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COVID-19 is a novel infectious disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in which neurological complications have been increasingly recognized. Acute symptomatic epileptic seizures and status epilepticus are frequently reported neurological complications associated with this infection. The nervous system damage caused by SARS-CoV-2 may be mediated by the immune system. Interleukin 6 (IL-6), an important component of the cytokine storm, is directly correlated with the severity of symptoms. Tocilizumab is an inhibitor of IL-6 receptors, which blocks IL-6-mediated signal transduction and is used in the treatment of COVID-19 and status epilepticus.A patient with the Unverricht-Lundborg disease is presented who had developed refractory recurrent status epilepticus during COVID-19 infection, which was finally controlled by treatment with tocilizumab.Tocilizumab, an IL-6 inhibitor, may be considered as a treatment option in patients with status epilepticus and refractory seizures.

To describeGMH (subcortical heterotopia [SCH] and periventricular nodular heterotopia [PNH]) patients with epilepsy who underwentThirty patients (19 PNH; 11 SCH) with a mean age of 28.46 ± 9.52 years were enrolled. The heterotopic nodules were ametabolic in 3 patients (10%), hypometabolic in 16 (33.33%), isometabolic in 13 (26.66%), and hypermetabolic in 4 (10%).Heterotopic nodules in GMH patients show different metabolic patterns on

This report examines the effects of a multimodal rehabilitation program which includes cognitive, physical, and somatosensory rehabilitation after right temporo-parietal tumor resection on cognitive, motor, somatosensory, and electrophysiological parameters.A 22-year-old patient presented with sensory loss in the dominant left hand and reduced writing ability after right temporo-parietal lobe resection. Cognitive, motor, and sensory evaluations were carried out pre and post-treatment. The patient's spontaneous electroencephalo-gram (EEG) and an EEG during application of transcutaneous electrical nerve stimulation (TENS) (TENS EEG) were recorded. As a reference for the patient's electrophysiological values, EEGs of 4 healthy individuals were also taken. Over a period of 1 year, the patient received multimodal rehabilitation which includes cognitive, physical, and somato-sensory rehabilitation on 2 days each week.An improvement of the patient's cognitive capacities, motor strength, superficial, deep and cortical sensations was achieved. After rehabilitation, an increase in parietal and occipital alpha activity as well as in frontal and parietal beta activity was seen both in spontaneous EEG and in TENS EEG. With increasing TENS intensity, alpha and beta power increased as well.Our findings suggest that a multimodal rehabilitation program may improve cognitive, sensory, and motor effects after resection due to tumor surgery.

The aim of this study was to conduct item reduction of the Memory Assessment Clinics Self-Rating Scale (MAC-S) to create a briefer measure that can be used to quickly evaluate subjective memory com...

Purpose The temporal lobe plays an important role in the perception of odors. Another important anatomical structure involved in the perception of smell is the olfactory bulb. The relationship between olfactory bulb volume (OBV) and olfactory function is unclear, however. In our study, we aimed to evaluate the relationship between OBV and olfactory function in patients with mesial temporal lobe epilepsy (MTLE). Method Forty-eight patients with MTLE who were managed in the Cerrahpasa Medical Faculty Neurology Department’s epilepsy outpatient clinic were recruited for the study. Tests with Sniffin’ Sticks and Taste Strips were performed and OBV measured in all patients. Two control groups were recruited, one for the Sniffin' Sticks and Taste Strip tests and one for OBV measurement. Results In the smell tests; the threshold, discrimination and identification scores were significantly lower in the MTLE group than in the control group. The mean taste test scores did not differ significantly between the MTLE and control groups. In the MTLE group, the mean right OBV was 40.2 ± 12.54 and the left OBV was 39.3 ± 10.54, both of which were significantly lower those in the control group. The mean OBVs of patients with hyposmia were significantly smaller bilaterally than the OBVs of those with normosmia. There was no correlation between the gustatory scores and OBV. Conclusions Olfactory function was significantly impaired in patients with MTLE compared with healthy controls in all domains, namely threshold, discrimination, and identification. In addition, olfactory bulbus volume was smaller in patients with olfactory dysfunction.

Aim of the study Temporal lobe epilepsy (TLE) has been associated with the phenomenon of accelerated long-term forgetting (ALF). In this study, we aimed to demonstrate the effect of surgery on the ALF phenomena thus contributing to potential explanation of the causal mechanism. Materials and methods We evaluated 51 patients with TLE related to hippocampal sclerosis who had amygdalohippocampectomy and had remained seizure-free after surgery. A control group consisted of 24 healthy individuals. All were given a verbal learning test assessing recall after 30 min, 1 week and 6 weeks. Results In our study, the Left-TLE (L-TLE) group showed a statistically significant reduction in the performance at all assessment intervals from 30 min to 1 week compared to the Right-TLE and control groups regarding verbal learning memory test (VLMT) as well as for logical memory. The forgetting rates in the VLMT from 30 min to 1 week were not statistically significantly different between all 3 groups. The logical memory test results equally showed no statistically significant difference in the forgetting rates for the 3 groups between 30 min and 1 week. Conclusions and clinical implications These results may support ongoing debates assuming the initial low performance in the memory of L-TLE patients to be directly related with left hippocampal-temporal tissue loss irrespective of epileptic activity. The discovery of the ALF phenomenon explains that standard memory tests are unable to detect memory loss in some patients who are experiencing a significant level of problems with forgetfulness in their daily lives.

Aim Vagus nerve stimulation (VNS) is a well-known and increasingly common therapy. However, efficacy may differ depending on the type of epilepsy. In this study, we aimed to investigate and compare the efficacy and safety of VNS therapy in different types of epilepsy. Material and methods Patients, who were implanted with VNS between the years 2005 and 2020, were retrospectively included in the study. Age, gender, age at seizure onset, epilepsy types, VNS implantation year, replacement year, pre and post-VNS seizure frequency, number of responders, number of antiseizure medication and adverse events were recorded. Results In total, 41 patients were included in the study. The number of patients with focal epilepsy was 21 (51.2%). 10 patients (24.4%) had generalized epilepsy and 10 patients (24.4%) had "combined generalized and focal epilepsy" (Lennox-Gastaut, Dravet syndrome). The Pre-VNS median seizure frequency was 1.5/day in the focal group, 0.6/day in the generalized group and 6/day in the combined group. Seizure frequencies dropped to 0.3/day in the focal group, 0.2/day in the generalized group and 3.0/day in the combined group at the 12th month after VNS (p 0.001, p=0.004, p 0.001). The response rate was found to be 68.3% at the 12th month after VNS. The number of antiseizure medications was decreased from 3.6/day to 3.1/day at the 12th months after VNS (p 0.001). Two patients\' (4.9%) VNS therapy was discontinued due to adverse events. Conclusion The study indicates that VNS therapy is safe and effective in focal, generalized and combined epilepsy types. Despite having a low seizure freedom rate, VNS is a good alternative treatment option for patients who for any reason are not candidates for resective surgery.

AIM Cerebral cavernous malformations (CCM) are the second most common type of vascular malformations and also regarded as highly epileptogenic lesions if they involve the cortex. We present one of the largest retrospective cavernoma related epilepsy (CRE) studies which include divergent supratentorial locations operated and followed up at least 2 years. We also investigated the factors affecting the seizure outcome. MATERIAL AND METHODS This study includes 56 patients with drug-responsive (40) and drug-resistant (16) CRE who underwent resective surgery. Age at seizure onset, age at surgery, gender, duration of epilepsy, seizure frequencies/type before and after treatment, EEG and brain MRI findings, prescribed AEDs, preoperative and post-operative neurological status, histopathological diagnosis, post-operative seizure outcomes and surgical information were documented. RESULTS The average follow-up period was 69.6 months (range 24-216 months). The seizure outcome was assessed according to Engel's classification at the last follow-up. Engel class I was achieved in 53 patients (95%); there was one patient at class II and two patients at class III. All patients in the drug-responsive group were at Engel class I after the surgery, while all patients at Engel classes II and III were in the drug-resistant patient group. This clearly shows that there were better outcomes in DRP group (p 0.01). Neither the locations of cavernomas nor the duration of epilepsy had any impact on seizure outcome (p 0.05). CONCLUSION An earlier surgical intervention may prevent the patients from becoming drug-resistant such that their chances of being seizure free after surgery increase.

Lafora disease (LD) is a severe form of progressive myoclonus epilepsy inherited in an autosomal recessive fashion. It is associated with biallelic pathogenic variations in EPM2A or NHLRC1, which encode laforin and malin, respectively. The disease usually starts with adolescent onset seizures followed by progressive dementia, refractory status epilepticus and eventually death within 10 years of onset. LD is generally accepted as having a homogenous clinical course with no considerable differences between EPM2A or NHLRC1 associated forms. Nevertheless, late-onset and slow progressing forms of the disease have also been reported. Herein, we have performed clinical and genetic analyses of 14 LD patients from 12 different families and identified 8 distinct biallelic variations in these patients. Five of these variations were novel and/or associated with the LD phenotype for the first time. Interestingly, almost half of the cases were homozygous for the rare rs769301934 (NM_198586.3(NHLRC1): c.436 G > A; p.(Asp146Asn)) allele in NHLRC1. A less severe phenotype with an onset at a later age may be the reason for the biased inflation of this variant, which is already present in the human gene pool and can hence arise in the homozygous form in populations with increased parental consanguinity.

The autonomic nervous system, formerly the vegetative nervous system, is a division of the peripheral nervous system that supplies smooth muscle and glands, and thus influences the function of internal organs. The hypothalamus is the main center responsible for the autonomic functions in the central nervous system. Other anatomical structures can be listed as cingulate gyrus, amygdala, paraventricular nucleus. The central autonomic network involved in the pathophysiology of autonomous epilepsy is represented in the non-dominant hemisphere. Autonomic seizures are shown in temporal and insular lobe epilepsies commonly. Blood pressure changes, heart rhythm disturbances (tachycardia, bradycardia, arrhythmia, asystole), respiratory changes (apnea, hypopnea, bradypnea), salivation increase, vomiting/retching, pilo-erection, sweating increase, mydriasis/miosis, spitting, water drinking, genital automatism, intestinal motility disorders are clinical signs in autonomic epilepsy. SUDEP (sudden unexpected death in epilepsy) is defined as sudden, unexpected, nontraumatic, non-drowning death in an individual with epilepsy, witnessed or unwitnessed, in which post-mortem examination does not reveal an anatomical or toxicological cause of death. The vast majority of SUDEPs occur in the aftermath of a generalised tonic-clonic seizure. It’s responsible for 17% of deaths in epileptic patients. Witnessed recorded SUDEP cases involve postictal cardiorespiratory dysfunction with failure of arousal. It is reported that the threshold value of SpO2 is 80-86% for the risk of sudden death. While patients who have had seizures remotely and have had successful epilepsy surgeries also carry the SUDEP risk, the most important risk factor is a history of generalised tonic-clonic seizures.

Lafora disease (LD) is a severe form of progressive myoclonus epilepsy inherited in an autosomal recessive fashion. It is associated with biallelic pathogenic variations in EPM2A or NHLRC1, which encode laforin and malin, respectively. The disease usually starts with adolescent onset seizures followed by progressive dementia, refractory status epilepticus and eventually death within 10 years of onset. LD is generally accepted as having a homogenous clinical course with no considerable differences between EPM2A or NHLRC1 associated forms. Nevertheless, late-onset and slow progressing forms of the disease have also been reported. Herein, we have performed clinical and genetic analyses of 14 LD patients from 12 different families and identified 8 distinct biallelic variations in these patients. Five of these variations were novel and/or associated with the LD phenotype for the first time. Interestingly, almost half of the cases were homozygous for the rare rs769301934 (NM_198586.3(NHLRC1): c.436 G A; p.(Asp146Asn)) allele in NHLRC1. A less severe phenotype with an onset at a later age may be the reason for the biased inflation of this variant, which is already present in the human gene pool and can hence arise in the homozygous form in populations with increased parental consanguinity.

Purpose In mesial temporal lobe epilepsy with hippocampal sclerosis, there is parietal atrophy and cognitive involvement in related domains. In this context, we hypothesized that inhibitory input into somatosensory cortex and thalamus may be increased in these patients, which could improve after epilepsy surgery. Thus, we analyzed the inhibitory function of somatosensory system by studying surround inhibition (SI) and recovery function of somatosensory evoked potentials in patients with mesial temporal lobe epilepsy with hippocampal sclerosis. Methods Nine patients with unoperated mesial temporal lobe epilepsy with hippocampal sclerosis, 10 patients who underwent epilepsy surgery, and 12 healthy subjects were included. For SI of somatosensory evoked potentials, we recorded somatosensory evoked potentials after stimulating median or ulnar nerve at wrist separately and after median and ulnar nerves simultaneously and calculated SI% in all participants. For recovery function of somatosensory evoked potentials, paired stimulation of median nerve at 40- and 100-millisecond intervals was performed. We compared the findings among groups. As a secondary analysis, we determined the outliers in the patient group and analyzed the relation to the clinical findings. Results The mean SI% or recovery function was similar among three groups. However, there were five patients with SI loss on normal side in the patient group, which was related to the antiseizure drugs. Conclusions In contrast to our hypothesis, both intracortical (SI) and thalamic/striatal (recovery function) inhibitory modulation of the somatosensory cortex was not altered in mesial temporal lobe epilepsy with hippocampal sclerosis and did not differ in surgical and nonsurgical groups.

This study aimed to investigate ultrastructural synaptic alterations in rat hippocampus after in utero exposure to irradiation (IR) and postnatal exposure to hyperthermia (HT). There were four groups in each of the time points (3(rd) and 6(th) months). IR group: Pregnant rats were exposed to radiation on the 17(th) gestational day. HT group: Hyperthermia was applied to the rat pups on the 10th day after their birth. IR+HT group: Both IR and HT were applied at the same time periods. Control group: No IR or HT was applied. Rat pups were sacrificed after 3 and 6 months. Thin sections from the dentate gyrus (DG) and the CA3 of hippocampus were evaluated for synapse numbers by electron microscopy. Synapses were counted, and statistical analysis was performed. Abnormalities in myelin sheath, mossy terminals and neuropil were observed in the CA3 and DG of all groups. The synapses in the CA3 region were significantly increased in the IR-3(rd) month, IR-6(th) month, and IR+HT-3(rd) month groups vs control group. Synapses were significantly increased in the DG of HT-3(rd) month group. A trend for an increase in synapse numbers was seen in the CA3 and DG. Increased number of synapses in the rat hippocampus may be due to mossy fiber sprouting, possibly caused by in utero irradiation and/or postnatal hyperthermia.

Background: In cases undergoing epilepsy surgery, postoperative psychogenic nonepileptic seizures (PNES) may be underdiagnosed complicating the assessment of postsurgical seizures outcome and the clinical management. We conducted a survey to investigate the current practices in the European epilepsy monitoring units (EMUs) and the data that EMUs could provide to retrospectively detect cases with postoperative PNES and to assess the feasibility of a subsequent postoperative PNES research project for cases with postoperative PNES. Methods: We developed and distributed a questionnaire survey to 57 EMUs. Questions addressed the number of patients undergoing epilepsy surgery, the performance of systematic preoperative and postoperative psychiatric evaluation, the recording of sexual or other abuse, the follow-up period of patients undergoing epilepsy surgery, the performance of video-electroencephalogram (EEG) and postoperative psychiatric assessment in suspected postoperative cases with PNES, the existence of electronic databases to allow extraction of cases with postoperative PNES, the data that these bases could provide, and EMUs' interest to participate in a retrospective postoperative PNES project. Results: Twenty EMUs completed the questionnaire sheet. The number of patients operated every year/per center is 26.7 (+/- 19.1), and systematic preoperative and postoperative psychiatric evaluation is performed in 75% and 50% of the EMUs accordingly. Sexual or other abuse is systematically recorded in one-third of the centers, and the mean follow-up period after epilepsy surgery is 10.5 +/- 7.5 years. In suspected postoperative PNES, video-EEG is performed in 85% and psychiatric assessment in 95% of the centers. An electronic database to allow extraction of patients with PNES after epilepsy surgery is used in 75% of the EMUs, and all EMUs that sent the sheet completed expressed their interest to participate in a retrospective postoperative PNES project. Conclusion: Postoperative PNES is an underestimated and not well-studied entity. This is a European survey to assess the type of data that the EMUs surgical cohorts could provide to retrospectively detect postoperative PNES. In cases with suspected PNES, most EMUs perform video-EEG and psychiatric assessment, and most EMUs use an electronic database to allow extraction of patients developing PNES. (C) 2020 Elsevier Inc. All rights reserved.

Jeavons syndrome (JS) is mainly characterized by eyelid myoclonia with or without absences. It is thought to be underdiagnosed rather than have a rare prevalence. We aimed to investigate the electroclinical features of JS to determine possible factors influencing the diagnosis. We retrospectively identified the medical records of 32 cases (0.55%) from 5,796 patients with epilepsy. The inclusion criteria were: (1) eyelid myoclonia with or without absences; (2) generalized paroxysmal activity on EEG; and (3) discharges triggered by eyelid closure and/or intermittent photic stimulation. Eighteen (56.2%) of the patients were female. The mean age at seizure onset was 8.7±5.3 years and the mean age at admission to hospital was 17.8±10.7 years. A family history of epilepsy was present in 15 (46.8%) patients. Eyelid myoclonias were noticed in six (18.7%) patients by themselves. Based on the analysis of video-EEG recordings, 26 (81.2%) patients were sensitive to eye closure, 22 (68.7%) had photoparoxysmal responses, and 16 (50%) presented with absence seizures. Ten (31.2%) patients had focal epileptic discharges. Eight (25%) patients were on monotherapy. Seven (21.8%) patients achieved seizure freedom. Three patients underwent ketogenic diet therapy, which was effective in two patients. A vagus nerve stimulator was implanted into three patients, one of whom reported seizure reduction. Eyelid myoclonias are the main seizure type of JS but are usually overlooked. The time interval between seizure onset and clinical diagnosis suggests that this syndrome continues to be under-recognized. The genetic heterogeneity and phenotypic variability are likely to be more extensive than currently recognized, making the diagnosis more phalangine. [Published with video sequence].

Prepulse modulation (PPM) is an electrophysiological method which enables to assess sensory processing in vivo. Reflex responses may be facilitated or inhibited (prepulse inhibition, PPI) after a weak stimulus. Theoretically, in animal studies, the generator of PPI involves pedunculopontine nucleus which is modulated by various structures, including amygdala. We aimed to investigate whether or not there was a role of limbic structures in the generation of PPM in humans. For this purpose, we studied PPM of the blink reflex (BR) in 10 patients with mesial temporal lobe epilepsy (MTLE group) and in nine patients who had previously undergone amygdala resection for medically resistant MTLE (surgery group). A control group including 19 healthy volunteers was formed. Blink reflex, BR-PPM and BR excitability recovery were recorded in all participants. Two components of BR, first early ipsilateral component (R1) and second late bilateral components (R2 and R2c) were identified. All BR parameters after single stimulation were normal in all groups. Compared to healthy subjects, R2-PPI was more pronounced in the surgery group whereas there was a R2-PPI deficit in the MTLE group. R2-PPI deficit in the MTLE group was more prominent on the lesion side. Ipsilesional R1 facilitation was more evident at ISI of 100 ms in both MTLE and surgery groups compared to healthy subjects. BR excitability recovery was not different between groups. MTLE in humans leads to a PPI deficit. Interestingly, removal of amygdala in humans with MTLE probably provides more efficient functioning of PPI network. Amygdala and hippocampus play roles in the human R2-PPI circuit. Modulation of R1 facilitation is unilateral whereas the modulation of R2-PPI is bilateral, though asymmetric.

To analyse the clinical, imaging and histopathological data of patients who were diagnosed to have Dysembrioplastic Neuroepithelial Tumour (DNET) and underwent surgery between 1995-2015.Age at seizure onset, age at surgery, gender, disease duration, seizure outcome of 44 patients were analysed together with Magnetic Resonance Imaging (MRI) of 21 patients. MRI types were classified as type 1 (cystic/polycystic-like, well-delineated, strongly hypointense T1), type 2 (nodularlike,heterogeneous), type 3 (dysplastic-like, iso/hyposignal T1, poor delineation, gray-white matter blurring).Histopathological classification revealed simple form in 19, complex in 14 and non-specific in 11 patients. Lobar distribution of the lesions was as follows: 21 Temporal (47.7%), 12 parietal (27.3%), 8 frontal (18.2%) and 3 occipital (6.8%). Type 1 MRI was observed in 10, type 2 was in 7, and type 3 in 4 patients on radiological evaluation. All cases with type 1 MRI corresponded to either simple or complex forms and all cases with type 3 MRI corresponded to nonspecific form. The histopathological distribution of cases with type 2 MRI was 4 as non-specific, 2 as simple, 1 as complex. There was no significant difference in the age of onset, age at operation and duration of epilepsy between the patients with different MRI subtypes. The majority of patients (N:36) had Engel I outcome (81,8%). In groups with Engel II and III outcome, duration of epilepsy was significantly higher (p:0,014) and simple form of DNET has significantly higher seizure freedom after surgery compared to complex and nonspecific forms of DNET (p:0,002).Patients with DNET constitute a group with favorable outcomes after epilepsy surgery especially with early referral to surgery. Longer duration of epilepsy was associated with worse seizure outcome for DNET patients. There was significant correlation between radiological and histopathological types of DNET especially in type 1 and 3.

Status epilepticus (SE) is one of the most frequent neurologic emergencies. Generalized convulsive SE, which is the most frequent and easily recognized type of SE, has the highest morbidity and mortality rates. It is important to initiate early and aggressive treatment without delay. In recent years, clinical studies have focused on the prehospital treatment of epileptic seizures. Therefore, written algorithms including prehospital settings will provide rapid application of diagnosis and treatment protocol steps. In this article, the results of the workshop on multidisciplinary treatment of SE are reported in light of new literature

Background and Objective. Auditory startle response (ASR) was normal in juvenile myoclonic epilepsy whereas it was suppressed in progressive myoclonic epilepsy. However, both groups were using valproic acid/Na valproate (VPA) in different doses. Therefore, we aimed to analyze whether VPA has an impact on ASR in a cohort of epilepsy. For this purpose, we included patients with epilepsy and analyzed ASR in patients who were using VPA. Patients and Method. We included 51 consecutive patients who had epilepsy and were using VPA between January 2014 and January 2016. Two control groups of 37 epilepsy patients using other antiepileptic drugs (AEDs) and of 25 healthy subjects were also constituted. All participants underwent investigations of ASR and startle response to somatosensory inputs (SSS) under similar conditions. Results. An analysis of patients using VPA, not using VPA and healthy subjects revealed significantly longer latency and lower probability of orbicularis oculi (O.oc) and sternocleidomastoid responses after auditory stimulation, decreased total ASR probability and longer latency of O.oc response after somatosensory stimulation in patient groups compared with healthy subjects. Multivariate analysis showed type of AED had a role in the generation of abnormalities. VPA, carbamazepine, and multiple AED use caused suppression of ASR. Total ASR probability was decreased or O.oc latency got longer with longer duration of VPA use whereas serum VPA level at the time of investigation did not correlate with total ASR probability. Discussion. Both ASR and SSS are suppressed by the effect of VPA, especially in patients using for a long period and in patients using other AEDs with VPA. Given the fact that VPA leads to long-standing synaptic changes of dopaminergic transmission, abnormalities of this network may be the more likely cause.

Idiopathic generalized epilepsy (IGE) is a type of epilepsy with a monogenic or polygenic inheritance pattern representing 47% of all epilepsies. Idiopathic epilepsies have a complex genetic structure and the genetic factors involved in the development of epilepsy are still unclear. Dog models are often used in researching the complex genetic basis of epilepsy. A study of Rhodesian Ridgeback (RR) dogs with generalized myoclonic epilepsy found a 4-bp-deletion in the second exon of the DIRAS1 gene. In the literature, there is no study on the presence of a relationship between DIRAS1 gene and epilepsy in human population. We aimed to analyze whether there is a relationship between human epilepsy and DIRAS1 gene. For genomic analysis, peripheral blood samples were taken from 109 patients diagnosed with IGE and 51 healthy volunteers in the Turkish population. Genomic DNA isolation was performed from blood samples and the DIRAS1 gene region of the genomic DNA was amplified by Polymerase Chain Reaction (PCR). PCR products were purified and sequenced. DNA sequencing results were analyzed by chi-squared test. In the patient and control groups, G/C change was found in nucleotide 549 of DIRAS1 gene. No significant correlation was found between the distribution of DIRAS1-549-G/C genotypes and allele frequencies (p = 0.8 and p = 0.7, respectively). In this study, DIRAS1-549-G/C polymorphism has been shown not to play an important role in the development of epilepsy in the Turkish population. As a result, new data on IGEs whose molecular mechanism is still not fully elucidated has been revealed.

Introduction Recent ILAE classification defined focal cortical dysplasia (FCD) patients with accompanying epileptic lesions as a separate group. We investigated data of patients with sole FCD lesions regarding long-term seizure outcome and different characteristics of FCD type 1 and type 2 patients. Methods Eighty children and adult patients underwent surgery for FCD were included to the analysis of factors differentiating FCD type 1 and type 2 groups and their effect on long-term outcome. Results FCD type 2 patients had earlier epilepsy onset (8.1 vs. 6.1 years. p = 0.019) and underwent surgery younger than type 1 (18.2 vs. 23.7 years. p = 0.034). FCD type 2 patients were more prominently MR positive (77.8% vs. 53.8%. p = 0.029), which increased within FCD type 2 group as patients become younger (p = 0.028). FCD Type 1 lesions showed mostly multilobar extension and FCD type 2 mostly located in frontal lobe. Seizure freedom was achieved in 65.4% of FCD type 1 patients and 70.4% of FCD type 2 patients. Seven patients had permanent de novo neurological deficits. Mean follow-up time was 5.5 years (Range: 1–11 years). Conclusion Surgical intervention in carefully selected patients may facilitate favorable seizure outcome leading to better quality of life. FCD type 1 and type 2 groups present with evident differences, which may promote medical and surgical management of these pathologies.

Reports on the clinical presentation of adult-onset neuronal ceroid lipofuscinoses (NCL) are scarce compared to infantile- and childhood-onset forms. Here, we aimed to present detailed temporal evolution of clinical and electrophysiological features of two siblings with adult-onset NCL and homozygous mutation in the CLN6 gene. We retrospectively analysed medical records and electrophysiological data in order to delineate evolution of clinical and electrophysiological findings. Electrophysiological studies included routine EEG and video-EEG, as well as polymyographic analysis of myoclonus and brainstem reflex studies. Both patients had seizures and cerebellar signs. Despite the slow progression of ataxia, they developed no mental deterioration, but had severe obsessive compulsive disorder and depression. EEG revealed frequent generalized spikes, polyspikes, and waves, prominent on awakening and during photic stimulation without significant change throughout the clinical course. Abnormalities concerning the blink reflex, auditory startle response, and startle response to somatosensory inputs manifested within four years. The patients underwent transient and mild improvement with valproate, whereas ataxia and seizures were dramatically ameliorated following high-dose piracetam. Patients with adult-onset NCL may present with slowly progressive ataxia, persistent photosensitivity, and seizures without dementia or extrapyramidal findings. Brainstem abnormalities become more evident with time, in line with ataxia. Piracetam is effective for both seizures and ataxia.

Aydin, Senay/0000-0003-4460-9056; OZKARA, CIGDEM/0000-0003-0548-1838 WOS:000480664600009 PubMed ID: 31252363 Purpose: There are conflicting data regarding the drug dose that is sufficient to achieve seizure control as well as the parameters of seizure remission in juvenile myoclonic epilepsy (JME). The present study aimed to identify factors that contribute to seizure control in JME and to evaluate factors associated with JME remission and the efficacy of low-dose valproic acid (VPA) therapy. Methods: This retrospective, cross-sectional study included a total of 215 patients (121 female and 94 male; mean age: 28.03 +/- 8.43 [14-59] years) diagnosed with JME. The patients were divided into remission and refractory groups. Remission was defined as a seizure-free (myoclonic, absence, and/or generalized tonic-clonic) period of at least 2 years. Patients in whom remission was achieved with VPA monotherapy were further divided into two groups according to the use of low-dose VPA therapy (VPA 750 mg/day). Potential contributing factors were evaluated in terms of the relationship between the dose and the remission parameters. Results: Remission was achieved with VPA monotherapy in 116 patients (87.9%) in the remission group; the VPA dose was

Objectives: Surgical intervention is a crucial and effective treatment option for patients with temporal lobe epilepsy whose seizures are not under con-trol. However, there is a possibility that surgical intervention may have a negative effect on cognitive functions. Cognitive rehabilitation is a treatment option that has been recently investigated for various neurocognitive problems. This study proposes a protocol for the rehabilitation of the cognitive dysfunctions after temporal lobe epilepsy surgery. Methods: To overcome the cognitive deficits that occurred after surgery, a six-step program was developed, which included the compensatory and adaptation strategies and memory and executive functions. This program was performed after epilepsy surgery in two patients who were evaluated with neuropsychometric test battery. Results: Cognitive rehabilitation program had a significant and positive effect on the neuropsychometric test results in two epilepsy patients who had postoperative attention, memory and executive function problems. Discussion: In the postoperative period, patients with temporal lobe epilepsy may be able to recover from cognitive disorders with an effective CR program. Although our results were obtained only with two patients, they suggest that cognitive rehabilitation had a significant and positive effect on epilepsy patients with postoperative attention, memory and executive function problems. However, the onset of the rehabilitation before or after surgery, the duration and the content of the rehabilitation are controversial issues. Conclusion: Future studies should show evidence basis of the standardized rehabilitation program for patients after epilepsy surgery, and the short and long-term effects of the rehabilitation with larger participants. Amaç: Nöbet kontrolü sağlanamayan temporal lob epilepsi hastaları için cerrahi müdahale önemli ve etkili bir tedavi seçeneğidir. Ancak cerrahi müdahalenin bilişsel fonksiyonlar üzerinde olumsuz etki oluşturma ihtimali vardır. Son zamanlarda bilişsel rehabilitasyon, çeşitli nörobilişsel problemler için araştırılan bir tedavi seçeneğidir. Bu çalışmanın amacı, temporal lob epilepsi cerrahisi sonrası bilişsel işlevlerdeki bozulmanın rehabilitasyonuna yönelik bir protokol önerisi sunmaktır. Gereç ve Yöntem: Cerrahi müdahale sonrası meydana gelmiş bilişsel bozuklukların üstesinden gelmek için kompansatuar ve adaptasyon stratejilerini içeren, bellek ve yürütücü fonksiyonları geliştiren, altı adımdan oluşan bir program düzenlendi. Nöropsikometrik test bataryası ile ayrıntılı şekilde değerlendirilen iki hastaya epilepsi cerrahisi sonrası bu program uygulandı. Bulgular: Ameliyat sonrası dikkat, bellek ve yürütücü işlev sorunları yaşayan iki epilepsi hastasında, kognitif rehabilitasyon programının nöropsikolometrik test sonuçlarına göre anlamlı ve olumlu etkisi olduğu görüldü. Sonuç: Ameliyat sonrası dönemde temporal lob epilepsi hastalarının yaşadıkları/yaşayacakları bilişsel bozuklukların iyileşmesi etkin bir BR programı ile mümkün olabilir. Her ne kadar sonuçlarımız iki hasta ile elde edilmiş olsa da ameliyat sonrası dikkat, bellek ve yürütücü işlev sorunları yaşayan epilepsi hastalarında kognitif rehabilitasyonun anlamlı ve olumlu etkisi olduğunu gösterdi. Rehabilitasyonun bilişsel yetenekler üzerinde olumlu bir etkisi vardır, ancak rehabilitasyonun cerrahiden önce veya sonra başlaması, rehabilitasyon süresi ve içeriği tartışmalıdır. Gelecekteki çalışmalar cerrahi sonrası epilepsi hastaları için standartlaştırılmış rehabilitasyon programını, rehabilitasyonun kısa ve uzun dönem etkilerini daha büyük katılımcılar ile kanıta dayalı şekilde göstermelidir.

Kufs disease is the major adult form of neuronal ceroid lipofuscinosis, but is rare and difficult to diagnose. Diagnosis was traditionally dependent on the demonstration of characteristic storage material, but distinction from normal age-related accumulation of lipofuscin can be challenging. Mutation of CLN6 has emerged as the most important cause of recessive Kufs disease but, remarkably, is also responsible for variant late infantile ceroid lipofuscinosis. Here we provide a detailed description of Kufs disease due to CLN6 pathogenic variants. We studied 20 cases of Kufs disease with CLN6 pathogenic variants from 13 unrelated families. Mean age of onset was 28 years (range 12-51) with bimodal peaks in teenage and early adult life. The typical presentation was of progressive myoclonus epilepsy with debilitating myoclonic seizures and relatively infrequent tonic-clonic seizures. Patients became wheelchair-bound with a mean 12 years post-onset. Ataxia was the most prominent motor feature. Dementia appeared to be an invariable accompaniment, although it could take a number of years to manifest and occasionally cognitive impairment preceded myoclonic seizures. Patients were usually highly photosensitive on EEG. MRI showed progressive cerebral and cerebellar atrophy. The median survival time was 26 years from disease onset. Ultrastructural examination of the pathology revealed fingerprint profiles as the characteristic inclusions, but they were not reliably seen in tissues other than brain. Curvilinear profiles, which are seen in the late infantile form, were not a feature. Of the 13 unrelated families we observed homozygous CLN6 pathogenic variants in four and compound heterozygous variants in nine. Compared to the variant late infantile form, there was a lower proportion of variants that predicted protein truncation. Certain heterozygous missense variants in the same amino acid position were found in both variant late infantile and Kufs disease. There was a predominance of cases from Italy and surrounding regions; this was partially explained by the discovery of three founder pathogenic variants. Clinical distinction of type A (progressive myoclonus epilepsy) and type B (dementia with motor disturbance) Kufs disease was supported by molecular diagnoses. Type A is usually caused by recessive pathogenic variants in CLN6 or dominant variants in DNAJC5. Type B Kufs is usually associated with recessive CTSF pathogenic variants. The diagnosis of Kufs remains challenging but, with the availability of genetic diagnosis, this will largely supersede the use of diagnostic biopsies, particularly as biopsies of peripheral tissues has unsatisfactory sensitivity and specificity.

Purpose Lennox-Gastaut syndrome (LGS) is a severe childhood-onset epileptic encephalopathy characterized by the presence of multiple types of intractable seizures, cognitive impairment, and specific electroencephalogram (EEG) patterns. The aim of this study was to investigate the electroclinical features of patients with LGS during adulthood. Methods We retrospectively identified the medical records of 20 patients aged over 18 years with a diagnosis of LGS from 3896 patients with epilepsy. Results Thirteen (65 %) patients were male. The mean age of the patients was 23.4 ± 7.1 (min-max; 18–43) years, and the mean follow-up period was 5.6 ± 4.5 (min-max; 1–14) years. The etiology was identified in 11 (55 %) patients. None of the patients achieved seizure freedom. The most prevalent seizure types were atypical absences in 14 (70 %) patients, tonic seizures in 13 (65 %) patients, and atonic seizures in 11 (55 %) patients. One (5 %) patient was diagnosed as having psychogenic non-epileptic seizures. Mental retardation was reported in all patients and only half of them could walk independently. All patients had drug-resistant seizures and 16 (80 %) patients were on polytherapy. A vagus nerve stimulator was implanted into ten (50 %) patients and five reported 50–80 % seizure reduction. Ketogenic diet was administered to two (10 %) patients and epilepsy surgery was performed in two (10 %) patients with no significant benefit. Conclusions Paying attention to all factors of seizure outcomes, cognitive impairment, and ambulatory status, all patients were dependent on caregivers for daily living abilities. LGS has life-long persistence with poor outcomes, even during adulthood.

Simultaneous central nervous system (CNS) infection with Cryptococcus and tuberculosis (TB) is very rare. Despite improved therapeutic options, treatment of CNS cryptococcosis is still difficult and needs invasive treatment modalities, such as intrathecal or intraventricular amphotericin B, in refractory cases. We describe a patient with systemic lupus erythematosus diagnosed with simultaneous cryptococcal and TB meningitis who had a poor response to intravenous liposomal amphotericin B and fluconazole, but was successfully treated with intraventricular amphotericin B, in addition to anti-TB therapy.

Objective: We aimed to identify the possible causes of myoclonus and related electrophysiological features in a cohort of young patients. Patients and Methods: We performed a retrospective analysis of all clinical and electrophysiological recordings of patients who had myoclonus and were under 60 years between 2005 and 2018. The clinical data included age at examination, gender, age at onset, and main neurological or systemic findings, underlying cause and electrophysiological features constituted surface electromyogram, long-loop reflexes, electroencephalography, and somatosensory-evoked potentials. Results: In the study period, we identified 155 patients with myoclonus. Myoclonus was most commonly related with epilepsy and movement disorders. Myoclonus with epilepsy was the leading cause between 10 and 30 years, whereas myoclonus with movement disorders was more common after 30 years. In our country, SSPE was an important cause of myoclonus under the 20 years of age. We identified cortico-subcortical subtype followed by cortical, cortical reflex, subcortical-basal ganglia, subcortical-brainstem and propriospinal subtypes, and correlated these subtypes with relevant disorders. Conclusion: The electrophysiological classification provides anatomical subtyping, which is favorable in diagnosing certain cases such as brainstem and propriospinal myoclonus. Certain characteristics such as reflex nature of myoclonus and accompanying features narrow the list of differentials and help in making the diagnosis.

Reflex epilepsies (REs) are identified as epileptic seizures that are consistently induced by identifiable and objective-specific triggers, which may be an afferent stimulus or by the patient’s own activity. RE may have different subtypes depending on the stimulus characteristic. There are significant clinical and electrophysiologic differences between different RE types. Visual stimuli-sensitive or photosensitive epilepsies constitute a large proportion of the RE and are mainly related to genetic causes. Reflex epilepsies may present with focal or generalized seizures due to specific triggers, and sometimes seizures may occur spontaneously. The stimuli can be external (light flashes, hot water), internal (emotion, thinking), or both and should be distinguished from triggering precipitants, which most epileptic patients could report such as emotional stress, sleep deprivation, alcohol, and menstrual cycle. Different genetic and acquired factors may play a role in etiology of RE. This review will provide a current overview of the triggering factors and management of reflex seizures.

ObjectiveResective surgery is effective in treating drug-resistant focal epilepsy, but it remains unclear whether improved diagnostics influence postsurgical outcomes. Here, we compared practice and outcomes over 2 periods 15 years apart.MethodsSixteen European centers retrospectively identified 2 cohorts of children and adults who underwent epilepsy surgery in the period of 1997 to 1998 (n = 562) or 2012 to 2013 (n = 736). Data collected included patient (sex, age) and disease (duration, localization and diagnosis) characteristics, type of surgery, histopathology, Engel postsurgical outcome, and complications, as well as imaging and electrophysiologic tests performed for each case. Postsurgical outcome predictors were included in a multivariate logistic regression to assess the strength of date of surgery as an independent predictor.ResultsOver time, the number of operated cases per center increased from a median of 31 to 50 per 2-year period (p = 0.02). Mean disease duration at surgery decreased by 5.2 years (p < 0.001). Overall seizure freedom (Engel class 1) increased from 66.7% to 70.9% (adjusted p = 0.04), despite an increase in complex surgeries (extratemporal and/or MRI negative). Surgeries performed during the later period were 1.34 times (adjusted odds ratio; 95% confidence interval 1.02–1.77) more likely to yield a favorable outcome (Engel class I) than earlier surgeries, and improvement was more marked in extratemporal and MRI-negative temporal epilepsy. The rate of persistent neurologic complications remained stable (4.6%–5.3%, p = 0.7).ConclusionImprovements in European epilepsy surgery over time are modest but significant, including higher surgical volume, shorter disease duration, and improved postsurgical seizure outcomes. Early referral for evaluation is required to continue on this encouraging trend.

Objective Hippocampal sclerosis (HS) is the most common pathological substrate associated with mesial temporal lobe epilepsy (MTLE), where inflammatory processes are known to play an increasingly important role in the pathogenesis. To further investigate the role of the immune system, both cytokine gene polymorphisms and human leukocyte antigen (HLA) genotyping in patients with MTLE-HS were investigated. Methods The DNA samples of 100 patients with MTLE-HS and 201 healthy individuals were genotyped for cytokines (IL-6,IL-10, TNF-α, TGF-β1 and IFN-γ) and HLA using polymerase chain reaction (PCR)-SSP and SSO methods. The results were statistically analyzed in patient and healthy control groups and then according to the presence of febrile seizures (FS) in the patient group. Results Analysis of cytokine genotyping did not reveal any significant difference between patients with MTLE-HS and controls and patients with or without FS. However, the HLA DRB1*13 allele was found to be more frequent in the patient population after Bonferroni correction. Conclusion This study suggests the possible role of HLA in the pathogenesis of MTLE-HS, although it failed to show any relationship with the cytokine system. However, data regarding the role of HLA are still lacking, and further studies are necessary to verify our results.

Recurrent, spontaneous seizure activity caused by abnormal neuronal firing in the brain is a hallmark of epilepsy, a common chronic neurological disorder. Recent research that has expanded the knowledge of the cellular and molecular mechanisms that modulate neuronal excitability and network activity in the brain provides the development and discovery of antiepileptic drugs (AEDs). AED therapy starts with the selection of the most appropriate drug for individual patient according to a strategic decision based on the risk-benefit ratio. Beyond three generations of AEDs developed over the last 40 years, more effective, better tolerated, disease-modifying pharmacological therapies are still needed to improve seizure outcome and reduce the safety burden. In this review, we aim to provide a special focus on epilepsy with an overview of underlying pathophysiological mechanisms that may contribute to seizure generation and propagation. Besides, knowledge of principle pharmacological properties of current AEDs which are essential for rational drug therapy is considered. Limited safety data are available especially for recent AEDs, we also discussed their safety profiles concerning major adverse effects, teratogenicity and their use in pregnancy and lactation. Epilepsy is a chronic disorder which needs special knowledge and experience during AED treatment with meticulous follow-up of patients to avoid short and long-term adverse effects with the best possible seizure controlled outcome and high quality of life.

Background Surgical results regarding MRI-negative epilepsy were presented and related clinical and histopathological parameters were discussed. Methods Thirty-six MRI-negative epilepsy patients were retrospectively analyzed. Histopathological specimens were re-reviewed by 2 blind neuropathologists and re-classified based on the current classifications. Results The mean age at surgery and seizure onset was 24.5 years and 9.3 years, respectively. Eight patients were younger than 18 years. Mean duration of seizures was 15.3 years. All but 2 underwent invasive monitorization. Eighteen patients had hypometabolism on FDG-PET with temporal lobe involvement in majority (66.7%). Hypometabolism was found in all patients with hippocampal sclerosis (HS), which was present in 50% and 66.7% of focal cortical dysplasia (FCD) type I and II patients, respectively. The frontal lobe resection was the most frequent type of operation followed by resections in temporal, parietal and occipital lobes. In 7 patients, multilobar resection was performed. Histopathological diagnosis was FCD type I, II, III, HS, and gliosis in 14, 12, 2, 3 and 2 patients, respectively. The mean follow-up was 5.8 years. Seventeen patients were seizure free and favorable outcome (Engel's I and II) was found in 69.7%. FCD type I tend to have more favorable seizure outcome. Duration of epilepsy and hypometabolism on FDG-PET was significantly related to outcome, whereas involved lobe was not. Conclusions Our results suggest it is worth pursuing resective surgery in adults as well as in children with drug-resistant epilepsy with normal MRI.

Purpose The present study aimed to determine if the specific characteristics of fluorodeoxyglucose-positron emission tomography (FDG-PET) analyses of the FCD subgroups were compatible with the magnetic resonance imaging (MRI) and clinical findings of the patients in these subgroups. Methods This study included 71 patients who had a presurgical evaluation workup performed due to drug-resistant seizures, who underwent epilepsy surgery, and who were histopathologically diagnosed with FCD. Relationships involving MRI and FDG-PET findings and clinical data from pathological subgroups and patients were assessed. Results According to the International League Against Epilepsy (ILAE) classifications of FCD, 28 of the patients were type I and 43 were type II. FCD was visible on the MRI scans of 53 patients, and a majority of this group was classified as type II FCD (n=34). Of these 53 patients, FCD was located in the temporal area of 21 patients, the extratemporal area of 29 patients. Of the patients who exhibited FDG-PET hypometabolism (PET-positive), 23 were classified as temporal, 17 as frontal, 11 showed involvement of the posterior cortex. The age of seizure onset was younger in PET-positive patients (p=0.032), and histopathological analyses revealed that 23 patients had type I FCD and 30 patients had type II FCD. Conclusion PET scans reveal a lesion by showing hypometabolism in patients who have refractory epilepsy and an early age of onset with FCD. The lesions of MRI-negative/PET-positive FCD patients tend to be localized in the temporal lobe and that FCD may be localized in the frontal lobe of MRI-negative/PET-negative patients. However, the histopathological examinations of MRI-positive/PET-positive, MRI-negative/PET-positive, and MRI-negative/PET-negative patients did not exhibit a particular histopathological subtype.

Phenytoin has a widespread use in epilepsy treatment and is mainly metabolized by hepatic cytochrome P450 enzymes (CYP). We have investigated CYP2C9*2, CYP2C9*3, CYP2C19*2 and CYP2C19*3 allelic variants in a Turkish population of patients on phenytoin therapy. Patients on phenytoin therapy (n = 102) for the prevention of epileptic seizures were included. Polymorphic alleles were analyzed by restriction fragment length polymorphism method. Serum concentrations of phenytoin were measured by fluorescence polarization immune assay method. The most frequent genotype was detected for CYP2C9 wild-type alleles (78.43 %), whereas CYP2C19*2/*2 (5.88 %) was the least frequent genotype group. According to the classification made with both enzyme polymorphisms, CYP2C9*1/*1-CYP2C19*1/*1 (G1: 41.17 %) genotype group was the most frequent whereas CYP2C9*1/*2-CYP2C19*1/*3 (G7: 0.98 %) was the least frequent one. The highest mean phenytoin level (27.95 ± 1.85 µg/ml) was detected in the G8 genotype group (CYP2C9*1/*3-CYP2C19*2/*3) and the G1 genotype group showed the lowest mean phenytoin level (7.43 ± 0.73 µg/ml). The mean serum concentration of phenytoin of the polymorphic patients with epilepsy was higher than that for the wild-type alleles both in the monotherapy and polytherapy patients. These results show the importance of the genetic polymorphism analysis of the main metabolizing enzyme groups of phenytoin for the dose adjustment.

OBJECTIVE: Vagal Nerve Stimulation (VNS), is an adjunctive therapy approved for use in patients with refractory epilepsy, based on stimulation of the exctracranial part of the vagus nerve. In this study, we provide an analysis of seizure outcomes after VNS implantation for pharmacologically resistant epilepsy patients. METHODS: We reviewed all patients who had VNS implantation in our center from 2005 to June 2013. Patient outcomes were evaluated using the VNS-specific outcome scale. RESULTS: This study consisted of 35 patients; 24 male (68.57%) and 11 females (31.42%). The mean age of patients was 13.7 years + 8.2 (7-40 years). The mean age at VNS implantation was 17.17 years + 8.4 (4- 35 years). The mean duration of VNS therapy was 26 months + 19.2 (3- 84 months). The 80% of our patients were in the responders, 20% of them were in the nonresponders group. CONCLUSION: Vagal nerve stimulation, is an alternative treatment approach. Given the potential for cognitive, behavioural improvement with or without improved seizure control, VNS may be considered in the course of treatment of refractory epilepsy.

This study is based on the psychodynamic and neurological analysis of three Turkish patients who displayed ictal kissing automatism during their seizures. To unveil the probable underlying causes of their kissing behaviors, all patients underwent psychiatric interviews after being evaluated by ictal video-EEG recordings. The group consisted of two females (ages 35 and 29) and one male (age 26). In addition to prominent oral automatisms, each patient also displayed behaviors of kissing or blowing kisses to individuals at close proximity. Seizures were related to the right temporal lobe in two patients and the left temporal lobe in one patient. Magnetic resonance imaging showed mesial temporal sclerosis in two of the patients (one left, one right) and was normal in one. According to the DSM-IV-TR criteria, each of the three patients also suffered from major depression, while the psychodynamic interviews revealed traumatic childhood histories and intense unfulfilled affective needs. (C) 2013 Elsevier Inc. All rights reserved.

Epilepsy is relatively uncommon in patients with Neurofibromatosis Type 1 (NF1) and seizures are usually well controlled with antiepileptic treatment. However, pharmacoresistance has been reported in patients with NF1 and MRI evidence of malformations of cortical development or glioneuronal tumours. Available information on epilepsy surgery in NF1 is limited to a few patients with gliomas and glioneuronal tumours who underwent lesionectomies. We conducted a survey amongst 25 European epilepsy surgery centres to collect patients with NF1 who had undergone surgery for drug-resistant seizures and identified 12 patients from eight centres. MRI abnormalities were present in all patients but one. They were unilateral temporal in eight, bilateral temporal in one and multilobar or hemispheric in two. Seizures originated from the temporal lobe in ten patients, from the temporo-parieto-occipital region in one, and were bitemporal in one. One year after surgery eight patients were seizure free, one had worthwhile improvement and the remaining three had experienced no benefit. Postoperative outcome, available at 2 years in ten patients and at 5 years in three, remained stable in all but one whose seizures reappeared. Histology revealed dysembryoplastic neuroepithelial tumour (DNET) in five patients, hippocampal sclerosis in four, mixed pathology in one and polymicrogyria in one. No histological abnormality was observed in the remaining patient. Epilepsy surgery can be performed effectively in patients with NF1 provided a single and well-delimited epileptogenic zone is recognized. The high prevalence of DNETs in this series might suggest a non-fortuitous association with NF1.

Although there have been a number of psychotherapy trials for chronic psychogenic nonepileptic seizures, evidence-based treatment options are limited. We developed an eclectic group psychotherapy which combines psychoeducation and behavioral and psychoanalytic techniques. Nine patients completed 12 weeks of psychotherapy. Patients were interviewed with SCID-I. They also filled in the following measures at the beginning and end of the therapy: Beck Depression Inventory, Dissociative Experiences Scale, Spielberger State-Trait Anxiety Scale, SF-36 Life Quality Scale, and Toronto Alexithymia Scale. Seizure frequency was assessed before and after the therapy and on follow-up visits at the fourth, sixth, ninth, and twelfth months. After one year of follow-up, the decrease in seizure frequency was highly significant (p