE [CSE], nonconvulsive SE [NCSE], and epilepsia partialis continua [EPC]) in terms of STESS and mSTESS. When patients with EPC were excluded, both STESS and mSTESS scores of the patients correlated with poorer long-term outcomes (p = 0.025 and 0.017, respectively). The patients with ≥ 64 points in the Epidemiology-based Mortality in SE-Etiology, age, comorbidity, EEG (EMSE-EACE) score and those with ≥ 27 points in EMSE-Etiology, age, comorbidity (EMSE-EAC) score did not have poorer outcomes in the short term (p = 0.06 and 0.274, respectively) while they had significantly poorer outcome in the long term (p Conclusion Being aged ≥ 65 years predicts poor short-term outcomes, and having potentially fatal etiology predicts poor long-term outcomes, which highlight the importance of SE treatment management in the elderly. Both STESS and mSTESS are not predictive for poor outcomes in EPC. Excluding patients with EPC, STESS, and mSTESS could predict poor long-term outcomes but not in the short term in SE. Epidemiology-based Mortality in Status Epilepticus score could predict poor outcome in the long term better than STESS and mSTESS. Specifically, EMSE scores correlated with poor outcome in patients with CSE but not with NCSE and EPC. New scales are needed to predict outcome especially in patients with NCSE and EPC. The presence of PEDs in EEG is a predictor of RSE, and EMSE score can also be used to predict RSE. There was no difference in the efficacy of IV LEV and IV PHT in SE. This study is significant for having one of the longest follow-up periods in the literature.

Background Sleep disorders and disturbances are generally underestimated in patients with epilepsy. The aim of this study is to determine the frequency of sleep disturbances and the comorbidity of sleep disorders in people with epilepsy without any complaints about sleep and their relation of sudden unexplained death in epilepsy (SUDEP) risk. Methods Sleep complaints and the presence of sleep disorders were assessed with 4 questionnaires in 139 patients with epilepsy. Subjective sleep features were evaluated with Pittsburgh Sleep Quality Index (PSQI), the Epworth Sleepiness Scale (ESS), the Berlin Questionnaire for sleep apnea, and restless legs syndrome with International Restless Legs Syndrome Study Group (IRLSSG) severity scale. The presence of rapid eye movement/nonrapid eye movement parasomnia was asked to the patients and their relatives who share the same house. The patients' SUDEP-7 scores were also determined and associations with sleep problems were investigated statistically. Results Ninety-two patients with focal and 47 patients with generalized epilepsy were evaluated after their consent. The daily sleep quality was poor in 34 (24.5%) patients with PSQI. Daily sleepiness was present in 7 (5%) patients with ESS. Twenty-five patients (18%) had severe sleep apnea risk with the Berlin Questionnaire. Mild or severe RLS was detected in 24 patients (17.2%). There were no significant differences between focal or generalized epilepsy groups' scores. No statistically significant relationship was identified between SUDEP-7 scores and sleep quality or sleep-related disorders. Conclusion Our results emphasized a remarkable magnitude of the comorbidity of sleep disorders in patients with epilepsy, even for those who do not have complaints about sleep. As SUDEP cases are frequently seen during sleep, it is important to evaluate sleep in patients with epilepsy.

Taskiran, Emine/0000-0002-4402-7606; Matur, Zeliha/0000-0002-3895-0410; WOS:000462821600018 PubMed ID: 31001016 Context: Seizures and accompanying situations including social, medical, and psychiatric problems threaten the quality of life (QOL) in patients with epilepsy. The World Health Organization defines health is a state of complete physical, mental, and social well-being, and not merely the absence of disease or infirmity. Aims: This study examines the prevalence of both depression and anxiety symptoms and also impact of the affective state on QOL in patients with focal epilepsy in Turkey. Settings and Design: One hundred and five patients with focal epilepsy over 18 years old were included in this study. The patients were classified into four groups according to the presence of AS and seizure control. Subjects and Methods: Patients' affective symptoms (AS) and QOL were examined using the Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), and QOL in Epilepsy Inventory-31 (QOLIE-31). Statistical Analysis Used: We used descriptive statistics, Chi-square test, independent samples t-test, one-way analysis of variance, Mann-Whitney U-test, Kruskal-Wallis H-test, and also Pearson's and Spearman's correlation test for correlations. Results: There were positive correlations between total QOLIE-31 score and epilepsy surgery, employment, and seizure freedom, whereas negative correlations were found with antiepileptic drug use, anxiety, and depression. Statistically significant differences were found in QOLIE-31 totals and subscores between Groups 3 and 4 (P < 0.05). Conclusions: The presence of AS has a negative impact on QOL in patients with focal epilepsy. Physicians should be aware that psychiatric comorbidities in epilepsy have a severe impact and epilepsy treatment requires comprehensive management.

The aim of the present study was to investigate the effect of a brief seminar focusing on medical and social aspects of epilepsy on information acquisition of and attitudes toward epilepsy among medical school students.The sample of this pretest-posttest study consisted of 57 fifth-grade medical students. The students participated in a one-hour seminar including medical and social aspects of epilepsy. An epilepsy-related awareness form developed by researchers and also the Epilepsy Attitude Scale were applied to the participants before and after the seminar.It was determined that half of the students (50.9%) encountered an epileptic seizure and 12.3% of them applied first aid. The students had difficulty in describing the seizure type before education. Before education, the rate of describing the seizure was 47.4% for myoclonic seizure, 50.9% for simple partial seizure, and 64.9% for absence seizure, and after education, these rates increased to 82.5% (p 0.001), 91.2% (p 0.001), and 98.2% (p 0.001), respectively. Students generally well described the seizure triggering factors; however, the rate of students reporting the menstrual period as triggering factors were lower (66.7%), and the rates increased after the education (93.0%) (p = 0.001). The percentages of correct answers increased also for the questions regarding seizure first aid. The percentage of students who felt competent for seizure first-aid management increased from 12.3% to 91.2% (p 0.001) after the education. The correct response rates of students for social aspects of epilepsy was generally high. In our study, attitude toward epilepsy was also evaluated. After the education, a mild increase in the attitude score of students was found (p = 0.009). Although it is minimal, the number of students who marked more positive attitude increased for each item of the Attitude scale.Although a lack of acquaintance was found in some areas, awareness of epilepsy in our sample was at a moderate level. This study showed a positive effect of the education given to students on information acquisition and attitude.

Status epilepticus (SE) is rarely described in patients with juvenile myoclonic epilepsy (JME), and little is known about its frequency, subtypes, and predictors and the prognosis of these patients. In this retrospective study, we aimed to analyze the incidence of SE in patients with JME and emphasize the risk factors and long-term outcome of SE in an epilepsy outpatient-based cohort. We included patients with JME with a history of predominant myoclonic seizures and at least one diagnostic EEG with normal background activity and bursts of typical ≥3-Hz generalized spike-polyspike and waves. We investigated the electroclinical features of patients with JME who had experienced SE and compared them with patients with JME without SE. Of the 133 patients with JME, only 5 patients were diagnosed with SE (3.8%) according to new criteria. The most common SE subtype was myoclonic SE (MSE, 4 patients), followed by generalized clonic-tonic-clonic SE (1 patient) and nonconvulsive SE (1 patient). One patient had both MSE and generalized clonic-tonic-clonic SE. In three out of five patients, recurrent episodes of SE were observed. Same seizure precipitants including sleep deprivation, inappropriate antiepileptic drug treatment, and noncompliance were identified in patients with JME with and without SE, not reaching a significant difference between the groups. Myoclonia limited to specific body parts (one arm, face, or head) were significantly more common in patients with JME with SE (p: 0.002). We did not find any significant correlation with drug-resistant course and SE. Status epilepticus is rarely observed in patients with JME, and MSE appears to be the most common subtype. Local myoclonia might predict SE in a subgroup of patients with JME. We may suggest that some patients with JME have a liability to SE, in addition to usual seizure precipitating factors of JME. It seems that SE per se does not affect the outcome of JME and the patients with SE did not have drug-resistant course in the final analysis.

Objectives Transcranial direct current stimulation (tDCS) is a non-invasive and safe method tried in drug-resistant epilepsies, in recent years. Our aim was to evaluate the effect of tDCS in patients diagnosed with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) which is a well-known drug-resistant focal epilepsy syndrome. Patients and methods Twelve MTLE-HS patients diagnosed with their typical clinical, EEG and MRI findings fulfilling the criteria for drug-resistance as suggested by the ILAE commission were included after Ethics Committee approval and their signed consent. All patients received modulated cathodal stimulation; 2 mA for 30 min on 3 consecutive days. All patients also received sham stimulation with the same electrode positions; designed as 60 s stimulation gradually decreasing in 15 s with placement of the electrodes for 30 min over the stimulation side. They were followed up by standard seizure diaries and their medical treatment was not changed during the study period. Their seizure frequencies both before and after cathodal tDCS and sham stimulation were compared statistically. Adverse effects were also questioned. Results Mean age of our study group was 35.42 ± 6.96 (6 males; median: 35.50). The mean seizure frequency was 10.58 ± 9.91 (median = 8; min-max = 2-30) at the baseline and significantly decreased to 1.67 ± 2.50 (median = 0.5; min-max = 0-8) after cathodal tDCS application (p = 0.003). Ten patients (83.33%) had more than 50% decrease in their seizure frequencies after cathodal tDCS. Two patients (16.67%) also showed positive sham effect. Six patients (50%) were seizure-free in the post-cathodal tDCS period of one month. No adverse effect has been reported except tingling sensation during cathodal stimulation. Conclusion Our small series suggested that cathodal tDCS may be used as an additional treatment option in MTLE-HS. It may be tried in TLE-HS patients waiting for or rejecting epilepsy surgery or even with ineffective surgery results. More studies are needed with large series of patients to investigate the effects of tDCS in drug resistant epilepsies.

Sialidosis are autosomal recessive inherited disorders caused by a mutation on the NEU1 gene. In type 1 sialidosis, a “cherry-red spot” can be observed in fundoscopic examinations. In this study, a woman aged 37 years without “cherry-red spot” on ophthalmologic examination is reported to draw attention to a new phenotypic variation. Although an ophthalmologic examination was normal, for patients with consanguineous parents with progressive ataxia, drug-resistant epilepsy and myoclonus must be investigated for progressive myoclonic epilepsy and genetic analysis for sialidosis must be performed. The diagnosis is also crucial for genetic consultancy of the family.

INTRODUCTION: Hippocampal sclerosis (HS) is usually associated with typical anterior temporal spikes/sharp waves in the electroencephalography (EEG). We aimed to investigate the clinical, electrophysiologic differences, and prognosis between HS patients with unusual epileptic foci in comparison with patients with typical foci. METHODS: Patients diagnosed who were diagnosed as having mesial temporal lobe epilepsy with HS in our center were included. All magnetic resonance imaging (MRI) results were investigated for the presence of two major criteria of HS diagnosis, namely T1 atrophy and T2 hyperintensity. All EEG and video-EEG investigations (a total of 496) were re-evaluated for unusual epileptic activities except T1/2, F7/8, T3/4 foci. Clinical and other laboratory data were retrospectively reevaluated by two investigators and the groups with usual and unusual epileptic activities for this syndrome were statistically compared. RESULTS: A total of 124 patients including 20 (16.1%) with unusual epileptic activities (group 1) and 104 patients with typical foci (group 2) were evaluated. We observed 11 posterior temporal (T5/T6), four frontal (F3/F4), four frontopolar (Fp1/2), two occipital (O1/O2), three central (CP/FC) unusual foci, and two generalized epileptiform discharges in these 20 patients. Impaired background activity, nonspecific interictal diffuse slowing, and atypical seizure offset patterns were more frequently observed in group 1. Forty-nine patients underwent surgery after appropriate preoperative diagnostics; there was no significant difference in the postoperative outcome between the groups despite a tendency to better prognosis in group 2. DISCUSSION AND CONCLUSION: Our study showed that unusual epileptic foci could be seen in 16.1% of patients with HS. Although some patients with HS have these atypical interictal EEG findings, they might obtain good post-operative outcomes when preoperative diagnostics are appropriately planned.

Objective The objective of the study was to investigate the effects of slow repetitive transcranial magnetic stimulation (rTMS) on patients with refractory juvenile myoclonic epilepsy (JME). Methods One thousand pulses with the intensity of 120% active motor threshold (AMT) at 0.2 Hz frequency were applied on 5 consecutive days in 10 patients with refractory JME. Sham rTMS was performed after 3 months. Electroencephalography (EEG) examinations were performed before rTMS, on the 5th day, and 1, 2, 4, and 8 weeks after rTMS. Resting motor threshold (RMT), AMT, and cortical silent periods (CSPs) were recorded before the application and at the end of day 5. The changes in the quality of life were evaluated using the Quality of Life in Epilepsy Inventory (QOLIE-31). Results No adverse effects were observed. The number of seizures decreased by 29–50%, and interictal discharge durations decreased 2 weeks after the real rTMS. No significant difference was observed between the AMT and RMT values recorded before and after the stimulations. Statistically significant increases in CSP duration and quality of life scores were found following real rTMS. Repetitive transcranial magnetic stimulation may be considered as a safe treatment option in refractory JME. Conclusion This study provides some positive evidence that rTMS may be effective in resistant JME.

Objective The objective of this study was to investigate unusual ictal propagation patterns in patients with drug-resistant temporal lobe epilepsy (TLE) and reveal their electrophysiological, neuroimaging, and prognostic properties after surgery. Methods Among 248 patients with TLE who underwent scalp video-electroencephalographic (EEG) monitoring, 24 patients with ‘switch of lateralization’ or ‘bilateral asynchrony’ in at least one of their seizures (9.3%) were analyzed retrospectively. The postoperative outcome was determined in 16 patients who had undergone epilepsy surgery. Results All but 5 of the included patients had hippocampal sclerosis (HS) as their magnetic resonance imaging (MRI) findings. Twelve out of 16 patients (75%) who had surgery were seizure-free for at least 1 year. Nine out of 12 patients (75%) with good outcome had unilateral interictal EEG discharges in temporal regions whereas 3 out of 4 patients with poor outcome had bilateral temporal interictal spiking (p = 0.018). Conclusion Unusual ictal propagation patterns are not always related to poor prognosis after surgery in patients with TLE. Patients with unilateral interictal spiking in the temporal region tend to have good outcome despite these unusual patterns. These patterns can also be seen in patients with TLE with other etiologies besides the well-known HS in MRI.

Purpose To draw attention to epileptic pain which is a rare seizure symptom mostly causing wrong diagnosis and delayed treatment. We present nine patients in whom pain was a prominent initial or early ictal symptom. Methods We reviewed the records of 4736 patients, and found nine patients who had pain as an aura or an early prominent symptom of their seizures. Seizure semiology, EEG, and cranial imaging features were evaluated retrospectively. Results Age at seizure onset ranged from 6 months to 50 years, and the mean age during the study was 37.7 ± 11.9 years. Pain was predominantly peripherally localized in four patients, whereas cephalic pain was detected in three patients, and abdominal pain was detected in two patients. Electroencephalography (EEG) revealed epileptic abnormalities on the temporoparietal and frontotemporal regions in three patients each. Photosensitive generalized epileptic discharges were detected in one and diffuse background slowing in the remaining two other patients. Electroencephalography abnormalities were lateralized to the contralateral site of the pain in four patients with strictly localized pain. Three patients revealed no abnormality on the cranial MR imaging, whereas others showed different types of abnormality such as heterotopias (n:1), mesial temporal lobe atrophy (n:1), white and gray matter sequela lesions (n:1), diffuse white matter lesion (n:1), chronic encephalomalacia and gliosis (n:1), and perivascular space dilatation (n:1). Conclusion Epileptic pain is a neglected, but important, semiologic symptom with localization and lateralization value in the patients with somatosensorial seizures of parietal as well as temporal lobe origin. It occurs mainly as peripherally localized, cephalic, or abdominal pain and is usually associated with a symptomatic etiology. Awareness of epileptic pain is important to avoid misdiagnosis and delayed treatment.

Background Studies on electrophysiological characteristics of patients with epilepsy and concomitant psychosis are limited. We aimed to investigate the clinical and video-electroencephalography (EEG) findings of patients with epilepsy-related psychosis (EP). Materials and methods Fifteen patients diagnosed with EP, assessed at the video-EEG monitoring unit and were under follow-up at both epilepsy and psychiatry clinics, were included. A total of 67 nonpsychotic epilepsy patients, investigated at the video-EEG monitoring unit were randomly selected as the control group and compared statistically with the EP group. Results In medical history, patients with EP had experienced significantly higher level of status epilepticus (P=0.002) and perinatal cerebral injury (P=0.04), whereas drug-resistant epilepsy was detected at a lower level (P=0.015). With respect to seizure onset zone, the EP group had significantly more seizures of unknown foci, whereas the control group had mostly temporal lobe origin (P=0.0004). EEG findings showed that slow background activity was significantly common among patients with EP (P=0.009). Although only 5 of 15 patients with EP had been operated, 43 of 67 patients had undergone epilepsy surgery (P=0.04) in the control group. However, there was no significant difference between the 2 groups with respect to postoperative seizure control as per Engel classification. Conclusions Although our sample size could be considered small, slowed EEG background activity, and the marked frequency of initial precipitant factors such as status epilepticus, perinatal cerebral injury, and detected neuronal autoantibodies suggested that EP is associated with more extensive involvement. EP is not a contraindication for epilepsy surgery, when appropriately investigated preoperatively.

Objectives Genital automatisms (GAs) are uncommon clinical phenomena of focal seizures. They are defined as repeated fondling, grabbing, or scratching of the genitals. The aim of this study was to determine the lateralizing and localizing value and associated clinical characteristics of GAs. Methods Three hundred thirteen consecutive patients with drug-resistant seizures who were referred to our tertiary center for presurgical evaluation between 2009 and 2016 were investigated. The incidence of specific kinds of behavior, clinical semiology, associated symptoms/signs with corresponding ictal electroencephalography (EEG) findings, and their potential role in seizure localization and lateralization were evaluated. Results Fifteen (4.8%) of 313 patients had GAs. Genital automatisms were identified in 19 (16.4%) of a total 116 seizures. Genital automatisms were observed to occur more often in men than in women (M/F: 10/5). Nine of fifteen patients (60%) had temporal lobe epilepsy (right/left: 4/5) and three (20%) had frontal lobe epilepsy (right/left: 1/2), whereas the remaining two patients could not be classified. One patient was diagnosed as having Rasmussen encephalitis. Genital automatisms were ipsilateral to epileptic focus in 12 patients and contralateral in only one patient according to ictal–interictal EEG and neuroimaging findings. Epileptic focus could not be lateralized in the last 2 patients. Genital automatisms were associated with unilateral hand automatisms such as postictal nose wiping or manual automatisms in 13 (86.7%) of 15 and contralateral dystonia was seen in 6 patients. All patients had amnesia of the performance of GAs. Conclusion Genital automatisms are more frequent in seizures originating from the temporal lobe, and they can also be seen in frontal lobe seizures. Genital automatisms seem to have a high lateralizing value to the ipsilateral hemisphere and are mostly concordant with other unilateral hand automatisms. Men exhibit GAs more often than women.

Purpose Generalized paroxysmal fast activity (GPFA), an EEG pattern with variable frequency and duration, is usually noted in symptomatic/cryptogenic generalized epilepsies. However, GPFA has also been reported in a few patients with genetic generalized epilepsy (GGE) who presented with typical absence seizures (TAS). Our aim was to report the results of long-term follow-up and genetic findings in these patients. Methods We investigated all EEGs of adult GGE patients with TAS, and identified 12 patients with GPFA (8.3% of adult GGE patients with TAS). Ten of these patients were available for long-term follow up. Their clinical and electroencephalographic courses and genetic features were investigated. The control group was composed of 24 adult GGE patients who also had TAS, but lacking GPFA with a similar follow-up duration in the same epilepsy center. Results The mean age at GPFA detection was 33±16.6 (16–71 years) and 80% still had GPFA in their last EEG. The duration of epilepsy and persistence of TAS were both significantly longer in the GPFA group despite a similar follow-up duration. Sixty percent of the GPFA group had consanguineous parents, whereas this rate was only 4.17% in the control group. Seven relatives of the GPFA group also had epilepsy. We could not show any known mutations in two families. At the end of the follow-up, none of the patients with GPFA was dependent in self-care, despite continuing seizures. Conclusion Our study shows that GPFA is an ignored EEG pattern of adult GGE patients with TAS, indicating a long and non-remitting course in almost all of the patients.

Background Subacute sclerosing panencephalitis (SSPE) is a late complication of measles infection. Immune dysfunction related to genetic susceptibility has been considered in disease pathogenesis. A functional single nucleotide polymorphism (SNP) of granzyme B gene ( GZMB ) reported in several pathologies may also be involved in susceptibility to SSPE. Patients and Methods An SNP (rs8192917, G → A, R→Q) was screened in 118 SSPE patients and 221 healthy controls (HC) by polymerase chain reaction-restriction fragment length polymorphism. Frequencies were compared between groups. In vitro production of GZMB was measured in controls with different genotypes. Results The SNP had a minor allele (G) frequency of 0.22 in patients and 0.31 in controls. GG genotype was significantly less frequent in patients (odds ratio, 0.23). G allele carriers produced relatively higher levels of GZMB , when stimulated in vitro. Conclusion These findings implicate possible effect of this genetic polymorphism in susceptibility to SSPE which needs to be confirmed in bigger populations.

Introduction Mitochondria have an essential role in neuronal excitability and neuronal survival. In addition to energy production, mitochondria also play a crucial role in the maintenance of intracellular calcium homeostasis, generation of reactive oxygen species and mechanisms of cell death. There is a relative paucity of data about the role of mitochondria in epilepsy. Mitochondrial genome analysis is rarely carried out in the investigation of some diseases. In mesial temporal lobe epilepsies (MTLE) cases, genome analysis has never been used previously. The aim of this study is to show mitochondrial dysfunctions using genome analysis in patients with MTLE-hippocampal sclerosis (HS). Methods 44 patients with MTLE-HS and 86 matched healthy unrelated controls were included in this study. The patients were divided into four groups according to their clinical presentation as the following: Group 1 consists of patients with intractable epilepsy who refused operation; Group 2 of operated seizure free patients; Group 3 of operated patients with seizures; and Group 4 unoperated seizure free patients with or without antiepileptic drugs. Blood samples were used to isolate DNA. Parallel tagged sequencing was employed to allow pyrosequencing of 130 samples. Complete mtDNA is amplified in two overlapping fragments (11 and 9 kb). The PCR amplicons were pooled in equimolar ratios. Titanium kits were used to produce shotgun libraries according to the manufacturer's protocol. Results The average coverage in total was 130 ± 30 and an average of 2365127 bases and 337 bp fragment length was received from all samples. The mean mtDNA heteroplasmy in patients was 26.35 ± 12.3 and in controls 25.03 ± 9.34. Three mutations had prominently high significance in patient samples. The most significantly associated variation was located in the MT-ATP-8 gene (8502 A > T, Asn46Ile) whereas the other two were in the MT-ND4 (11994 C > T, Thr412Ile) and MT-ND5 (13231 A > C, Lys299Gln) genes. Conclusions We have observed that three mutations were significantly related to the presence of epilepsy. These mutations were found at the 8502, 11994, and 13231 bp of mtDNA, which resulted in amino acid changes at the MT-ATP-8, MT-ND4 and MT-ND5 genes. Finding mutations can lead us to knowing more about the pathophysiology of the MTLE disease.

The purpose was to document 4 patients with different epilepsy syndromes, showing electrical status epilepticus during sleep (ESES), without marked cognitive and behavioral regression in the long-term follow-up. The mean age at onset of seizures was 8 years. Absences, myoclonic, focal motor, or generalized tonic - clonic seizures and drop attacks were the prominent seizure types. The neurological examination and neuroimaging findings revealed no abnormality. Focal epileptiform electroencephalographic (EEG) activity was seen in 3 cases, whereas generalized photosensitive epileptic discharges were detected in 1 patient with juvenile myoclonic epilepsy. Neuropsychological evaluations of all cases were within the normal range, and deterioration in mental status was not observed during their mean follow-up duration of 14 years. Our data support the view that ESES can emerge along with different types of childhood epilepsy syndromes, including idiopathic generalized epilepsies, and may not always be a poor prognostic factor.

Hyperintensity at diffusion weighted magnetic resonance imaging (DWI) and hipointensity at ‘apparent diffusion coefficient’ (ADC) mapping are indicators of cytotoxic edema. These changes are typical for acute ischemic cerebrovascular accidents. During epileptic seizures, transient imaging findigs can be seen in the cranial computed tomography and magnetic resonance imaging. MRI studies during partial status epilepticus in animals show DWI and ADC anomalies which can be related to cytotoxic oedema. Same findings can also be seen in humans. Migraine and transient ischemic attacks can cause transient changes in the MRI which may also accompany epileptic seizures. We discuss here a 44 years old patient who had sensorial complaints and weakness after new onset migraine -like headache accompanied by transient MRI findings.

A B S T R A C T Purpose: To investigate eye closure sensitivity (ECS) in the EEGs of patients diagnosed with juvenile myoclonic epilepsy (JME) and its relationship to prognosis. Methods: We included 76 JME patients with a minimum follow-up of one year and evaluated a total of 254 EEGs to obtain evidence of ECS. The patients were grouped according to their response to treatment, and these subgroups were compared in relation to ECS and other clinical and EEG features. Results: There were 12 patients (15.8%) with poor prognosis who showed resistance to appropriate antiepileptic drug treatment, 15 (19.7%) patients with pseudo-resistance, and 49 (64.5%) patients with good prognosis. The EEGs of only four of the patients displayed pure ECS (5.3%), and only one of these exhibited poor prognosis. Furthermore, 11 patients (14.5%) had both ECS and photosensitivity, and two of these patients exhibited poor prognosis. Thus, neither pure ECS nor ECS with photosensitivity correlated with poor prognosis. A family history of epilepsy and focal findings on the EEG was correlated with poorer prognosis. Conclusions: ECS is a rare EEG finding in JME and does not appear to be a marker for poor prognosis.

Objective: Antiepileptic drugs (AED) have chronic teratogenic effects, the most common of which are congenital heart disease, cleft lip/palate, urogenital and neural tube defects. The aim of our study is to examine teratogenic effects of AED and the correlation between these malformations and AED in single or multiple pregnancies. Methods: This is a retrospective study of malformations in children born to mothers currently followed up by our outpatient clinics who used or discontinued AED during their pregnancy. Their children were then investigated using echocardiography, urinary ultrasound, cranial magnetic resonance image, and examined by geneticists and pediatric dentists. Results: One hundred and seventeen children were included in the study. Ninety one of these children were exposed to AED during pregnancy. The most commonly used AED were valproic acid and carbamazepine in monotherapy. The percentage of major anomaly was 6.8% in all children. Dysmorphic features and dental anomalies were observed more in children exposed especially to valproic acid. There were 26 mothers with two and four mothers with three pregnancies from the same fathers. No correlation was found between the distribution of malformations in recurring pregnancies and AED usage. Conclusion: Our study has the highest number of dysmorphism examined in literature, found in all the children exposed to valproic acid, which may account for the higher rate of facial dysmorphism and dental anomalies. On lower doses of valproic acid, major malformations are not seen, although the risk increases with polytherapy. Our data also indicate possible effects of genetic and environmental factors on malformations., Research Fund of Istanbul University

We present the characteristics of eating epilepsy (EE), which is an underrecognized and complex form of reflex epilepsy. The files of 8996 patients with epilepsy were investigated retrospectively and only 6 cases (0.067%) were found. Four males and 2 females, aged 20 to 63 years, had focal seizures, mostly with dyscognitive and experiential aura triggered by eating, as well as spontaneous seizures. All had an initial precipitating event for seizures (such as head/birth trauma or encephalitis). In 4 patients, the seizures were induced in the middle of the meal or even closely after the end. In the remaining 2 cases the seizures were at the beginning of the meal, suggesting 2 different mechanisms. Two patients had normal magnetic resonance imaging (MRI), whereas the others had heterogeneous findings. The interictal electroencephalographs (EEGs) showed frequent spikes over a large area on the left temporal region in 5 and over the right temporal area in 1 case. We recorded eating-related seizures originating from the left temporal region in 3 cases. Positron-emission tomography (PET) investigations were concordant with the EEG foci in 2 patients. All but 1 had a therapy-resistant course. Our study suggested that EE is extremely rare and occurred many years after an initial precipitating event for seizures. These focal seizures, starting mostly with experiential/dyscognitive aura, usually originated from the left temporal region, had mostly a therapy-resistant course, and were triggered by different mechanisms during eating with a long latency in most of the cases.

Background Cardiac problems have been suggested as causes of sudden unexpected death in epilepsy (SUDEP). Our aim was to investigate possible associations of cardiac autonomic functions based on heart rate variability (HRV) parameters with risk factors of SUDEP in patients with drug-resistant epilepsy. Methods Forty-seven patients with drug-resistant seizures and 45 healthy control subjects were enrolled in the study. Interictal time domain parameters of HRV were evaluated with 24-hour Holter recordings. Potential SUDEP risk in patients with epilepsy was estimated using an inventory of seven validated SUDEP risk factors (The SUDEP-7 inventory). Results When compared with the healthy controls, all time domain measures (SDNN-24, SDNN-index, SDANN-index, RMSSD and pNN50) were significantly suppressed in the patient group. Scores of the SUDEP-7 inventory ranged from 1 to 9 with a median 4 out of a maximum possible risk score of 10. Maximum heart rate value in 24-hour Holter recordings and epilepsy duration were correlated with the SUDEP-7 scores (r = 0.3, p = 0.03). We found no significant association with HRV measures and SUDEP-7 risk factors. One patient diagnosed with Dravet syndrome died of SUDEP, which was autopsy confirmed; his SUDEP-7 inventory score was 7, HRV measures were significantly diminished, and his maximum heart rate (HR) was 208 beats/min (maximum HR is between 104 and 188 beats/min in normal subjects). Conclusion Patients with drug-resistant epilepsy present with significantly lower HRV measures, which may increase the risk for sudden cardiac death. Increased heart rate and diminished HRV measures may constitute one of the possible mechanisms underlying SUDEP and should be diagnosed in patients with epilepsy.

Purpose. There is a lack of knowledge on consecutive patients with epilepsy associated with bilateral hippocampal sclerosis (BHS). We aimed to investigate the differentiating features of BHS in comparison with unilateral HS (UHS). Method. We investigated our database for patients with epilepsy fulfilling the major magnetic resonance imaging criteria for BHS; namely, presence of bilateral atrophy and high signal changes on T2 and FLAIR series in the hippocampi. UHS patients seen in past 2 years were included as the control group. Clinical, EEG, and other laboratory findings, data on treatment response and epilepsy surgery were investigated from their files. Results. A total of 124 patients (31 with BHS and 93 with UHS; 49 right-sided and 44 left-sided) were included. We found that 16.1% of the BHS and 18.3% of the UHS groups were not drug-refractory. A binary logistic regression analysis performed with significant clinical features disclosed that history of febrile status epilepticus, mental retardation, and status epilepticus were statistically more common in BHS group. Moreover, diagnosis of psychosis established by an experienced psychiatrist and slowing of the EEG background activity were both found significantly more frequent in BHS. 66.67% of the operated BHS patients showed benefit from epilepsy surgery. Conclusions. BHS is a heterogeneous group, showing significant differences such as increased frequencies of mental retardation, status epilepticus, febrile status epilepticus and psychosis, in comparison to UHS. In all, 16.1% of the BHS cases showed a benign course similar to the UHS group and some patients with drug-resistant epilepsy may show benefit from epilepsy surgery.

Eyelid myoclonia (EM) with or without absences are a rare type of generalized seizures associated with a variety of epilepsy syndromes with an unknown pathophysiology. The aim of this study was to investigate the possible contribution of the brainstem structures in this underrecognized special type of seizures. Sixteen consecutive patients with EM, diagnosed with genetic generalized epilepsy (GGE) according to International League Against Epilepsy 2010 criteria were included. Brainstem excitabilities were examined by blink reflex (BR) studies. The results of BR studies in GGE patients with EM were statistically compared with 2 control groups; namely age- and gender-matched healthy volunteers and juvenile myoclonic epilepsy (JME) patients without any absences and using similar antiepileptic drugs. There were no statistical differences between the thresholds of the BR studies and the BR recovery curves in terms of amplitudes and areas of healthy subjects, JME patients and GGE patients with EM. Our findings do not support a profound interictal hyperexcitability in the BR-related brainstem structures of the GGE patients with EM. It can be considered that EM may be associated with excitability changes of the occipital cortex and other cortical areas.

Objective The attitude of patients with epilepsy towards their disease is an important factor in disease management and quality of life. The aim of this study was to define the attitudes of patients with epilepsy towards their disease and the factors that affect their attitudes. Patients and method This descriptive study was performed on patients admitted to an epilepsy outpatient clinic of a university hospital between May and September 2015. The sample consisted of 70 patients over 18 years of age with a diagnosis of epilepsy and no health problem other than epilepsy. Patients with no seizure in the last two years were excluded. The Epilepsy Attitude Scale was used to evaluate attitudes of the patients towards epilepsy; the Epilepsy Knowledge Scale, Rotter's Locus of Control Scale, Hospital Anxiety and Depression Scale (HADS), and the Quality of Life in Epilepsy-10 (QOLIE-10) were used to investigate the attitude-related factors. Results Among the 70 participants, 43 were female, and the mean age was 31.4 years. The educational level of the patients was lower (primary school) in 38.6% of the sample, and 18.6% were unemployed. Time since diagnosis was 15.1 years, 75.7% of the participants had generalized type of seizures, and more than half had seizures more frequently than once a month. The mean score of the attitude scale was 59.7 ± 6.62 (range: 14–70). The attitudes of the patients towards epilepsy were found to be related to their educational status, living alone, and the attitudes of their families. The attitude scores were also related to the level of knowledge on epilepsy, stigma, and depression. Furthermore, the attitude was found to be correlated with quality of life. Conclusion Patients with epilepsy had moderate-to-good attitude towards their disease. It was observed that the attitude was related to the knowledge, stigma, and depression rather than to demographic factors and the seizures, and furthermore, the attitude was found to be correlated with quality of life.

Summary Backgrand. Lateralized periodic discharges (LPDs) are infrequent electroencephalograph (EEG) findings, and may present in ictal or interictal form. They are regarded as potential electrophysiologic signs of convulsive or nonconvulsive status epilepticus (NCSE). We report four patients who presented with NCSE and one who presented with convulsive status epilepticus in the postictal period, characterized by LPDs in EEG recordings in the first year after stroke. Material and methods. We prospectively evaluated patients who clinically presented with status epilepticus associated LPDs between March 2014 and March 2015. We investigated patients that presented with a new stroke occurrence. We excluded the other LPD etiologies. EEG studies of five patients (two men) who were admitted to our emergency unit with confusion, three of whom had visual symptoms; four were treated for NCSE as diagnosed with LPDs. The fifth patient had convulsive status epilepticus with LPD in the postictal period. Results and Discussion. None of the five patients, who were aged between 68 and 92 years, showed any etiologic factor other than a history of cerebrovascular disease (CVD). Magnetic resonance imaging studies of the patients revealed old infarcts and transitional diffusion restrictions. The clinical and EEG findings decreased substantially upon antiepileptic drug treatment. Herein, we illustrate the first patient who had confusion, visual hallucinations, and ictal and interictal LPD in her consecutive EEGs. Conclusions. CVDs may pave the way for LPDs in patients with a history of stroke because CVDs cause structural brain damage. Patients who present with a similar clinical profile and imaging signs of stroke should be checked for NCSE, particularly in the presence of LPDs in EEGs.

Some patients with idiopathic/genetic generalized epilepsy (IGE) experience visual aura, which can confuse the diagnosis. We sought to determine the frequency and characteristics of visual auras in IGE patients. Among the 176 IGE patients, 4 men and 7 women reported visual auras (mean age — 24 years). Syndromic diagnoses were juvenile myoclonic epilepsy in four, eyelid myoclonia with absences (EMA) in three, juvenile absence epilepsy in three, and other in one. Visual auras consisted of flashing lights, macropsia, illusional movements, and blindness. Eyelid myoclonia with absences was significantly more common in the group with visual aura (3 of 11 patients vs. 8 of 165 IGE patients; P = 0.02). Furthermore, photosensitivity was found significantly more common in IGE patients with visual aura (90% vs 46% of the total IGE patients) (P = 0.004). In conclusion, the visual auras do not exclude a diagnosis of IGE. The presence of visual aura in the EMA syndrome is also remarkable.

Lennox-Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by several types of seizures, special EEG patterns, and cognitive deterioration with resistance to therapy and poor prognosis. It is a well known phenomenon that some antiepileptic drugs (AED) have a worsening effect on some seizure types, especially in the generalized epilepsies of childhood. However, its underlying pathogenetic mechanisms are not fully understood. In this paper, a case with LGS who developed tonic status epilepticus induced by diazepame given intravenously is reported and the topic of seizure aggravation caused by AED and LGS is discussed.

Stiripentol (STP), a recent antiepileptic drug, has been approved for use in addition to valproat (VPA) and clobazam in Dravet syndrome. On the other hand, the incidence of sudden unexpected death in epilepsy (SUDEP) commonly seen in patients with intractable epilepsy, is high in patients with Dravet yndrome. In our case, who was resistant to polytherapy and had multiple cardiopulmonary arrest due to status epilepticus (SE) or intravenous diazepam; STP was used in combination with other antiepileptic drugs (AED). However, acute reversible encephalopathy developed after six months of therapy. It is argued that the condition may have been caused by hyperamonnemia due to pharmacokinetic interaction between VPA and STP. Treatment options and protective measures are discussed using recent literature on Dravet syndrome, SUDEP and STP.

The typical clinical presentation of subacute sclerosing panencephalitis (SSPE) includes behavioral and intellectual changes followed by myoclonia. However, there are a considerable number of SSPE cases which present with distinct clinical features that can lead to a diagnostic difficulty. In this report, we summarize the clinical features of patients with SSPE who have uncommon presentations or features of the disease or coexisting medical conditions which may lead to diagnostic difficulties. We studied 173 patients, all under the age of 17. Patients were included in the study group according to following criteria: onset of the disease before age 2 years, seizures occurring before the onset of myoclonia and/or behavioral symptoms, extrapyramidal or cerebellar signs and ocular manifestations as initial presenting symptoms, fulminant course including coma or death within 6 months. Additionally, patients with onset of SSPE at the setting of a known neurological disorder are defined as another group in the study. Out of 173 patients with SSPE who were followed in two neurology centers, 31 (17.9%) met our criteria. We found a relative high frequency of these clinical features. Our findings suggest that clinicians should be aware of this clinical characteristics and rule out the disease in cases were other common causes have been excluded, especially in countries with insufficient measles immunization

Summary Purpose To evaluate the efficacy and tolerability of Levetiracetam as an add-on therapy in patients with startle epilepsy (StEp). Methods Ten (7 males and 3 females) were enrolled in the study. LEV was started at 500mg bid, escalating over 1–2 weeks to maximal doses of 3000mg daily, based on seizure control and tolerance for 13–28 months. Results The onset of startle seizures in patients with StEp varied from birth to 11 years. Six in 10 patients gave good responses to the treatment. There were adverse effects in three patients. Conclusion Many AEDs have been used by medically intractable patients with StEp for many years but the results were almost discouraging. It was observed that 60% of the patients gave good response to LEV. Advanced studies are required to indicate the efficiency of LEV which proved to be effective on animals with audiogenic seizures on reflex epilepsies.

Objective: Our aim was to assess the long-term follow-up of juvenile myoclonic epilepsy (JME), with an emphasis on the course of the myoclonic seizures. Methods: We enrolled 48 patients with JME (29 F, 19 M; aged 39.9 ± 9.5 years) followed up for a mean of 19.6 ± 5.7 years. The remission for 5 years and relapses were evaluated for all seizure types and the changes in severity/frequency of myoclonia were systematically questioned. The clinical and EEG features, antiepileptic drug (AED) treatment regimen, and systemic and psychiatric comorbid diseases were evaluated. Results: We found a benign course in 66.6% whereas 16.7% had pseudo-resistance due to problems in treatment or lifestyle. The true-resistant course observed in the remaining 16.7% was significantly associated with psychiatric disorders and the presence of thyroid diseases. In 54.2% of the patients, myoclonia were in remission for a mean duration of 8.4 ± 7.7 years, after an average age of 32.9 ± 9.6. Of these patients, 6 were on a lower dose of AED in comparison to the dosage needed to control the seizures in the beginning, and 5 patients had stopped AED treatment. None of the latter 11 patients except one relapsed during the follow-up. Furthermore, 21 other patients (43.8%) described substantial alleviation after age 31.3 ± 8.4 in the severity of myoclonia. Conclusion: Although a great majority of the patients with juvenile myoclonic epilepsy had continuing seizures after a follow-up of 20 years, almost all had either 5-year remission or a substantial alleviation of the myoclonic seizures.

Porphyrias present themselves with autonomic or peripheral neuropathy or central nervous system dysfunction. They are a varied group of inborn errors of metabolism that are characterized by specific inherited enzyme defects in haem biosynthesis. A patient whose mother was diagnosed as having porphyria was admitted to hospital because of her abdominal pains and dysuria. She had three generalized convulsions and, in a few hours, she lost the vision in both of her eyes. As the seizures continued, she became quadriparetic and fell into a coma after gradually losing consciousness. She improved but with sequelae; her serial MRIs, including apparent diffusion coefficient map imaging, diffusion-weighted imaging and angiography, showed ischaemic lesions that were both unlike and more severe than the ones reported in the literature.

INTRODUCTION Recent studies showed that hippocampal sclerosis (HS) patients with unilateral involvement had more diffuse cognitive impairment than expected. Therefore, we aimed to compare the cognitive profiles of bilateral HS (BHS) patients with unilateral HS (UHS) patients. METHODS Consecutive patients, diagnosed with epilepsy, who fulfilled two major magnetic resonance imaging (MRI) criteria (T1 atrophy and T2-FLAIR hyperintensity) for HS were included. Standard neuro-psychological test (NPT) battery consisted of the Turkish version of 15-word verbal memory processes test, Wechsler memory scale visual reproduction subtest, forward and backward digit span, phonemic and semantic fluency, and Stroop test were applied; and the groups with right HS, left HS, and bilateral HS were compared statistically. RESULTS Ninety-one patients, completing the NPT (34 males, 57 females)-16 with BHS, 36 with right HS, and 39 with left HS-were included. Six out of 43 operated patients had BHS. There were no significant differences in education and handedness of the groups. Even though NPT performances of the BHS group were found to be poor compared to UHS subgroups, this was beyond statistical significance. Comparison of BHS with the right HS group showed a significant difference in the learning score of the Verbal Memory Processes Test, but recognition scores were found to be similar in all groups. Compared to the BHS group, both right and left HS groups revealed a significant difference in delayed recall score of the Verbal Memory Processes Test. Although there were no significant differences in the postoperative parameters of the BHS group, UHS subgroups had deficits in many postoperative parameters. CONCLUSION Our study revealed that bilateral involvement of the hippocampi was correlated with a poor cognitive performance. Retrieval failure, rather than a total recall problem, in the memory of the patients resembles a more diffuse involvement not only limited to limbic structures.

Objective Our aim was to investigate the prevalence of neuronal autoantibodies (NAbs) in a large consecutive series with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) and to elucidate the clinical and laboratory clues for detection of NAbs in this prototype of frequent, drug-resistant epilepsy syndrome. Methods Consecutive patients diagnosed with MTLE fulfilling the MRI criteria for HS were enrolled. The sera of patients and various control groups (80 subjects) were tested for eight NAbs after ethical approval and signed consents. Brain tissues obtained from surgical specimens were also investigated by immunohistochemical analysis for the presence of inflammatory infiltrates. The features of seropositive versus seronegative groups were compared and binary logistic regression analysis was performed to explore the differentiating variables. Results We found antibodies against antigens, contactin-associated protein-like 2 in 11 patients, uncharacterised voltage-gated potassium channel (VGKC)-complex antigens in four patients, glycine receptor (GLY-R) in 5 patients, N -methyl-d-aspartate receptor in 4 patients and γ-aminobutyric acid receptor A in 1 patient of 111 patients with MTLE-HS and none of the control subjects. The history of status epilepticus, diagnosis of psychosis and positron emission tomography or single-photon emission CT findings in temporal plus extratemporal regions were found significantly more frequently in the seropositive group. Binary logistic regression analysis disclosed that status epilepticus, psychosis and cognitive dysfunction were statistically significant variables to differentiate between the VGKC-complex subgroup versus seronegative group. Conclusions This first systematic screening study of various NAbs showed 22.5% seropositivity belonging mostly to VGKC-complex antibodies in a large consecutive series of patients with MTLE-HS. Our results indicated a VGKC-complex autoimmunity-related subgroup in the syndrome of MTLE-HS.

Mutated measles virus variants have been claimed as the causing agent for subacute sclerosing panencephalitis (SSPE) developing several years after the recovery from measles infection. However, immune dysfunction may be considered related to a genetic susceptibility to this rare disease. Interleukin (IL)-2 -330 (rs2069 762) and +160 (rs2069 763), IL-12 p40 3' UTR (rs3213113), and interferon (IFN)-gamma +874 (rs2430561) polymorphisms are screened by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) and PCR-sequence-specific priming (SSP) methods in 87 SSPE patients and 106 healthy controls (HCs) as candidate genes of susceptibility. The distribution of the IL12B genotypes (rs3213113) showed a trend for a significant difference (P = .053). The frequency of IL12B C allele (P = .04, OR: 1.6) and CC genotype (P = .03, OR: 3.2) were both higher in SSPE patients than in HC. The IL2 -330 genotypes revealed lower frequencies of GG genotype (P = .03, OR: 0.4) as well as G allele (P = .02, OR: 0.6) in SSPE. IL2 -330+160 TG haplotype was more frequent in patients (P = .005, OR: 1.8), whereas GG haplotype was less frequent, compared to controls (P = .02, OR: 0.6). IFNG +874 polymorphism revealed no difference. These findings implicate possible effects of genetic polymorphisms in the susceptibility to SSPE, which need to be confirmed in other populations.

Autonomic dysfunction has frequently been reported in autoimmune encephalitis associated with seizures and there is growing evidence that epilepsy patients may display neuronal autoantibodies (NAAb). The aim of this study was to investigate the frequency of NAAb in epilepsy patients with peri-ictal autonomic findings. Fifty-eight patients (37 women/21 men; average age of 34.2 +/- A 9.9 years and epilepsy duration of 19.1 +/- A 9.6 years) who had at least one video-EEG recorded focal or secondary generalized seizure with clear-cut documented peri-ictal autonomic findings, or consistently reported seizures with autonomic semiology, were included. NAAb were tested by RIA or cell based assays. NAAb were present in 17 of 58 (29.3 %) patients. Among seropositive patients, antibodies were directed against N-methyl-d-aspartate receptor (NMDAR) in 5 (29 %), contactin-associated protein-like 2 (CASPR2) in 5 (29 %), uncharacterized voltage gated potassium channel (VGKC)-complex antigens in 3 (18 %), glutamic acid decarboxylase (GAD) in 2 (12 %), glycine receptor (GLYR) in one (6 %) and type A gamma aminobutyric acid receptor (GABA(A)R) in one patient (6 %). Peri-ictal gastrointestinal manifestations, piloerection, ictal fever, urinary urge, and cough occurred more commonly in the seropositive group. The prevalences of psychotic attacks and status epilepticus were significantly increased in the seropositive group. Seropositivity prevalence in our patient group with peri-ictal autonomic findings is higher than other previously reported epilepsy cohorts. In our study, ictal fever-VGKC-complex antibody and pilomotor seizure-GABA(A)R antibody associations were documented for the first time. Chronic epilepsy patients with peri-ictal autonomic semiology, history of status epilepticus and psychotic disorder may benefit from autoantibody screening.

To investigate T cell responses in subacute sclerosing panencephalitis (SSPE), we analyzed proliferation and cytokine secretion of cells from 35 patients and 42 healthy controls (HC) in response to central nervous system (CNS) antigens. The proliferation in response to myelin basic protein (MBP), myelin oligodendrocyte-glycoprotein (MOG) and αB-crystallin did not differ between groups. There was a trend towards a decrease in IL-12 production in response to MBP and in vitro IL-12 secretion of SSPE patients to measles virus vaccine (MVV) was lower than controls. Proliferation, as well as IFN-γ, IL-12 and IL-10 production in response to purified protein derivate (PPD) was impaired in SSPE patients. The results did not demonstrate any by-stander cellular response against myelin antigens, implicating that CNS is not a predominant target of an autoimmune response in SSPE. The recall responses were lower in SSPE as reported in measles before.

Immunosuppression associated with measles virus (MV) can be demonstrated by cytokine production failure in subacute sclerosing panencephalitis (SSPE) and may have implications on the pathogenesis of the disease. Cytokines (IL-12, IL-10, IL-4, IL-17, IL-18, IFN-alpha, IFN-gamma) and chemokines (CXCL8, CXCL10, CCL2 and CCL5) were measured in the cerebrospinal fluid (CSF) and serum samples from 60 patients with SSPE, 36 patients with infectious and/or inflammatory (IN) and 28 with other non-inflammatory (NIN) neurological diseases by ELISA. IL-12 p70+p40 was elevated in CSF and sera of SSPE when compared to the NIN group. However, the CSF levels of IL-12 p70 alone were not increased, indicating an increase of p40. The CSF of SSPE patients also showed relatively higher levels of IL-10 than that of the NIN group. CXCL10 levels in CSF were significantly higher in SSPE, whereas CXCL8 was increased in sera compared to NIN. No difference was detected in IFN-gamma, IFN-alpha, IL-17, IL-18, IL-4 or CCL2 and CCL5 levels. These results demonstrate that immune response against MV in SSPE may be impaired, although some T cell/Th1 inducing stimulations are present.

ISTANBUL'DA BIR KIRSAL ALANDA EPILEPSI PREVALANSI**** OZET Bu calisma Istanbul-Kucukcekmece Bolgesi'nde Haziran 1999 – Subat 2000 tarihlerinde gerceklestirilmis kesitsel ve vaka kontrollu bir alan calismasidir. Bolgede basit rastlantisal ornekleme yontemi ile secilen evler ( n = 493) tek tek gezilerek anket yontemi ile bireylerden ( n = 2187) % 2.65 inin epilepsi supheli oldugu ( n = 58) saptandi. Oyku alma, norolojik muayene ve EEG den yararlanilarak epilepsi tanisi alanlar ( n = 17) belirlendi. Yasamboyu Epilepsi Prevalansi % 0.8 idi. 17 epilepsili olgudan % 41.2 ( n = 7) si parsiyel, % 47.0 ( n = 8) si jeneralize, % 11.8 ( n = 2) i fokal ya da jeneralize oldugu belirlenemeyen epilepsi idi. Epilepsili bireyler ile ayni bolgeden olusturulan kontrol grubunda ( n = 125) epilepsi ile ilgili risk faktorleri Lojistik Regresyon Analizi ile arastirildi. Ogrenim durumu (Odds Ratio = 1.82 , % 95 Guven Araligi = 1.13–2.94, P = 0.01), meslek (OR = 0.76 , % 95 GA = 0.60 –0.97, P = 0.03), ailede epilepsili birey olmasi (OR = 0.67 , % 95 GA = 0.47 –0.94, P = 0.02) ile epilepsi arasinda istatistik anlamlilik verecek derecede iliski oldugu saptandi. Sonuclar bireylerin epilepsi konusunda bilgilendirilmesi gerekliligine dikkat cekmektedir. Anahtar Kelimeler : Noroepidemiyoloji, epilepsi, tarama. ****Bu calisma Istanbul Tip Fakultesi Arastirma Fonu tarafindan T – 693 / 280699 nolu proje olarak desteklenmistir. This study is a field study with cross-section and case control conducted in the Kucukcekmece Region of Istanbul between June 1999 and February 2000. Four hundred and ninety three dwellings selected by a simple random sampling method were visited. From a population of 2187, 58 people, after filling in a questionnaire were suspected to have epilepsy. Following an interview, neurological examination and an electroencephalogram (EEG) 17 were diagnosed as having epilepsy. Lifetime epilepsy prevalence was 0.8%. 41.2% of the 17 epilepsy cases had partial epilepsy ( n = 7), 47.0% had generalized ( n = 8) and in 11.8% seizures could not be classified ( n = 2). The risk factors for epilepsy in the control group ( n = 125) from the same region and those with epilepsy were investigated by means of logistic regression analyses. Educational status (odds ratio : 1.82, 95% confidence interval : 1.13–2.94; P = 0.01), profession (OR : 0.76, 95% CI : 0.60–0.97; P = 0.03), history of epilepsy in the family (OR : 0.67, 95% CI : 0.47–0.94; P = 0.02) were determined to be correlated with epilepsy. The results have drawn attention to the fact that individuals should be informed about epilepsy.

In order to outline the clinical and EEG characteristics of recurrent absence status epilepticus (ASE), eight cases with more than two attacks of ASE were studied. Their current ages were between 13 and 84 years, and five of the patients were women. There was a history of epilepsy in five of the patients before the first ASE episode. A varying degree of confusion was the main clinical symptom with associated mild motor signs like perioral, eyelid and generalised myoclonus, seen in one, two and four patients respectively. Two of the patients had juvenile myoclonic epilepsy. One patient had an atypical form of childhood absence epilepsy characterised by recurrent ASE attacks on awakening. There were two patients with phantom absences and late onset generalised convulsions, one patient with perioral myoclonia and absences, and finally two patients with eyelid myoclonia with absences, which are proposed syndromes. On the EEGs that revealed the diagnosis of ASE, there was a marked variability of the generalised multispike and wave discharges. The EEG findings appeared to be syndrome-related with some exceptions. IV Clonazepam lead to a dramatic improvement. Our study shows that the majority of recurrent ASE cases do not fit into the International syndrome classification.

Summary: Purpose: Our aim is to outline the clinical and electroencephalographic (EEG) features of patients with hot water epilepsy (HWE), a rare and unique form of reflex epilepsy. Methods: Twenty-one patients with HWE, seen in our clinic until 1999, were studied. Male outnumbered female subjects in a ratio of 3:1. The age at the onset of seizures ranged from 19 months to 27 years (mean age at onset, 12 years). Results: The main factors precipitating seizures were bathing with hot water and/or pouring water over the head. Six patients reported self-induction, either by increasing the heat or the amount of water and/or recalling earlier bathing experiences. Nine patients expressed feeling pleasure during the seizures. Twenty patients had partial seizures, eight of whom also had secondarily generalized seizures. One patient had apparent generalized seizures only. Spontaneous seizures were present in 62% of the cases. Interictal epileptogenic abnormalities were documented in the EEGs of eight patients; the other eight had normal EEGs. The major sites of epileptogenic activity were over the unilateral temporal regions (in 40% of patients). Neuroimaging studies available for 12 patients (four cranial computed tomography and eight cranial magnetic resonance imaging scans) revealed normal findings. Seizure control in patients who were followed up was achieved by reducing the temperature or the duration of the bath or shower; several of the patients required medication. Conclusions: The major findings of this study are that HWE has a male preponderance, can be self-induced, is often done for pleasure, has complex triggering factors, and shows temporally located abnormalities in the EEGs. Although HWE is generally known to be self-limited, antiepileptic drug treatment may sometimes be necessary to control seizures. Hot water epilepsy should be classified separately among the epileptic syndromes. Key Words: Reflex epilepsy—Reflex seizure— Hot water—Self-induction.

In this retrospective study 67 patients with SSPE seen between the years 1980 and 1998 were reviewed. Using the criteria of SSPE diagnosis (clinical signs, characteristic EEG patterns, high titres of measles antibodies in the serum and CSF), the patients were divided into two groups. Group A fulfilled all criteria, however, due to the inability of measuring measles antibody before 1987, it was not possible to observe the third criterion in Group B. Among 67 patients, groups A and B consisted of 51 boys and 16 girls ranging in age between 1 to 23 years, mean age 13.1. The male/female ratio was 3.1. The periodic EEG complexes (PCs) were usually bilateral, synchronous and symmetrical. PC amplitude asymmetry was seen in 12 patients and 2 patients had no PC synchronization between the hemispheres. Six patients had more than one form of PC. Delta activity in anterior hemispheres after PC was seen in 40 patients, mostly in stage 2A. Thirty-two patients had focal epileptiform abnormalities in multiple locations at every stage but most frequently in frontal, central and temporal regions. One patient had PC over both hemispheres and periodic lateralized epileptiform discharges (PLEDs) over the right hemisphere. The EEG findings described and observed in our study do not seem to be specific to SSPE but these findings were not atypical or unusual.