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Purpose: This study aimed to identify risk factors of postoperative seizure outcome in a consecutive cohort of patients operated on for TSC-related focal epilepsy, by evaluating several presurgical and surgical variables, including also MRI-visible brain abnormalities other than cortical tubers., Methods: This retrospective study included 51 patients surgically treated for drug-resistant focal epilepsy with a histological diagnosis of cortical tuber and followed for at least 12 months postoperatively. We investigated the association between several potentially explanatory variables and seizure outcome by univariate and multivariate analysis in the whole cohort and in the subgroups of patients with single and multiple tubers, respectively., Results: The median postoperative follow-up was 115 months (IQR 63-168) and 54.9% of patients were in Engel's class I at final control. In the whole cohort, variables independently associated with an unfavorable seizure outcome (Engel's classes II-IV) were: preoperative non-focal interictal EEG (RR 5, CI 2.46-6.39), presence of sub-ependymal nodules (SEN) (RR 3.53, CI 1.71-4.56) and seizure onset before the first year of age (RR 3.56, CI 0.91-6.89). Non-focal interictal EEG was independently associated with an unfavorable outcome also in the subgroup of patients with multiple tubers (RR 4.34, CI 2.23-5.37), while the presence of SEN (p=0.0221) and of extra-central nervous system lesions (p= 0.0152) predicted an unfavorable seizure outcome in patients with a single tuber., Conclusion: Surgery represents an effective option for seizure control in patients with TSC-related epilepsy. The identification of preoperative risk factors for seizure outcome could be helpful for optimizing patients' selection for surgery and pre-surgical counseling., (Copyright © 2022. Published by Elsevier Ltd.)

Background: Accurate localization of the probable Epileptogenic Zone (EZ) from presurgical studies is crucial for achieving good prognosis in epilepsy surgery., Objective: To evaluate the degree of concordance at a sublobar localization derived from noninvasive studies (video electroencephalography, EEG; magnetic resonance imaging, MRI; 18-fluorodeoxyglucose positron emission tomography FDG-PET, FDG-PET) and EZ estimated by stereoEEG, in forecasting seizure recurrence in a long-term cohort of patients with focal drug-resistant epilepsy., Methods: We selected patients with a full presurgical evaluation and with postsurgical outcome at least 1 yr after surgery. Multivariate Cox regression analysis for seizure freedom (Engel Ia) was performed., Results: A total of 74 patients were included, 62.2% were in Engel class Ia with a mean follow-up of 2.8 + 2.4 yr after surgery. In the multivariate analysis for Engel Ia vs >Ib, complete resection of the EZ found in stereoEEG (hazard ratio, HR: 0.24, 95%CI: 0.09-0.63, P = .004) and full concordance between FDG-PET and stereoEEG (HR: 0.11, 95%CI: 0.02-0.65, P = .015) portended a more favorable outcome. Most of our results were maintained when analyzing subgroups of patients., Conclusion: The degree of concordance between noninvasive studies and stereoEEG may help to forecast the likelihood of cure before performing resective surgery, particularly using a sublobar classification and comparing the affected areas in the FDG-PET with EZ identified with stereoEEG., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Musicogenic epilepsy is rare focal epilepsy in which seizures are triggered by music. Both spontaneous and reflexes seizures may occur. To date there are limited data about this epilepsy, particularly about its etiopathogenesis. We report the clinical, neurophysiological and imaging data about musicogenic epilepsy in a patient who underwent Stereo-electroencephalography (SEEG) study. A 27 year-old right-handed woman suffering from drug-resistant epilepsy since the age of 17 years, was evaluated for surgery. She had weekly seizures characterized by an unpleasant ascending gastric sensation, tachycardia, occasionally late oro-alimentary automatisms, déjà-vu and vomiting. Only during longer seizures a partial loss of awareness was reported. Interestingly, familiar songs triggered seizures. Rarely, she had spontaneous seizures with the same features. The ictal EEG onset appeared to be right temporal, but there was seizure propagation to suprasylvian areas. Brain MRI was negative. A SEEG implantation was performed to study the right temporo-perisylvian regions. SEEG data clearly indicated the antero-mesial temporal regions as origin of the seizures, without any spread to other close or distant cortical areas. Right temporal antero-mesial resection was performed 24 months ago and the patient is seizure-free since surgery. Neuropathology was uninformative. SEEG data highlighted the hypothesis regarding a temporo-mesial emotional-mnesic network triggered by particular music with an affective component for the patient. The primary auditory cortex and lateral mid-posterior temporal and extratemporal cortices were not involved. Different triggers as mentally singing and hearing the music can induce seizure as well as electrical stimulation in the mesial temporal structures., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

This retrospective description of a surgical series is aimed at reporting on indications, methodology, results on seizures, outcome predictors and complications from a 20-year stereoelectroencephalography (SEEG) activity performed at a single epilepsy surgery centre. Prospectively collected data from a consecutive series of 742 SEEG procedures carried out on 713 patients were reviewed and described. Long-term seizure outcome of SEEG-guided resections was defined as a binomial variable: absence (ILAE classes 1-2) or recurrence (ILAE classes 3-6) of disabling seizures. Predictors of seizure outcome were analysed by preliminary uni/bivariate analyses followed by multivariate logistic regression. Furthermore, results on seizures of these subjects were compared with those obtained in 1128 patients operated on after only non-invasive evaluation. Survival analyses were also carried out, limited to patients with a minimum follow-up of 10 years. Resective surgery has been indicated for 570 patients (79.9%). Two-hundred and seventy-nine of 470 patients operated on (59.4%) were free of disabling seizures at least 2 years after resective surgery. Negative magnetic resonance and post-surgical lesion remnant were significant risk factors for seizure recurrence, while type II focal cortical dysplasia, balloon cells, glioneuronal tumours, hippocampal sclerosis, older age at epilepsy onset and periventricular nodular heterotopy were significantly associated with seizure freedom. Twenty-five of 153 patients who underwent radio-frequency thermal coagulation (16.3%) were optimal responders. Thirteen of 742 (1.8%) procedures were complicated by unexpected events, including three (0.4%) major complications and one fatality (0.1%). In conclusion, SEEG is a safe and efficient methodology for invasive definition of the epileptogenic zone in the most challenging patients. Despite the progressive increase of MRI-negative cases, the proportion of seizure-free patients did not decrease throughout the years., (© The Author(s) (2019). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: The objective of the study was to evaluate clinical characteristics and outcome of hemispherotomy in children and adolescents with hemispheric refractory epilepsy in an Italian cohort of patients., Methods: We retrospectively evaluated the clinical course and outcome of 92 patients with refractory epilepsy who underwent hemispherotomy in three Italian epilepsy centers between 2006 and 2016. Three different approaches for hemispherotomy were used: parasagittal, modified parasagittal, and lateral., Results: Mean age at epilepsy onset was 1.8 ± 2.51 years, and mean duration of epilepsy prior to surgery was 7.4 ± 5.6 years. Mean age at surgery was 9.2 ± 8.0 years. After a mean follow-up of 2.81 ± 2.4 years, 66 of 90 patients (two lost from follow-up, 73.3%) were seizure-free (Engel class I). The etiology of epilepsy was related to acquired lesions (encephalomalacia or gliosis) in 44 patients (47.8%), congenital malformations (cortical dysplasia, hemimegalencephaly, other cortical malformations) in 38 (41.3%), and progressive conditions (Rasmussen or Sturge-Weber syndrome) in 10 patients (10.9%). Regarding seizure outcome, we could not identify statistically significant differences between vertical and lateral approaches (p = 0.154). Seizure outcome was not statistically different in patients with congenital vs acquired or progressive etiologies (p = 0.43). Acute postoperative seizures (APOS) correlated with poor outcome (p < 0.05). On multivariate analysis, presurgical focal to bilateral tonic-clonic seizures (Odds Ratio (OR) = 3.63, 95% Confidence Interval (CI): 1.86-15.20, p = 0.048) independently predicted seizure recurrence. Twenty-one patients (22.8%) exhibited postoperative complications, with no unexpected and persistent neurological deficit. More than 50% of the patients completely tapered drugs., Significance: Our data confirm hemispherotomy to be a safe and effective procedure in patients with drug resistant epilepsies due to hemispheric lesions. Presurgical focal to bilateral tonic-clonic seizures are the strongest predictor of seizure recurrence after surgery, independently from the type of hemispherotomy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Several attempts have been made to coregister in vivo MRI with the histopathology of surgical samples, aiming to validate new MRI biomarkers and improve the detection of epileptogenic lesions. As a further implementation, we propose a method to reconstruct the anatomical localization of the intracerebral electrodes on the histological sections, developing a coregistration protocol to match the in vivo MRI onto the ex vivo MRI obtained from the surgical specimen., New Method: Since the ex vivo MRI is natively in geometrical correspondence with histology slices, the goal of the coregistration process is to compute the transform function mapping the clinical MRI space to the ex vivo MRI. Electrodes and leads, identified in CT-MRI, can then be segmented and translated onto the histological slices., Results: Step-by-step, qualitative visual inspection showed an improved matching of the anatomical structures or boundaries and electrodes positions between the two modalities. The quantitative evaluation of the coregistration protocol reported a mean error ranging between 0.82 and 1.27 mm when a sufficient number of landmarks, particularly in the core of the specimen, were clearly identified., Comparison With Existing Methods: Because histology was performed according to ex vivo MRI geometry we chose to transform the in vivo onto the ex vivo MRI, differently from other methods., Conclusions: Interesting applications of the method will include correlating the locally-generated pathological electrical activity with the subtle morphological alterations of the tissue, and histologically validating the origin of signal alterations or quantitative parameter variations in MRI studies., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: To analyze the attitude and results of Italian epilepsy surgery centers in the surgical management of "low grade epilepsy associated neuroepithelial tumors" (LEATs)., Methods: We conducted a retrospective study enrolling 339 consecutive patients with LEATs who underwent surgery between January 2009 and June 2015 at eight Italian epilepsy surgery centers. We compared demographic, clinical, pathologic, and surgical features of patients with favorable (Engel class I) and unfavorable (Engel class II, III, and IV) seizure outcome. In addition, we compared patients with tumor-associated focal cortical dysplasia (FCD) and patients with solitary tumors to identify factors correlated with FCD diagnosis., Results: Fifty-five (98.2%) of 56 patients with medically controlled epilepsy were seizure-free after surgery, compared to 249 (88.0%) of 283 patients with refractory epilepsy. At multivariate analysis, three variables independently predict unfavorable seizure outcome in the drug-resistant group. Age at surgery is largely the most significant (p = 0.001), with an odds ratio (OR) of 1.04. This means that the probability of seizure recurrence grows by 4% for every waited year. The resection site is also significant (p = 0.039), with a relative risk (RR) of 1.99 for extratemporal tumors. Finally, the completeness of tumor resection has a trend toward significance (p = 0.092), with an RR of 1.82 for incomplete resection. Among pediatric patients, a longer duration of epilepsy was significantly associated with preoperative neuropsychological deficits (p < 0.001). A statistically significant association was observed between FCD diagnosis and the following variables: tailored surgery (p < 0.001), temporal resection (p = 0.001), and surgical center (p = 0.012)., Significance: Our nationwide LEATs study gives important insights on factors predicting seizure outcome in refractory epilepsy and determining variability in FCD detection. Timely surgery, regardless of pharmacoresistance and oriented to optimize epileptologic, neuropsychological, and oncologic outcomes should be warranted., (Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.)

Objective: To report the presurgical workup, surgical procedures, and outcomes in a series of pediatric patients with drug-resistant epilepsy involving the perisylvian/insular regions., Methods: We retrospectively assessed 16 pediatric patients affected by drug-resistant focal epilepsy involving perisylvian/insular regions, who consecutively underwent tailored resective surgery. All patients underwent a detailed presurgical workup, which included the analysis of the anatomoelectroclinical correlations with scalp electroencephalography (EEG) and/or with stereo-electroencephalography (SEEG), brain magnetic resonance imaging (MRI), and comprehensive cognitive and neuropsychological evaluations. After surgery, all patients underwent serial clinical and laboratory evaluations., Results: Focal motor seizures restricted to perioral area, associated with symptoms related to the surrounding areas (as auditory hallucinations, unpleasant paresthesia, fear, and epigastric sensation), characterized the ictal semiology in 75% of patients. In 50%, autonomic manifestations were present and in 56% subjective manifestations were reported. The 50% of the patients underwent SEEG with insular sampling to better define the epileptogenic zone. In all patients, the insular cortex was always part of the epileptogenic zone, and tailored resections also involved, with variable degree, the frontal, parietal, and temporal opercula. Preoperatively, the neuropsychological assessment revealed impairments in specific cognitive functions and mild or moderate cognitive compromise in 88% of the patients. Postoperatively, one patient had permanent slight hemiparesis. At the most recent follow-up (median 39 months), seizure outcome was satisfactory in 69% of patients: seven patients were completely seizure-free (Engel class Ia), two were free of disabling seizure (class Ic), and two had rare disabling seizures (class II). The cognitive functioning remained unchanged in 62%, and improved in 38%., Significance: The assessment of perisylvian/insular epilepsy in children is particularly challenging. However, tailored resections based on a careful presurgical evaluation, including SEEG recording, may lead to a good seizure control and to a better overall outcome., (Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.)

Introduction: Posterior reversible encephalopathy syndrome (PRES) with status epilepticus may occur after liver transplant. This may rarely lead to refractory epilepsy and hippocampal sclerosis (HS)., Case Description: We report the first case of epilepsy surgery in a liver-transplanted patient with refractory temporal lobe epilepsy. A 3-year-old girl underwent liver transplant for congenital biliary atresia. Four days after transplant she manifested PRES with status epilepticus, but she recovered within a couple of weeks. At the age of 5 years she started presenting complex partial seizures, that became refractory to antiepileptic drugs (AED), worsening psychosocial performances. The pre-surgical work-up identified a left HS and temporal pole alterations. A left antero-mesial temporal lobectomy was performed, leading to epilepsy remission and allowing AED withdrawal., Conclusion: Drug-resistant temporal lobe epilepsy and HS may occur as sequelae of PRES with status epilepticus related to liver transplant and cyclosporine use. In this setting early epilepsy surgery may reduce the time of chronic exposure to AED and severe illness due to repeated seizures. This option might have additional advantages in the subgroup of epileptic patients with liver transplant, preserving the liver from the potential damage due to multiple AED trials and their interaction with commonly used immunosuppressant drugs., (Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Objective: Hippocampal sclerosis (HS) is the most frequent neuropathologic finding in patients undergoing surgery for intractable temporal lobe epilepsy (TLE). The International League Against Epilepsy (ILAE) has recently proposed a new classification of HS based on specific patterns of cell loss. The aim of this study was to investigate the relationships between HS types, their etiologic factors, and the short- and long-term postsurgical outcomes of patients undergoing surgery because of drug-resistant TLE with HS., Methods: Two hundred thirteen patients with a neuropathologic diagnosis of HS and a minimum follow-up of 2 years were divided on the basis of their ILAE HS type and further classified into: (1) isolated HS, (2) HS associated with focal cortical dysplasia (FCD IIIa), or (3) HS associated with other lesions. Their clinical and neuropathologic data were correlated with their Engel class postsurgical outcomes., Results: The main findings were the following: (1) HS type 1 was associated with a longer duration of epilepsy; (2) >80% of the patients had an Engel class I short- and long-term outcomes, regardless of HS type and associated pathology; (3) short- and long-term postsurgical outcomes were less satisfactory in the patients who were completely seizure-free (Engel class Ia), and patients with HS type 2 had better long-term seizure outcomes than those with type 1; (4) the concomitant presence of FCD contributed to a worse outcome, regardless of HS type; and (5) a shorter duration of epilepsy significantly correlated with an Engel class Ia outcome., Significance: These data suggest that HS type and associated pathologies may predict the risk of recurrence, but other variables such as the duration of epilepsy need to be considered. A common neuropathologic classification system may help to identify preoperative predictive factors and improve the selection of patients who may benefit from epilepsy surgery., (Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.)

The aim of this retrospective case series analysis was to identify the predictors of postoperative depression (PostOp-D) in a sample of 248 subjects with focal drug-resistant focal epilepsy. The presence or absence of PostOp-D during a 12-month follow-up period was the outcome variable. Demographic, neurologic, psychiatric characteristics, and antiepileptic therapy were the explanatory variables. After preliminary bivariate analysis, a multivariate logistic regression model was fitted to identify variables associated with PostOp-D. Sixty-seven patients (27%) experienced PostOp-D. At multivariate analysis, lifetime depression, age at surgery, and levetiracetam (LEV) are positive predictors of PostOp-D; carbamazepine (CBZ) and anxiety disorders are protective factors. LEV increases the risk for PostOp-D by about half; the relative risk (RR) is 1.48. Conversely, CBZ decreases the risk for PostOp-D by about half (RR 0.59). Our results suggest that careful psychiatric evaluation and follow-up should be recommended for subjects at risk. It is advisable to treat patients with depression before surgery. Antiepileptic drugs should be selected carefully when patients present with not modifiable risk factors, such as positive personal history for depression., (Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.)