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Abstract Objective The aim of the study is to evaluate the complications and long-term bowel function in Hirschsprung’s disease (HD) patients following total transanal endorectal pull-through (TERPT) surgery operated in a tertiary hospital in Oman. Methods A retrospective data retrieval was done for patients diagnosed with Hirschsprung’s disease and underwent total transanal endorectal pull-through procedure during the period from December 2008 to June 2019 with concomitant prospective phone call questionnaire interview to assess the long-term functional outcome of children aged 3 years and older. Results Total transanal endorectal pull-through was performed in 61 patients. The mean age at the time of operation was 7.71 ± 8.26 months. The most common late complication was anal excoriation (19.7%). Of the patients, 61.5% had a normal to fair score, and the remaining 38% had a poor score in their functional outcome questionnaire. The majority had no social problems (65.4%), while 15.4% had problems causing varying degrees of social restrictions. Conclusion The short-term outcomes of total transanal endorectal pull-through are found to be acceptable. In addition, the long-term functional outcome post total transanal endorectal pull-through was found to be satisfactory; however, longer follow-up studies with larger sample sizes are needed for more accurate functional outcome evaluation.

Gastrointestinal basidiobolomycosis (GIB) is a rare fungal infection with few reported cases worldwide. We report here the first case diagnosed in Oman in a previously healthy 5-year-old Omani female child who had been thought initially to have an abdominal malignancy. The case was referred to the Royal Hospital, Muscat, Oman, in July 2012. She was treated successfully with surgical resection and prolonged antifungal therapy (voriconazole). Physicians, including clinicians, radiologists and pathologists, should have a high index of suspicion for GIB when a patient presents with an abdominal mass and fever.

Delayed herniation of the abdominal contents through a congenital diaphragmatic hernia may occur beyond the neonatal period. This report describes a 9-week-old female baby who presented with excessive crying, irritability and respiratory distress secondary to late presentation of left-sided congenital diaphragmatic hernia. The chest radiograph showed tension gastrothorax. She underwent surgical reduction of the hernia. She made an excellent recovery and was discharged a few days after the operation. It is assumed that sudden increase of the intra-abdominal pressure caused herniation of abdominal content through a pre-existing diaphragmatic defect. This report aims to increase the awareness of this condition among physicians and pediatric surgeons to allow early diagnosis and management.

Objective: Typical semiology with associated 5As (Antecedent, Aura, Arrest, Automatisms, and Amnesia) is the prototypical seizure manifestation of temporal lobe epilepsy (TLE) in adults. However, patients with TLE can present with seizure phenomenology referred to as "hypermotor TLE" (HMS-TLE) which can mimic extratemporal focal epilepsy. We studied the clinico-electrographic, imaging profile, and surgical outcomes of drug-resistant HMS-TLE patients., Methods: We analyzed the ictal signs and electroencephalography data of HMS-TLE patients who became seizure free following epilepsy surgery with a minimum follow-up of 1 year and compared them with patients with typical TLE (1:2 ratio). Hypermotor seizures were identified based on the presence of stereotyped ictal complex movements at seizure onset including body rocking, pelvic thrusting, and bimanual and bipedal movements with/without affective components., Results: Out of 684 patients with drug-resistant TLE who underwent surgery during 2010-2020, 16 patients (2.34%) met criteria for HMS-TLE and were compared with 32 patients with typical TLE. Predominant "hypermotor" components included motor agitation, bicycling/kicking movements, body gyration followed by violent automatisms, and pelvic thrusting. Age at epilepsy onset, age at video telemetry recording, age at surgery, duration of epilepsy at surgery, gender distribution, and presence of aura were comparable between the two groups. History of febrile seizures (31.3% vs. 65.6%; p-value = .024), presence of hippocampal sclerosis on MRI, and histopathology (56.3% vs. 90.6%; p-value = .006 and 50% vs. 96.9%; p-value = .002, respectively) were lower, and temporal neocortical abnormalities on MRI (87.5% vs. 59.4%, p-value = .048), dysplasia on histopathological evaluation (25% vs. 0%, p-value = .003) were higher in HMS-TLE. HMS-TLE group had lower number of patients with focal-onset ictal patterns (37.5% vs. 90.6%, p-value = .0001)., Significance: Hypermotor seizures are rare in TLE, however it does not preclude non-invasive selection for surgery. Hence, physicians should be aware of this subtype of drug-resistant TLE as early intervention can have good surgical outcomes, akin to typical TLE., (© 2025 International League Against Epilepsy.)

Purpose: The occurrence of new seizure(s) in older adults is two- to three-fold higher as compared to the younger population, and previous studies have characterized the clinical features of seizures in patients beyond 60 or 65 years. This study, in response to the recent ILAE task force on epilepsy in the elderly (2023), has assessed the clinical characteristics of individuals who are over 85 years old., Methods: Data was collected from prospectively maintained electronic medical records, and the participants were divided into seizure-free and seizure-recurrence groups, and their clinical characteristics were compared., Results: Over a 30-year period, a single comprehensive epilepsy care center included a total of 40 patients, with a mean age of 88.7 ± 2.79 years - most common seizure type was generalized tonic-clonic seizures (45%), with a positive neuroimaging yield of 42.5% and an EEG showing specific abnormalities in 50% of cases. Etiology was unidentified in 50%, while stroke was the most common among identified etiologies (27.5%). Over a mean follow-up of 1.4 [0.2-5.8] years, monotherapy effectively controlled 62.5% of patients, but 47.5% experienced recurrence. When compared to the non-recurrence group, these patients showed significant risk factors such as a history of prior stroke [47.3% versus 23.8%, p = 0.031], use of phenytoin [68.4% versus 33.3%, p = 0.056], and clobazam [42.1% vs. 19%, p = 0.049]., Conclusion: This study contributes to better identification and improved characterization of late-onset seizures and adds that appropriate, timely management results in better outcomes., Competing Interests: Declarations. Conflict of interest: On behalf of all the authors, the corresponding author states that there are no conflicts of interest. Ethical approval: This study was conducted after obtaining institutional ethical clearance—SCT/IEC/1780/FEBRUARY – 2022. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Patient consent: N/A., (© 2024. The Author(s), under exclusive licence to European Geriatric Medicine Society.)

Background: The role of imaging in autoimmune encephalitis (AIE) remains unclear, and there are limited data on the utility of magnetic resonance imaging (MRI) to diagnose, treat, or prognosticate AIE., Purpose: To evaluate whether MRI is a diagnostic and prognostic marker for AIE and assess its efficacy in distinguishing between various AIE subtypes., Material and Methods: We analyzed data from 96 AIE patients from our prospective autoimmune registry. MRI sequences examined were FLAIR, diffusion, SWI, T2WI, ASL, and contrast enhancement. Short-term outcomes were measured using the Modified Rankin Scale (mRS) at discharge; long-term outcomes were assessed with the Functional Independence Measure (FIM) at 6 months., Results: MRI confirmed AIE in cases of new-onset seizures (82.1%, P  < 0.001) and dementia (100%, P  = 0.02). Antibody-negative AIE exhibited significant multifocal FLAIR abnormalities compared to antibody-positive cases ( P  = 0.002). LGI1 and CASPR2 encephalitis frequently involved the mesial temporal region ( P  = 0.004), while ASL revealed hyperperfusion of the contralateral basal ganglia in faciobrachial dystonic seizures ( P  = 0.016). GAD65 encephalitis predominantly affected the cerebellum ( P  = 0.002), and NMDA encephalitis showed contrast enhancement in five cases ( P  = 0.045). MRI was not useful for predicting short-term outcomes but was associated with long-term outcomes; specifically, a normal MRI was linked to a better long-term outcome in 47.8% of patients ( P  = 0.035), and resolution of abnormalities correlated with a favorable FIM score (>54) in 76.7% ( P  = 0.016)., Conclusion: MRI is valuable for early detection of seizures or dementia as initial manifestations of AIE and for differentiating AIE subtypes. Follow-up MRI is significant in predicting long-term outcomes., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Surgically remediable epilepsy of the eloquent brain poses the added challenge of preserving function while curing disease. Long-standing epileptogenic lesions have tenacious seizure networks and significant functional reorganizations. Large multilobar lesions may involve multiple functional areas, thereby challenging the limits of functional brain mapping. In this video, the authors describe a dominant hemisphere fronto-parieto-temporo-occipital malformation of cortical development, involving the language, sensorimotor, and visual cortices, placing multiple eloquent cortical and subcortical regions at risk. They illustrate the technique of hexamodal mapping/monitoring involving the language sensorimotor areas, optic radiation, auditory pathway, and ictal irritative zone for a multilobar resection, with good seizure outcome and function preservation. The video can be found here: https://stream.cadmore.media/r10.3171/2024.10.FOCVID24111., Competing Interests: Disclosures Dr. Thomas reported grants from DST, Government of India; and ICMR, Government of India, outside the submitted work.Dr. Thomas reported grants from DST, Government of India; and ICMR, Government of India, outside the submitted work., (© 2025, The Authors.)

Electroencephalography-based (EEG) microstate analysis is a promising and widely studied method in which spontaneous cerebral activity is segmented into sub second level quasi-stable states and analyzed. Currently it is being widely explored due to increasing evidence of the association of microstates with cognitive functioning and large-scale brain networks identified by functional magnetic resonance imaging (fMRI). In our study using the four archetypal microstates (A, B, C and D), we investigated the changes in resting state EEG microstate dynamics in persons with temporal lobe epilepsy (TLE) and idiopathic generalized epilepsy (IGE) compared to healthy controls (HC). Machine learning was applied to study its feasibility in differentiating between different groups using microstate statistics. We found significant differences in all parameters related to Microstate D (fronto-parietal network) in TLE patients and Microstate B (visual processing) in IGE patients compared to HCs. Occurrence, duration and time coverage of Microstate B was highest in IGE when compared to the other groups. We also found significant deviations in transition probabilities for both epilepsy groups, particularly into Microstate C (salience network) in IGE. Classification accuracy into clinical groups was found to exceed 70% using microstate parameters which improved on incorporating neuropsychological test differences. To the best of our knowledge, the current study is the first to compare and validate the use of microstate features to discriminate between two disparate epilepsy syndromes (TLE, IGE) and HCs using machine learning suggesting that resting state EEG microstates can be used for endophenotyping and to study resting state dysfunction in epilepsy., Supplementary Information: The online version contains supplementary material available at 10.1007/s11571-024-10095-z., (© The Author(s), under exclusive licence to Springer Nature B.V. 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Objective: To identify the rate of successful antiseizure medication (ASM) withdrawal after resective surgery in patients with long-term epilepsy-associated tumors (LEATs)., Methods: A retrospective analysis (from our prospectively archived data) on the post-operative ASM profile of 123 consecutive patients who completed a minimum of 2 years after resection of LEATs for ASM-resistant epilepsy. A comparison between recurred and non-recurred groups in terms of seizure recurrence was used to identify the potential predictors of seizure recurrence whose attributes were further analyzed using univariate and multiple logistic regression analysis. Kaplan-Meier survival curves were used to study the probability of ASM freedom following surgery., Results: We attempted ASM withdrawal in 102 (82.9 %) patients. Forty-eight (47.1 %) had seizure recurrence while reducing ASM, of which 22 (21.6 %) continued to have seizures even after ASM optimisation. On univariate analysis, presence of pre-operative secondary generalized seizure(s) was the only factor associated with seizure recurrence. At a mean follow-up of 6.1 years, 72 (58.5 %) patients were seizure-free and aura-free at terminal follow-up (53 patients were off any ASM). The cumulative probability of achieving complete ASM-free status was 29 % at fourth year, 42 % at sixth year, 55 % at eighth year, and 59 % at 10th year after surgery., Conclusions: Following resective surgery for LEATs, ASM(s) could be successfully discontinued in half of the patients. About one-third of the patients may have recurrent seizures on follow-up. Presence of secondary generalized seizure(s) prior to surgery predicts seizure recurrence, whereas MRI defined completeness of resection will not. This information will help in rationalising decisions on ASM management post-resection., Competing Interests: Declaration of Competing Interest All co-authors have seen and agreed with the contents of the manuscript, the ICMJE requirements for authorship have been met. Each author certifies that the manuscript represents honest work and that the submission including the abstract is not under review in any other publication. There is no competing interest/conflict of interest. No funding sources were involved in its completion. The authors report no disclosures relevant to the manuscript. As the corresponding author, I confirm that I have full access to all the data in the study and had final responsibility for the decision to submit for publication. No medical writer or editor was involved in the creation of your manuscript., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest withrespect to the research, authorship, and/or publication of thisarticle.

Microstate analysis is a spatiotemporal method where instantaneous scalp potential topography represents the current state of the brain. The temporal evolution of these scalp topographies gives an understanding of quasi-stable periods of long-range coherence between distant electrodes, reflecting functional coordination within large-scale cortical networks. It has been proven potential in identification and characterization of neurophysiological indicators associated with neuropsychiatric conditions. Changes in microstates connected to symptoms and cognitive impairments of neuropsychiatric conditions. It is useful in the study of cognitive processes and disorders related to memory. Researchers may probe into the relationships between microstates and other cognitive processes, such as memory retrieval and encoding. This is a tool for clinicians to enhance the precision of diagnosis and inform possibilities for treatment by acquiring information regarding individual diversity in microstates could lead to tailored medical methods. Customizing treatment according to a patient's microstate patterns could improve the efficacy of treatment. The papers selected for the review span a broad-spectrum including memory related disorders, psychiatry and neurological disorders. A section in the review article has been dedicated to source localization of EEG microstates. The selection of review papers shed light on the importance and huge potential of application of EEG microstate analysis in various neuropsychological processes. The review concludes with the need for standardization of microstate analysis. It suggests the incorporation of widely accepted machine learning techniques for increasing the accuracy, reliability and acceptability of microstate analysis as reliable biomarkers for neurological conditions in the future., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Epilepsy, a chronic non-communicable disease is characterized by repeated unprovoked seizures, which are transient episodes of abnormal electrical activity in the brain. While Electroencephalography (EEG) is considered as the gold standard for diagnosis in current clinical practice, manual inspection of EEG is time consuming and biased. This paper presents a novel hybrid 1D CNN-Bi LSTM feature fusion model for automatically detecting seizures. The proposed model leverages spatial features extracted by one dimensional convolutional neural network and temporal features extracted by bi directional long short-term memory network. Ictal and inter ictal data is first acquired from the long multichannel EEG record. The acquired data is segmented and labelled using small fixed windows. Signal features are then extracted from the segments concurrently by the parallel combination of CNN and Bi-LSTM. The spatial and temporal features thus captured are then fused to enhance classification accuracy of model. The approach is validated using benchmark CHB-MIT dataset and 5-fold cross validation which resulted in an average accuracy of 95.90%, with precision 94.78%, F1 score 95.95%. Notably model achieved average sensitivity of 97.18% with false positivity rate at 0.05/hr. The significantly lower false positivity and false negativity rates indicate that the proposed model is a promising tool for detecting seizures in epilepsy patients. The employed parallel path network benefits from memory function of Bi-LSTM and strong feature extraction capabilities of CNN. Moreover, eliminating the need for any domain transformation or additional preprocessing steps, model effectively reduces complexity and enhances efficiency, making it suitable for use by clinicians during the epilepsy diagnostic process., (© 2024 IOP Publishing Ltd.)

Objective: We aimed to compare the electroclinical correlates of truncating and missense variants of SCN1A variants in children with Dravet syndrome (DS) and to determine phenotypic features in relation to variants identified and seizure outcomes., Methods: A single center prospective study was carried out on a South Indian cohort. Patients below 18 years of age who met the clinical criteria for DS who had undergone genetic testing and completed a minimum of one year follow up were included. We compared the differences in clinical profile, seizure outcome, developmental characteristics and anti-seizure medication (ASM) responsiveness profiles between patients with missense and truncating variants., Results: Out of a total of 3967 children with drug-resistant epilepsy during the period 2015-2021, 49 patients who fulfilled the inclusion criteria were studied. Thirty-seven had positive genetic tests, out of which 29 were SCN1A variants and 9 were other novel variants. The proportion of missense (14; 48.3%) and truncating SCN1A variants (15; 51.7%) was similar. A significant trend for developing multiple seizure types was noted among children with truncating variants (p = 0.035) and seizure freedom was more likely among children with missense variants (p = 0.042). All patients with truncating variants had ASM resistant epilepsy (p = 0.020). Developmental outcomes did not differ between the variant subtypes., Conclusion: Our results show that children harbouring missense variants demonstrated a significantly lower propensity for multiple seizure subtypes and a higher proportion with seizure freedom. However developmental implications appear to be independent of variant subtype., Competing Interests: Declaration of competing interest Nil., (Copyright © 2024 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Arginase deficiency, which leads to hyperargininaemia is a rare urea cycle disorder caused by a mutation in the ARG1 gene. It is an under-recognized cause of pediatric developmental epileptic encephalopathy, with the key coexistent clinical features being developmental delay or regression and spasticity. Detection of ARG1 gene mutation on genetic testing is the confirmatory diagnostic test. However, elevated levels of plasma arginine and low plasma arginase level can be considered as biochemical markers for diagnosis. We present two cases of arginase deficiency with genetically confirmed ARG1 mutation in one and biochemical confirmation in both. As the spectrum of epilepsy in arginase deficiency has been less explored, we attempted to elucidate the novel electroclinical features and syndromic presentations in these patients. Informed consent was obtained from families of patients. Electroclinical diagnosis was consistent with Lennox Gastaut syndrome (LGS) in the first patient while the second patient had refractory atonic seizures with electrophysiological features consistent with developmental and epileptic encephalopathy. Though primary hyperammonaemia is not a consistent feature, secondary hyperammonaemia in the setting of infectious triggers and drugs like valproate (valproate sensitivity) has been well described as also observed in our patient. In the absence of an overt antecedent in a child with spasticity and seizure disorder, with a progressive course consistent with a developmental epileptic encephalopathy, arginase deficiency merits consideration. Diagnosis often has important therapeutic implications with respect to dietary management and choice of the appropriate antiseizure medications., (© 2023 International League Against Epilepsy.)

In this study, we classify the seizure types using feature extraction and machine learning algorithms. Initially, we pre-processed the electroencephalogram (EEG) of focal non-specific seizure (FNSZ), generalized seizure (GNSZ), tonic-clonic seizure (TCSZ), complex partial seizure (CPSZ) and absence seizure (ABSZ). Further, 21 features from time (9) and frequency (12) domain were computed from the EEG signals of different seizure types. XGBoost classifier model was built for individual domain features and combination of time and frequency features and validated the results using 10-fold cross-validation. Our results revealed that the classifier model with combination of time and frequency features performed well followed by the time and frequency domain features. We obtained a highest multi-class accuracy of 79.72% for the classification of five types of seizure while using all the 21 features. The band power between 11-13 Hz was found to be the top feature in our study. The proposed study can be used for the seizure type classification in clinical applications.

Epilepsy is a neurological disorder characterized by recurrent seizures. Automated prediction of epileptic seizures is essential in monitoring the health of an epileptic individual to avoid cognitive problems, accidental injuries, and even fatality. In this study, scalp electroencephalogram (EEG) recordings of epileptic individuals were used to predict seizures using a configurable Extreme Gradient Boosting (XGBoost) machine learning algorithm. Initially, the EEG data was preprocessed using a standard pipeline. We investigated 36 minutes before the onset of the seizure to classify between the pre-ictal and inter-ictal states. Further, temporal and frequency domain features were extracted from the different intervals of the pre-ictal and inter-ictal periods. Then, the XGBoost classification model was utilized to optimize the best interval for the pre-ictal state to predict the seizure by applying Leave one patient out cross-validation. Our results suggest that the proposed model could predict seizures 10.17 minutes before the onset. The highest classification accuracy achieved was 83.33 %. Thus, the suggested framework can be optimized further to select the best features and prediction interval for more accurate seizure forecasting.

Purpose: Telemedicine gained popularity in the setting of the COVID-19 pandemic. We aimed to study the satisfaction levels of persons with epilepsy (PWE) with online video consultation (OVC) and physical consultation (PC)., Methods: This was a cross-sectional questionnaire-based study conducted in a tertiary referral care center for epilepsy in India. All PWE who had availed of both OVC and PC were included. Those who did not give consent to a questionnaire were excluded. A questionnaire was given to assess patients' satisfaction regarding OVC and PC. Scores for each question for both OVC and PC were compared., Results: One hundred and forty-one patients who had PC earlier and later availed of OVC from December 2020 to July 2021 formed the cohort. Seventy one patients who responded to the questionnaire were included. 49% and 51% of the patients belonged to urban and rural regions respectively. 8.5% of the patients were off anti-seizure medications (ASM), while 5.6% and 85.9% were on single and multiple ASMs respectively. There were no differences between PC and OVC regarding ease of getting the appointment, privacy during a consultation, patients' perceived chances of missing consultations, and overall comfort and experience on either type of consultation. Physical consultation scored more than OVC in patients' satisfaction with the time doctor spent with them, an opportunity to communicate their queries well, clarifications received from the doctor, and the likelihood of patients recommending the particular type of consultation to others(p < 0.05)., Conclusions: Online video consultation can be a satisfactory alternative to PC and can improve patient satisfaction if some of the issues in OVC are addressed properly., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: A third of people with juvenile myoclonic epilepsy (JME) are drug-resistant. Three-quarters have a seizure relapse when attempting to withdraw anti-seizure medication (ASM) after achieving seizure-freedom. It is currently impossible to predict who is likely to become drug-resistant and safely withdraw treatment. We aimed to identify predictors of drug resistance and seizure recurrence to allow for individualised prediction of treatment outcomes in people with JME., Methods: We performed an individual participant data (IPD) meta-analysis based on a systematic search in EMBASE and PubMed - last updated on March 11, 2021 - including prospective and retrospective observational studies reporting on treatment outcomes of people diagnosed with JME and available seizure outcome data after a minimum one-year follow-up. We invited authors to share standardised IPD to identify predictors of drug resistance using multivariable logistic regression. We excluded pseudo-resistant individuals. A subset who attempted to withdraw ASM was included in a multivariable proportional hazards analysis on seizure recurrence after ASM withdrawal. The study was registered at the Open Science Framework (OSF; https://osf.io/b9zjc/)., Findings: Our search yielded 1641 articles; 53 were eligible, of which the authors of 24 studies agreed to collaborate by sharing IPD. Using data from 2518 people with JME, we found nine independent predictors of drug resistance: three seizure types, psychiatric comorbidities, catamenial epilepsy, epileptiform focality, ethnicity, history of CAE, family history of epilepsy, status epilepticus, and febrile seizures. Internal-external cross-validation of our multivariable model showed an area under the receiver operating characteristic curve of 0·70 (95%CI 0·68-0·72). Recurrence of seizures after ASM withdrawal (n = 368) was predicted by an earlier age at the start of withdrawal, shorter seizure-free interval and more currently used ASMs, resulting in an average internal-external cross-validation concordance-statistic of 0·70 (95%CI 0·68-0·73)., Interpretation: We were able to predict and validate clinically relevant personalised treatment outcomes for people with JME. Individualised predictions are accessible as nomograms and web-based tools., Funding: MING fonds., Competing Interests: AAA received a grant from the 10.13039/501100012155National Institute for Medical Research Development, royalties for a book publication from 10.13039/501100007723Oxford University Press, and speaker fees from Cobel Daruo, Tekaje, and Raymand Rad. CPB received research grants and honoraria from 10.13039/100011110UCB and 10.13039/501100003769Eisai, support for attending meetings by UCB, and served in the advisory board of Arvelle. CDB received consulting fees and honoraria from 10.13039/100014114GW Pharmaceuticals, 10.13039/100015661UCB Pharma, 10.13039/501100003769Eisai, 10.13039/501100006546Angelini Pharma and Bial. JPS received grants from the 10.13039/100000002National Institutes of Health, 10.13039/100000005Department of Defense, and the 10.13039/100000001National Science Foundation; and consulting fees from 10.13039/100015661UCB Pharma, AdCel Biopharma, LLC, iFovea, SK Life Sciences, and 10.13039/100013410LivaNova; and has stock options for iFovea and AdCel Biopharma. LEH-V participates in the Young Epilepsy Society, received speaker honorario from Armstrong, and was supported by Abbott pharmaceuticals to attend the Mexican Congress of Neurology. NP received honoraria from Zogenix and Ethos for Angelini Pharma. NS received honoraria from Biomarin, Livanova, GW Pharma, Zogenix and Marinus; and support to attend meetings from Livanova, GW Pharma and Zogenix; and participated on a data safety monitoring board for Marinus. SB received speaker fees from Eisai. PK received lecture honorarium from UCB Pharma and Eisai, consulting fees from 10.13039/501100003769Eisai and 10.13039/100013410LivaNova and his institution received research grants from 10.13039/100015661UCB Pharma and 10.13039/501100003769Eisai. MS received speaker honoraria from UCB Pharma and Eisai. KKS received research grants from the 10.13039/501100005416Norwegian Research Council, the DAMFoundation and the Norwegian National Advisory Unit on Rare diseases; and a networking grant from the 10.13039/501100004785NordForsk Foundation; and she acted as a paid PhD defense opponent at the University of Bergen, and attended a meeting for Nordic clinicians organised by Eisai. BJV received grants from the 10.13039/501100007870German Society for Epileptology and the 10.13039/501100001659Deutsche Forschungsgemeinschaft; and honoraria from University Medical Center Schleswig–Holstein and Cornelsen Verlag. MH received consulting fees and honoraria from Arvelle, Bial, Desitin, Eisai, GW Pharmaceuticals, UCB Pharma, and Zogenix. FvP has received speaker honoraria from Bial, Eisai, GW Pharmaceutical companies, Angelinipharma, Zogenix and UCB Pharma; and scientific advisory board honoraria from GW Pharmaceutical companies, UCB Pharma, and Angelinipharma. WD's salary is part-funded by The University of Melbourne; he has received travel, investigator-initiated, scientific advisory board and speaker honoraria from UCB Pharma Australia and Global; investigator-initiated, scientific advisory board, travel and speaker honoraria from Eisai Australia and Global; advisory board honoraria from Liva Nova and Tilray; educational grants from Novartis Pharmaceuticals, 10.13039/100014476Pfizer Pharmaceuticals and Sanofi-Synthelabo; educational; travel and fellowship grants from GSK Neurology Australia, and honoraria from SciGen Pharmaceuticals; and he has an equity interest in the device company EpiMinder. CPB received honaries and research support from EISAI, UCB and Arvelle. ET received speaker's honoraria from Arvelle, Abbott, Angelini Pharma, UCB, Biogen, Gerot-Lannacher, Bial, Eisai, Epilog, Takeda, Newbridge, Hikma, GW Pharmaceuticals, Sunovion Pharmaceuticals Inc., LivaNova and Novartis; consultancy funds from 10.13039/501100006546Angelini Pharma, Argenix, Arvelle, Epilog, 10.13039/100011110UCB, 10.13039/100005614Biogen, Gerot-Lannach, Bial, 10.13039/501100003769Eisai, 10.13039/100007723Takeda, Newbridge, 10.13039/100014114GW Pharmaceuticals, 10.13039/100009655Sunovion Pharmaceuticals Inc., Marinus, and 10.13039/100004336Novartis; directorship funds from Neuroconsult GmbH. ET's Institution received grants from 10.13039/100005614Biogen, Red Bull, 10.13039/100004334Merck, 10.13039/100011110UCB, 10.13039/501100000780European Union, FWF Österreichischer Fond zur Wissenschaftsförderung, and 10.13039/501100007148Bundesministerium für Wissenschaft und Forschung. JWS reports personal fees from Arvelle, personal fees from 10.13039/100011110UCB, grants from UCB, grants from 10.13039/100003997NEF, grants from UCB, personal fees from 10.13039/100012739Zogenix, grants from GW Phama, outside the submitted work; and his current position is endowed by the Epilepsy Society, he is a member of the Editorial Board of the Lancet Neurology, and receives research support from the Marvin Weil Epilepsy Research Fund. All other authors declare no potential competing interests. None of the above mentioned declarations represent a conflict of interest directly related to the present publication., (© 2022 The Author(s).)

Objective: To explore novel word learning via fast mapping (FM) and explicit encoding (EE) in temporal lobe epilepsy (TLE)., Methods: 16 right and 16 left temporal lobe epilepsy (RTLE and LTLE) patients along with 32 normal controls (NC) underwent learning of 24 novel object name pairs through standard FM and EE techniques. Their learning was assessed via a three-choice alternate delayed recognition task on the day of learning and on the following day. Recognition scores were compared using nonparametric statistics across the groups with P value set at <.05., Results: RTLE and NC performed similarly, while LTLE and NC differed significantly in novel word learning irrespective of the method of encoding. LTLE and RTLE differed in EE-based novel word learning alone. Further, with respect to encoding techniques, all groups performed better on EE compared to FM. The novel word associations learned via FM showed a lesser decline compared to EE following overnight integration in RTLE and NC., Conclusion: Novel word learning via FM did not facilitate learning above EE in TLE patients or NC. But FM-based words could better overcome forgetting following overnight integration in RTLE and NC. Hence, it is possible that FM has the potential to improve retention of novel information following overnight integration in RTLE as in NC. However, its efficacy in improving retention in LTLE needs further evidence., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Annals of Indian Academy of Neurology.)

Purpose: To study if one can conceptualize the scalp ictal onset pattern through analysis of interictal spike domain analysis in temporal lobe epilepsy (TLE)., Methods: Seventy-four patients with unilateral mesial temporal sclerosis (MTS) were categorized into "type A" interictal epileptiform discharges (IEDs) with negativity over infero-lateral scalp electrodes over temporal region and contralateral central region showing positivity; all IEDs other than type A were categorized as type B. The ictal electrographic patterns was termed "focal" when confined to side of MTS, was "regional" when lateralized to the ipsilateral hemisphere; "diffuse" if nonlateralized/localized; and ictal onset contralateral to MTS termed as "discordant.", Results: A total of 377 seizures and 5,476 spikes were studied. These were divided into four types: (1) type A IEDs ipsilateral to MTS (44 patients), (2) type A IEDs bitemporally (16 patients), (3) type A IEDs contralaterally (7 patients) and type B IEDs ipsilaterally, and (4) bilateral type B IEDs (7 patients). The ictal pattern was either focal or regional in 51 of 60 patients (85%) with type A IEDs; it was "diffuse" in 9 patients (15%). Diffuse ictal onset was seen in 12 of 14 (86%) with either ipsilateral/bitemporal type B IEDs. Ictal onset on the opposite hemisphere was noted in 2 (14%)., Conclusions: Type A IEDs signify a focal ictal onset and type B IEDs suggest a diffuse ictal onset in patients with MTS on one side., Significance: Interictal spike domain analysis helps predicting ictal patterns in temporal lobe epilepsy., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 by the American Clinical Neurophysiology Society.)

Background: Prediction of outcome of West syndrome (WS) in relation to etiology and electrophysiology remain pertinent challenges., Objective: This study aimed to compare electro-clinical and imaging characteristics between WS of "unknown-etiology"; "symptomatic"WS; to gauge the evolution and impact of electroencephalographic (EEG) patterns on seizure outcomes., Materials and Methods: Electro-clinico-radiological data of 76 children with WS who were followed up for atleast 1 year was collected for reviewing clinical, therapeutic and EEG profiles (sub-typed as typical and modified hypsarrhythmia [HA]). Quantified seizure scores were assessed., Results: Among 76 children included in this retrospective analysis, 31 (40.8%) were of unknown-etiology and 45 (59.2%) were "symptomatic" (structural cause/developmental-encephalopathy). Children with symptomatic WS (p = 0.037), specifically with gliosis on imaging (p = 0.05) and typical HA (including the multifocal subtype; P = 0.023) were more likely to have other seizure types before onset of spasms and exhibit prior delay or regression in milestones (p = 0.017). There was negative correlation between time to diagnosis and reduction in seizure scores (r = -0.32; p = 0.005).Significant reduction was noted in seizure scores with pharmacotherapy, irrespective of etiology (P < 0.001 in unknown-etiology and symptomatic subgroups). Seizure freedom rates did not differ between typical and modified HA groups (p = 0.215) with a higher proportion of children with meaningful reduction in seizure scores in the former sub-group (p = 0.030). Children who failed to achieve seizure remission were more likely to exhibit developmental impairment (p = 0.019)., Conclusions: Early diagnosis and initiation of optimal therapy is crucial towards improving outcome, irrespective of etiology (which impacts pre-spasm development) and HA subtypes., Competing Interests: None

Purpose: Locus of control (LOC) is the degree to which people believe that they have control over the outcome of events in their lives. A person's locus can be internal, external, or chance. A person with internal locus of control believes that one can control one's own life. A person with external locus of control believes that his life is controlled by external factors or people over which he has no influence. A person with chance locus of control believes that fate, chance, or luck controls his own life. The aim of the current study was to determine the health locus of control, anxiety, and depression levels in persons with epilepsy (PWE) and to assess whether locus of control has relation to anxiety, depression, and seizure control., Methods: Patients aged 18 years or older with a history of epilepsy for at least 1 year were recruited from the outpatient epilepsy clinic or from the inpatient epilepsy monitoring unit at SCTIMST, Trivandrum from January 2019 to May 2020. Patients filled the questionnaire form consisting of demographic data, age of onset of seizures, present seizure control, and the current antiepileptic drugs. The Hospital Anxiety and Depression (HAD) scale was used to estimate the level of anxiety and depression in these patients. The Form-C of the Multidimensional Health Locus of Control (MHLC) scale was used to evaluate the health locus of control. Healthy controls aged 18 years or older and free of any chronic disease or psychiatric illness were also recruited. They were asked to fill the questionnaire forms with basic demographic data. HAD scale was used to estimate the level of anxiety and depression and form-C of MHLC was used to evaluate the health locus of control in the healthy controls. The mean scores of anxiety, depression, and locus of control were compared between the two groups., Results: A total of 170 participants were recruited which consisted of 100 PWE and 70 healthy controls. The mean anxiety and depression scores were 8.13(SD = 4.23) and 5.85(SD = 3.66) in the PWE group and 6.75(SD = 3.39) and 4.14(SD = 2.96) in the control group, respectively. The mean internal, external, and chance LOC scores were 24.95(SD = 10.92), 26.94(SD = 4.96), and 24.41(SD = 6.46) in the PWE group; and 29.44(SD = 5.62), 26.53(SD = 5.79), and 19.9(SD = 7.13) in the control group, respectively. Persons with epilepsy had higher chance LOC scores and lower internal LOC scores compared to controls (p = 0.00003, p < 0.00001 respectively). There were no differences in the external LOC scores between the two groups (p = 0.620). Persons with epilepsy with some level of anxiety had lower internal LOC scores compared to patients with no anxiety (p = 0.04). PWE with poor seizure control had higher external LOC score and lower internal LOC scores which however did not reach statistical significance. Persons with epilepsy with poor seizure control had higher anxiety and depression scores., Conclusions: Persons with epilepsy had low perceptions of internal and strong perceptions of chance health locus of control. This means that PWE feel that luck plays an important role in their disease control. This information is important in the counseling of persons with epilepsy., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: To study the trends for pediatric epilepsy surgery between 2000 and 2014 in a tertiary epilepsy surgery center in India in order to gain a lower- and middle-income country (LMIC) perspective., Methods: Children aged <18 years and undergoing epilepsy surgery were divided into three groups based on the year that they underwent surgery-group 1: year 2000-2004; group 2: year 2005-2009; and group 3: year 2010-2014. Data including the rate of surgery, type of surgery, and duration of epilepsy before referral were analyzed from the medical records and compared., Results: Between 2000 and 2014, 463 pediatric epilepsy surgeries were performed. The proportion of pediatric epilepsy surgeries showed an increasing trend-218 (total 510 surgeries, 42.74%) in group 3, compared with 115 (total 375 surgeries, 30.66%) in group 1 and 130 (total 466 surgeries, 27.9%) in group 2. A significant decrease in the age at evaluation and duration of epilepsy before referral was noted between 2000 and 2014, particularly in patients belonging to the lowest income group. There was a two-fold increase in the number of extratemporal surgeries over time. The proportion of children undergoing surgery for benign tumors, cortical malformations, and gliosis/atrophy showed an upward trend while that for mesial temporal sclerosis did not show an increase., Significance: Promising trends in pediatric epilepsy surgery were noted with increasing number of surgeries and decreasing age at presurgical evaluation. Seen from an LMIC perspective, this reflects an evolution in the practice of pediatric epilepsy surgery, mirroring trends in high-income countries., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Purpose: We conducted this study to determine the clinical, electrophysiological and radiological predictors of outcome in Super Refractory Status Epilepticus (SRSE)., Methods: Data of patients treated for SRSE between January 2000 and November 2019, archived prospectively in our SE registry were analyzed. Functional outcome was measured by Glasgow outcome score (GOS) at the time of hospital discharge and was divided into: good i.e. GOS ≥ 3 and bad outcome i.e. GOS < 3. The predictors of outcome were determined using appropriate statistical tests by univariate and multivariate analysis, p < 0.05 was considered as statistically significant., Results: Of the 384 patients with status epilepticus (SE) identified during the study, 28 (8%) were diagnosed as SRSE and were included in the final analysis. Acute symptomatic SE comprising 15 (53.6%) patients was the most common etiology of SRSE. Thirteen patients (three patients with viral encephalitis and 10 patients with clinically possible autoimmune encephalitis) had New Onset Refractory Status Epilepticus (NORSE) like clinical presentation.12 patients (42.9%) had good outcome and 16 patients (57.1%) had bad outcome. Multivariate logistic regression analysis showed that independent predictors of poor outcome were: duration of ICU stay (p < 0.001); EEG findings such as non-convulsive SE in coma (0.032), spontaneous burst suppression (0.001) and postictal diffuse attenuation (<0.001); delay in starting anesthesia (0.002); and delay in starting immunotherapy in NORSE due to autoimmune encephalitis (0.002)., Conclusion: We could determine independent therapeutic and electrophysiological prognostic factors for SRSE. Early initiation of treatment and stringent management of these factors especially in an younger age-group, aided by continuous EEG monitoring and a thorough etiological work-up can result in good outcomes in more than one-third of cases., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: McGurk effect is a perceptual phenomenon that demonstrates an interaction between hearing and vision in speech perception. A wide range of neuropsychological deficits have been described in people with long-standing epilepsy, which affect multimodal integration in speech perception and hence refractory epilepsy patients are ideal for testing the McGurk effect., Materials and Methods: We studied the McGurk effect in 50 patients diagnosed with medically refractory left or right hemispheric epilepsy based on clinical, radiological, and electrophysiological data., Results: The McGurk effect was better perceived (p = 0.006) in patients with left hemispheric epilepsy (n = 12, 71%) compared to right (n = 5, 29%). The other factors which compromised the perception of the McGurk effect were impairments in visual memory (p = 0.041), facial emotion recognition (p = 0.001), and lip-reading (p = 0.006). Perception of the McGurk effect reduced significantly (p = 0.006) when the epilepsy duration was 10 years or beyond., Conclusion: The McGurk effect can be used in refractory epilepsy patients, to detect subtle abnormalities in speech perception, before significant irreversible speech and language dysfunction become evident., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Psychiatric disease is the most common co-morbidity seen in persons with epilepsy (PWE), often secondary to psychosocial disturbances due to poor adaptation to the disease. Optimizing epilepsy treatment by targeting both seizure control and addressing psychological issues go a long way in improving psychological outcome among PWE. This study aims to assess the psychological status among PWE using validated questionnaires and to assess the effect of viewing their own seizures on their psychological milieu post-viewing., Methods: A prospective interventional study was conducted enrolling 52 PWE. Their baseline psychological scores were assessed using Hospital Anxiety Depression Scale (HADS), Self Esteem Inventory (SEI), Locus of Control (LOC) and Quality of Life in Epilepsy (QOLIE-31). The scores were reassessed 3 months post seizure viewing and compared with the baseline scores., Results: Among the 52 patients analyzed, anxiety was present in 20 (38.4%) and depression in 8 (15.3%). The mean HADS-A (anxiety) scores for study population was 9.56 ± 4.12. Gender, income status, risk factors antedating index seizure, type of seizure, temporal semiology, seizure frequency, MRI or EEG characteristics did not have any significant association with baseline psychological scores. There was statistically significant reduction in HADS-A scores from 9.56 ± 4.12 to 8.35 ± 3.85 in the study population after seizure viewing (p = 0.049). We identified patient characteristics that benefit seizure viewing with respect to each of the psychological batteries mentioned earlier., Conclusion: This study emphasizes that viewing one's own seizures improves the psychological milieu of PWE and improves their quality of life by helping them cope up with their illness more judiciously., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS), with its associated impact on language, is an important cause of morbidity with epilepsy in children. The effects of various treatment-approaches and the aetiological/electrophysiological factors affecting therapeutic response are not fully understood., Method: A retrospective study of patients admitted to the institute and diagnosed to have CSWS pattern on EEG was conducted. Spike and Wave Frequency/100 s(SWF) was calculated from sleep-EEG records. Language development and seizure outcomes were assessed at baseline and 1 year., Results: Fifty-two children were included (idiopathic CSWS, N = 19; symptomatic CSWS N = 33).The 2 groups differed in terms of younger age at seizure onset in symptomatic CSWS (p = 0.006), early age at language regression (p = 0.046), history of neonatal seizures (p = 0.038) and slowing of background activity on EEG (p = 0.024). Language regression was noted in 63.5 % of the cohort. Twenty-five (48%) patients received steroids with improvement in seizures (p < 0.001). Twenty-one (40.3 %) received steroids and intravenous immunoglobulin (IVIG) with improvement in seizure score (p = 0.002) at 1 year. Both immune-modulation arms irrespective of etiological subgroups had comparable proportions of children with expressive and receptive language gains. On 1 year follow-up, seizure remission was noted in 13(25 %) patients, with improved seizure score in 32/39 (61.5 %) patients and language improvement in 32 children (60.8 %). Patients with normal background on baseline EEG, generalised spikes, absent frontal-negative spikes and SWF < 170 were found to have improved language estimates on follow-up., Conclusions: Despite clinical and therapeutic outcome differences between idiopathic and symptomatic CSWS, immune-modulation appears effective irrespective of aetiology. Analysis of EEG variables enables prediction of language outcomes at 1 year follow-up., (Copyright © 2020 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Introduction: A paucity of literature exists on genotype- phenotype correlates of 'unknown-etiology' infantile-onset developmental-epileptic encephalopathies (DEE) from India. The primary objective was to explore the yield of genetic testing in identifying potential disease causing variants in electro-clinical phenotypes of DEE METHODS: An observational hospital-based study was undertaken on children with unexplained refractory seizure-onset ≤12 months age and developmental delay, whose families consented and underwent genetic testing during a three year time period (2016-2018) by next-generation sequencing (NGS) or multiplex ligand protein amplification. Yield was considered based on demonstration of pathogenic/likely pathogenic variants only and variants of unknown significance (VUS) were documented., Results: Pathogenic/likely pathogenic variants were identified in 26 (31.7 %) out of 82 children with DEE. These included those variants responsible for primarily DEE- 21(76.7 %); neuro-metabolic disorders- 3(18.6 %) and chromosomal deletions- 2(4.7 %). Of these patients, early-infantile epilepsy onset ≤ 6 months age was noted in 22(84.6 %). The DEE studied included Ohtahara syndrome associated with STXBP1 and SCN8A variants with yield of 50 % (2/4 tested); early myoclonic encephalopathy (no yield in 2); West syndrome with CDKL5, yield of 13.3 % (2/15 tested); epilepsy of infancy with migrating partial seizures due to CACNA1A and KCNT1 variants, yield of 67 % (2/3 tested); DEE-unclassified with KCNQ2, AP3B2, ZEB2, metabolic variants (SUOX, ALDH7A1, GLDC) and chromosome deletions (chr 1p36, chr2q24.3); yield of 32 % (8/25 tested). Patients with Dravet syndrome/Dravet-like phenotypes (N = 33) had variants in SCN1A (N = 10), SCN1B, CHD2; yield of 36.4 % (12/33 tested; 57.1 % from NGS). Eighteen patients with potential variants (SCN1A, SCN2A, SCN8A, KCNQ2, ALDH7A1 which also included VUS) could be offered targeted therapy., Conclusions: Our study confirms a good yield of genetic testing in neonatal and infantile-onset DEE provided robust phenotyping of infants is attempted with prognostic and therapeutic implications, particularly relevant to centres with resource constraints., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Purpose: To compare the seizure outcome following early and late complete antiepileptic drug (AED) withdrawal following anterior temporal lobectomy (ATL) for mesial temporal lobe epilepsy (MTLE)., Method: All the patients who were seizure free for one year following ATL were offered early or late AED withdrawal. AEDs were discontinued starting at one year in those who opted for early withdrawal. Patients who opted for late withdrawal were continued on single AED for three years following surgery before attempting complete discontinuation., Results: Of the 135 study patients, 65 opted for early AED withdrawal and 70 for late withdrawal. The mean postoperative follow-up duration was 10.4 ± 1.3 (Range, 8-12) years. At three years following surgery, seizure recurrence occurred in 23 (35.4 %) patients in the early withdrawal group and in 10 (14.3 %) patients in late withdrawal group (p = 0.005; relative risk [RR], 2.48; 95 % confidence interval [CI], 1.28-4.80). At last follow-up, 27 (41.5 %) patients in the early withdrawal group and 26 (37.1 %) in late withdrawal group had recurrence (p = 0.60; RR, 1.12, 95 % CI, 0.74-1.70). At last followup, 80 (59.3 %) patients were off AEDs. During the terminal one year, 123 (91 %) patients were seizure free, similar in the two groups., Conclusions: This nonrandomized controlled study suggests that early complete AED withdrawal starting one year following ATL is associated with a higher risk of early seizure recurrence. However, long term seizure outcome is similar in early and late AED withdrawal groups., Competing Interests: Declaration of Competing Interest None of the authors have any conflict of interest related to this work., (Copyright © 2019 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Background: There is lack of uniform treatment protocol for status epilepticus (SE) in pregnancy, with majority of data being limited to individual cases or case series. Devising a uniform treatment protocol will facilitate prompt control of SE in pregnancy and reduce adverse maternal and fetal outcomes., Methods: Literature search was done in various databases including PubMed, CINAHL, EMBASE, TRIP, and the gray literature, including relevant organizational websites, for the topics "Status Epilepticus" and "Pregnancy". English language original research articles, case reports, and systematic reviews that were published in the last 18 years (2000-2018) and addressed SE in relation to pregnancy (i.e., antepartum, labor, or postpartum) were considered for inclusion., Results: Over the past 15 years, a total of seven articles reporting 29 cases of SE related to pregnancy, satisfying the inclusion criteria were analyzed. The most common cause of SE was posterior reversible encephalopathy syndrome (PRES)/reversible cerebral vasoconstriction syndrome (RCVS) spectrum (n = 11, 38%), followed by cortical venous sinus thrombosis (CVT) and autoimmune encephalitis (n = 5, 17%). Twenty-three out of 29 cases (79%) had good maternal outcomes in terms of recovery to baseline. Seventeen fetuses (58%) were delivered at term and seven at preterm (2.4%). First-line agent used was lorazepam in 15 patients (52%) and midazolam in two patients (7%). The most common antiepileptic drug (AED) and anesthesia used for treatment of SE and refractory SE were phenytoin/fosphenytoin (n = 21, 72%) and midazolam (n = 12, 52%), respectively. In all cases due to eclampsia (n = 5), magnesium sulfate was the preferred first-line drug., Conclusion: Management of SE in pregnancy is influenced by etiology of SE and duration of pregnancy. It carries a good prognosis if detected early and treated appropriately. Large-scale multicentric studies are warranted for formulating definite guidelines for management of SE in pregnancy. This article is part of the Special Issue "Proceedings of the 7th London-Innsbruck Colloquium on Status Epilepticus and Acute Seizures"., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Aim: The aim is to study survival outcome and sudden unexpected death in epilepsy (SUDEP) of people with epilepsy who underwent epilepsy monitoring., Subjects and Methods: Between 2000 and 2004, 558 persons were admitted to the epilepsy monitoring unit of SCTIMST who fulfilled the selection criteria. Their survival status as on December 31, 2013, was ascertained by clinic attendance record and by mail or telephone contact., Results: Five hundred and fifty-eight persons with epilepsy (males 319, 6408 person-years) were included in this study. There had been 20 deaths till December 31, 2013, and 5 additional deaths were reported when followed up till May 2016. The standardized mortality ratio was 5.35 and higher for males (6.25) than for females (3.52). Those with generalized seizures (tonic and myoclonic seizures), electroencephalography (EEG) showing multifocal and generalized interictal discharges, and polypharmacy had a higher risk of mortality. SUDEP accounted for 5 deaths., Conclusion: People with refractory epilepsy who had undergone presurgical video EEG monitoring had higher mortality risk. Generalized or myoclonic seizures, multifocal or generalized interictal discharges, and polypharmacy independently increased their risk of mortality. SUDEP is an important cause of death., Competing Interests: There are no conflicts of interest., (Copyright: © 2006 - 2019 Annals of Indian Academy of Neurology.)

Background: We conducted a retrospective study examining the trends in referral to a tertiary epilepsy surgery center in India over 2 decades., Methods: Data of patients who underwent long-term video electro encephalography monitoring for presurgical evaluation were retrospectively analyzed. Patients aged >16 years and diagnosed to have drug-resistant temporal lobe epilepsy (TLE) were included. They were divided into 3 groups comprising 5-year periods each during which they underwent presurgical evaluation, group 1: year 2000-2004; group 2: year 2005-2009; and group 3: year 2010-2014. Referral data with particular reference to duration of epilepsy before referral, age at onset of seizures, and number of antiepileptic drugs tried before referral were analyzed., Results: A total of 1362 patients fulfilled the inclusion criteria. There were 385 referrals in group 1, 488 in group 2, and 489 in group 3. The mean duration of epilepsy before referral was 18.10 ± 9.44 years; there was no change in the duration of epilepsy before referral ( p = 0.638). A significant increase in the age at onset of seizures and age at presurgical evaluation was noted over time., Conclusion: There is evidence for delayed referral of patients with refractory TLE to a surgical epilepsy center in this study. Renewed efforts to confront challenges beholding epilepsy surgery and steps to ensure timely referral are desirable., (© 2019 American Academy of Neurology.)

Objectives: To explore the effect of duration of epilepsy and delay in surgery on seizure outcome in patients operated for drug-resistant temporal lobe epilepsy (TLE)., Materials & Methods: A total of 664 consecutive patients who underwent anterior temporal lobectomy (ATL) for TLE from 1995 to 2008 formed the study cohort. We divided them into two, one as seizure-free with or without antiepileptic drugs after ATL as "good outcome" (Engel class I a) and seizures of any type, any time after surgery as "poor outcome." The probability of seizure freedom/seizure recurrence based on the duration of epilepsy was compared using Kaplan-Meier curves, univariate Cox regression survival analysis, and multivariate Cox proportional hazards regression model., Results: A total of 136 children and 528 adults underwent ATL during this period. Mean duration of epilepsy pre-ATL was 17.1 + 9.4 years. At mean follow-up of 8.5 years, 331 patients (49.8%) had good outcome and 333 (50.2%) had poor outcome. The hazard of seizure recurrence linearly increased with duration of epilepsy pre-ATL, from 1.5 (duration of epilepsy, 5-10 years) to 1.9 (duration of epilepsy, 10-15 years) to 2 (duration of epilepsy over 15 years). In addition, encephalitis as antecedent, bilateral mesial temporal sclerosis in MRI, normal histopathology, and spikes in postoperative EEG at 3 months and 1 year predicted poor seizure outcome., Conclusions: "Epilepsy duration" independently predicted both short- and long-term seizure outcome after surgery in TLE. "Lost years" translate into poor seizure outcome after ATL. Therefore, all cases of drug-resistant TLE should be referred to a surgical center at the earliest., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Purpose: The success of epilepsy surgery lies in identifying the ictal onset zone accurately. The significance of auras has little been explored on surgical outcome in drug-resistant epilepsy. This study focuses on the clinicopathological correlation of aura(s) and its role in predicting surgical outcome in drug-resistant temporal lobe epilepsy (TLE). We compared surgical outcome in TLE between patients with and without aura and identified the clinico-pathological, radiological and surgical differences between the two groups., Methods: Consecutive patients who underwent presurgical evaluation from January 2009 to December 2014 for drug-resistant TLE who underwent anterior temporal lobectomy (ATL) were included. Patients were followed up at 3months, 12 months and then annually., Results: Among 456 patients, 344(75%) had aura. Multivariate logistic regression showed that prototype EEG pattern at ictal onset (OR 2.12, 95% CI 1.18-3.06, p = 0.012) and right sided epileptogenic zone (OR 1.82 95% CI 1.18-3.78, p = 0.007) were significantly associated with presence of aura. There was no difference in surgical outcome between those with and without aura. But patients with auditory aura (OR 7.28, CI 2.80-18.95, p = 0.0002) and vertiginous aura (OR 3.01, CI 1.55-7.85, p = 0.028) had a poor surgical outcome. Bivariate analysis showed that normal MRI (p = 0.028) and normal/indeterminate pathology (p = 0.001) were significantly more common with auditory/vertiginous auras., Conclusions: Mere presence of aura does not affect outcome after TLE surgery. However, auditory and vertiginous auras are predictors of poor surgical outcome. These patients require more extensive screening for an ictal onset zone beyond standard limits of ATL before surgery., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: To validate concurrent utility of within-scanner encoding and delayed recognition-memory paradigms to ascertain hippocampal activations during task-based memory fMRI., Methods: Memory paradigms were designed for faces, word-pairs and abstract designs. A deep-encoding task was designed comprising of a total of 9 cycles run within a 1.5T MRI scanner. A recall session was performed after 1 h within the scanner using an event-related design. Group analysis was done with 'correct-incorrect' responses applied as parametric modulators in Statistical Parametric Mapping version 8 using boot-strap method to enable estimation of laterality indices (LI) using custom anatomical masks involving the medio-basal temporal structures., Results: Twenty seven subjects with drug-resistant mesial temporal lobe epilepsy due to hippocampal sclerosis (MTLE-HS) [17 patients of left-MTLE and 10 patients of right-MTLE] and 21 right handed age-matched healthy controls (HC) were recruited. For the encoding paradigm blood oxygen level dependent (BOLD) responses in HC demonstrated right laterality for faces, left laterality for word pairs, and bilaterality for design encoding over the regions of interest. Both right and left MTLE-HS groups revealed left lateralisation for word-pair encoding, bilateral activation for face encoding, with design encoding in right MTLE-HS demonstrating a left shift. As opposed to lateralization shown in controls, group analysis of cued-recall BOLD signals acquired within scanner in left MTLE-HS demonstrated right lateralization for word-pairs with bilaterality for faces and designs. The right MTLE-HS group demonstrated bilateral activations for faces, word-pairs and designs., Conclusion: Recall-based fMRI paradigms indicate hippocampal plasticity in MTLE-HS, maximal for word-pair associate recall tasks., (Copyright © 2018 Elsevier B.V. All rights reserved.)