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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.

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.

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.

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

The purpose of this cross-sectional study was to explore the attitudes of individuals with epilepsy (IWE) toward their condition via self-reported changes in the feelings/thoughts about having epilepsy. We aimed to investigate whether individuals with epilepsy perceive a change in their initial attitudes toward the condition over time. Additionally, if a change was perceived, the variables which predict the current thoughts/feelings toward epilepsy were also examined. Seventy adult IWE participated in the study. Of the 70 participants in the study, 25.7% retained their initial feelings/thoughts without any change. Among the IWE who reported no-change, the most common category was "negative from the beginning". On the other hand, 74.3% of the participants reported a change. Of the 74.3% of participants reporting change, the most common direction was from negative to positive" (79.2%) seeming to indicate a positive adjustment over the passage of time. In a logistic regression, only three factors were found to predict positive feelings/thoughts toward epilepsy: a clear understanding of the condition, low levels of depression and current employment status. Finally, there is a discussion of the potential applications of the IWE' self-assessment of their attitudes.

Lamotrigine (LTG) which has a widespread use in epilepsy treatment as an antiepileptic agent is metabolized by UDP-glucuronosyl transferase (UGT) enzymes. In this study, single nucleotide polymorphisms, P24T and L48V, of the UGT1A4 enzyme have been investigated in a Turkish population of patients with epilepsy (n=131) by comparing serum levels of LTG of wild type and polymorphic subjects. High performance liquid chromatography (HPLC) was used to measure serum concentrations of LTG. The P24T and L48V polymorphisms of the UGT1A4 enzyme were analyzed with a matrix assisted laser desorption-time of flight (MALDI-TOF) mass spectrometry method. The frequencies of the heterozygous alleles for L48V or P24T polymorphisms were 22.4% and 3.8%, respectively. L48V polymorphism was found to decrease the serum concentration of LTG in patients on monotherapy or polytherapy. The LTG levels of non smoking monotherapy patients were 52% lower for the L48V polymorphism than for wild type alleles. Also the LTG levels were significantly lower for non smoking or smoking polymorphic alleles than for normal. The high frequency of the L48V polymorphism detected in the Turkish population indicates that LTG dose adjustments in patients with the UGT1A4 L48V polymorphic enzyme should be taken into account.

Introduction Long latency reflexes (LLRs) are medium to late latency responses which are obtained over a muscle after a peripheral stimulation. In epilepsy, presence and amplitudes are supposed to be higher due to increased cortical excitability. We aimed to analyze LLRs in juvenile myoclonic epilepsy (JME) and progressive myoclonic epilepsy (PME), to determine the frequency of three subtypes and to analyze whether parameters regarding LLRs may be used in differentiating these two disorders. Our hypothesis was cortical excitability and amplitudes and frequency of LLRs would be higher in PME. Methods We included 30 patients with JME, 18 patients with PME and 28 healthy subjects. LLRs were recorded over abductor policis brevis muscle after stimulating median nerve at wrist during rest and active movement. Latencies and amplitudes of segmental reflex and LLRs, presence of LLR I, II, and III, the ratio of segmental reflex amplitude and LLR amplitude were compared among three groups. ROC curves were formed for LLR I amplitude and amplitude ratio to evaluate ‘cut-off’ values in differentiating PME and JME. Results C reflex was detected in only PME group and LLR I presence during active movement was the highest in PME group (57.9%, p = 0,001). Although LLR I amplitude was also the highest in PME group, ratio was higher in JME group since amplitude of segmental reflex was also higher in patients with PME. Both parameters were higher in PME and JME groups compared to healthy subjects (LLR I amplitude: p = 0.005 and ratio: p = 0.011, Kruskal–Wallis test). LLR I amplitude was able to differentiate patients with epilepsy and healthy subjects with an accuracy of 57–82% (p = 0.008) whereas ratio was able to recognize patients with an accuracy of 60–86% (p = 0.003). Ratio above 1.05 had the sensitivity and specificity of 77.8% and 71%, respectively in diagnosing patients with PME or JME. Conclusion Presence of C reflex and higher LLR I amplitudes suggest the presence of exaggerated cortical response to peripheral stimuli in PME. However, cortical excitability measured by LLR I amplitude is high in both JME and PME and hence does not provide differentiation of these two disorders.

Uzan, Mustafa/0000-0001-7214-380X; Sanus, Galip Zihni/0000-0001-6539-7254 WOS: 000249375100004 PubMed: 17482840 Recent arouse of interest indicated that drug resistant proteins are markedly over-expressed in the epileptogenic tissue and they may be responsible for the one-third of the epileptic patients who were refractory to anti-epileptic drugs (AEDs). Since several AEDs may act as substrates for these drug resistant proteins, the enhanced function of such proteins may increase drug extrusion, resulting in inadequate response to drug therapy in patients with epilepsy. We studied expression of the muttidrug resistance protein 1 (MDR1) and muttidrug resistance-associated protein 1 (MRP1) in the epileptic tissues resected surgically in 28 patients with focal cortical dyspLasia (FCD) by immunohistochemistry. The results were compared with 10 normal necropsy brain tissues. Normal brain showed no MDR1 expression in neurons and astrocytes, while MRP1 expression was very weak, which were encountered in a few samples. MDR1 expression was mainly localized on the vascular endothelial cells. In contrast to normal brain, we found intense MDR1 and MRP1 expression in both neurons and reactive astrocytes in the vast majority of dysplastic tissues. The majority of the dysplastic neurons demonstrated moderate to strong MRP1 immunoreactivity. Endothelial cells showed both MDR1 and MRP1 expression in the majority of the specimens studied. Muttidrug transporters are over-expressed in the epiteptogenic zone in patients with FCD. These results are concordant with previous studies, in which over-expression of multidrug proteins were shown in epiteptogenic brain tissue in patients with FCD, that the over-expression of drug transport proteins in tissue from patients with refractory epilepsy may explain one possible mechanism for drug resistant in these pathologies. (c) 2007 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is one of the most common medically intractable epilepsy syndromes and the pathogenesis of HS remains highly obscure. Recent studies demonstrated controversial results about the relationship between interleukin (IL) gene polymorphism and epilepsy in different ethnic groups. This correlation was investigated in Turkish patients with MTLE-HS. The allele distribution of IL-1alpha and IL-1beta in 47 patients of Turkish ancestry was determined and compared with 99 ethnically matched control subjects. Analysis of genotype frequencies between patients and controls showed no statistically significant difference (p0.05). Our data suggest that IL-1alpha and IL-1beta gene polymorphisms do not act as a strong susceptibility factor for MTLE-HS in individuals of Turkish ancestry.

This study was performed to investigate some clinical parameters of febrile seizures (FSs) in patients with epilepsy, testing any possible correlation between those parameters and the type of subsequent epilepsy. One hundred and nine patients with epilepsy having a history of FSs were evaluated for age at onset of FSs, interval between first FS and first afebrile seizure, recurrence rate, type of FSs, incidence of febrile status, family history for epilepsy and for FSs and the neurological status of the patient. The epilepsy that developed subsequently, were classified as generalised versus partial and also according to their syndromic subgroups. In temporal lobe epilepsy with mesial temporal sclerosis (TLE–MTS), statistical analyses revealed a younger age at onset of FSs, and a high incidence of episodes of febrile status and of complex FSs. Clinical characteristics of FSs followed by partial epilepsies were younger age at onset, presence of focal features and of febrile status, longer interval between the first FS and the first afebrile seizure, and a high incidence of FSs in the family history. In generalised epilepsies, however, a shorter interval between the first FS and the first afebrile seizure, a high incidence of single FS and of a family history of epilepsy were predominating characteristics. Results suggest that certain features of FSs may be predictive of a particular type of subsequent epilepsy.

Panayiotopoulos syndrome is a common benign epilepsy affecting otherwise healthy children that present with autonomic seizures, in which nausea, retching, and vomiting are particularly common and prominent. Because of the unusual ictal symptoms and lengthy manifestations, misdiagnosis is a common major problem. We describe a young girl with intractable and lengthy vomiting attacks, several admissions to hospitals, and extensive gastroenterological workup for several years from early childhood. On all previous occasions the diagnosis varied from psychosomatic disease, to functional dyspepsia, to cyclic vomiting syndrome. The possibility of autonomic epileptic seizures and Panayiotopoulos syndrome, though likely, was not considered.

WCN 2013 No: 1069 Topic: 1 — Epilepsy Autosomal recessive cortical myoclonic tremor and epilepsy: Association with a mutation in the potassium channel associated gene CNTN2 E. Stogmann. Department of Clinical Neurology, Medical University of Vienna, Vienna, Austria We characterize a consanguineous Egyptian family with an autosomal recessively inherited familial corticalmyoclonic tremor and epilepsy.We usedmultipoint linkage analysis tomap the causative mutation to a 12.7 megabases interval within 1q31.3–q32.2 with a LOD score of 3.6. For further investigation of the linked region in an efficient and unbiased manner, we performed exome sequencing. Within the suspected region we identified a homozygous single base pair deletion (c.503_503delG) leading to a frameshift in the coding region of the 6th exon of CNTN2 alias TAG-1 (p.Trp168fs), which segregated in the respective family. Many studies point towards an important role of the CNTN2 product Contactin2 in neuronal excitability. Contactin-2, a glycosylphosphatidylinositolanchored neuronal membrane protein, and another transmembrane protein called Contactin-associated protein-like 2 are together necessary to maintain voltage gated potassium channels at the juxtaparanodal region. CNTN2 knockout mice were previously reported to suffer from spontaneous seizures and mutations in the gene encoding Contactinassociated protein-like 2 (CNTNAP2 alias CASPR2) have been described to cause epilepsy in humans. To further delineate the role of CNTN2 in epilepsy patients, we sequenced the coding exons in 189 Caucasian epilepsy patients. No recessivemutationwas detected, and heterozygote carriers of rare CNTN2 variants do not seem to be predisposed to epilepsy. Given the severity of themutation and the proposed function of the gene, we consider this mutation as the most likely cause for cortical myoclonic tremor and epilepsy in this family. doi:10.1016/j.jns.2013.07.076 Abstract — WCN 2013 No: 1041 Topic: 1 — Epilepsy Subjective handicap in people with epilepsy in Georgia and Turkey WCN 2013 No: 1041 Topic: 1 — Epilepsy Subjective handicap in people with epilepsy in Georgia and Turkey N. Gzirishvili, E. Togonidze, G. Lomidze, C. Ozkara, G. Gozubatik, S. Kasradzea, A. Tsiskaridzec, J.W. Sander. Institute of Neurology and Neuropsychology, Tbilisi, Georgia; Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey; Tbilisi State University, Tbilisi, Georgia; Department of Clinical and Experimental Epilepsy, UCL Institute of Neurology, Queen Square, London, UK; SEIN — Epilepsy Institute in The Netherlands Foundation and WHO Collaborating Centre for Research, Training and Treatment in Epilepsy, Hoofddorp, Netherlands Antilles Background: People with epilepsy have an impaired quality of life due to the impact of the condition on various domains of their life. Objective: To assess epilepsy-related subjective handicap in people with epilepsy in two neighboring countries: Georgia and Turkey. Methods: We distributed self-administrated questionnaires to consecutive people with epilepsy taking antiepileptic drugs attending outpatient clinics at I.U. Cerrahpasa Medical Faculty in Istanbul and at the Institute of Neurology and Neuropsychology in Tbilisi. The SHE questionnaire was used which contains 32 items in six domains. Low score indicates higher handicap. Probabilities of b0.05 were considered as significant. Results: A total of 200 people took part (equal number at each site). Mean age was 31.7 years.62% of responders were female. 24% had completed higher education. 27% had a “Work and activities” score below 50 points, 13% for “Social and personal”, 28% for “Self perception”, 30% for “Physical”, 19% for “Life-satisfaction” and 23% for “Change” domains. In ‘Soc. personal’ (p = 0.003), ‘Life satisfaction’ (p = 0.042) and ‘Change’ (p = 0.006) subdivisions mean rank among people in Georgia were significantly lower except in “Social and personal” domain in which the Georgian cohort had higher scores. Association was found between social class and subdivision variables such as ‘Physical’ (p = 0.036), ‘Self perception’ (p = 0.001), ‘Life satisfaction’ (p= 0.001) and ‘Change’ (p b 0.001). People in lower social strata had lower scores. Conclusion: Epilepsy imposes a high social burden on people in both countries with people with epilepsy in the lower strata of society may have a high burden. doi:10.1016/j.jns.2013.07.077 Abstract — WCN 2013 No: 1091 Topic: 1 — Epilepsy Status epilepticus in glioma WCN 2013 No: 1091 Topic: 1 — Epilepsy Status epilepticus in glioma K.M. Knudsen-Baas, J.F. Owe, S.E. Hegrestad, B.A. Engelsen, A.M. Storstein. Neurology, Haukeland University Hospital, Bergen, Norway; Neurology, Sentralsjukehuset i Sogn og Fjordane, Forde, Norway Background: Symptomatic epilepsy is common in glioma, especially in low grade glioma. There are few studies of status epilepticus (SE) in glioma, but they are assumed to be relatively infrequent and usually at the time of diagnosis or progression. We wanted to look at the semiology, relation to cancer disease, treatment response and recurrent SE in this population. Material and methods: Patients with glioma WHO grades II–IV and ≥1 epileptic seizure at the time of diagnosis or later, have since 2009 been included in a prospective clinical observational study at Haukeland University Hospital. N = 61 per 04.03.2013. SE is defined as seizure N30 min or repeated seizures without restitution. Results: We identified 17 SE in 13 glioma patients. Nine patients had glioma grade IV, one patient grade III and three patients grade II. The semiology of 11/17 SE was secondary generalized. 9/17 SE occurred within six months of diagnosis. In half of the cases, SE was onset symptom or sign of progression. In 10/17 antiepileptic drugs (AEDs) were already in use at the time of SE. Intravenous diazepam was sufficient in 10/17 SE. Midazolam was given in 2/17, one AED in 3/17 and two AEDs in 2/17, in addition to diazepam. General anesthesia was not discussed. Recurrent SE was seen in 4 patients. Conclusion: SE was more frequent in high grade glioma and the seizures were secondary generalized. Half of SE occurred in early phase of cancer disease. All SE responded to first or second line treatment. Most patients had not recurrent SE. doi:10.1016/j.jns.2013.07.078 Abstracts / Journal of the Neurological Sciences 333 (2013) e1–e64 e19