Excellent Seizure Outcome Research Articles

Subpial resection of epileptogenic tubers: operative nuances and lessons learned.

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder. Tubers of the central nervous system are a hallmark of the disorder and often cause epilepsy. Many TSC patients fail to achieve seizure control with medication alone. Several case series have demonstrated high seizure freedom rates after resective surgery. However, the technique for the resection of epileptogenic tubers has largely been unreported. Here the authors present 2 cases to illustrate their multistage approach for localizing and resecting the seizure onset zone in patients with TSC. At their institution, they have excellent seizure outcomes and a low complication rate with this technique. The video can be found here: https://stream.cadmore.media/r10.3171/2024.4.FOCVID2411.

Predicting seizure outcomes and functional outcomes after hemispherotomy: are we any better?

Present study attempted to analyze seizure freedom and detailed functional outcomes after functional hemispherotomy and utility of hemispherotomy outcome prediction scale (HOPS) scores in predicting outcomes. Patients who underwent functional hemispherotomy were analyzed for clinical presentation, neuroimaging, seizure outcomes, and functional outcomes. A total of 76 procedures were performed on 69 patients. Mean age at the surgery was 8 ± 6.1years. Fourteen patients were < 2years. Age of onset epilepsy of the cohort was 2.0 ± 3.3years. All had severe catastrophic epilepsy with multiple daily seizures. All patients had motor deficits with 36 (52%) patients had contralateral dysfunctional hand. Perinatal stroke (49%) was most common substrate followed by cortical malformations (21.7%). Eight patients had contralateral imaging abnormalities. Fifty-nine (86.76%) patients remained seizure free (Engle 1a) at 41 + -20.9 months. HOPS scores were available for 53 patients and lowest seizure outcome was 71% for HOPS score of 4. Lower HOPS scores predicted better seizure outcomes. Cortical malformations operated earlier than 2years predicted poor seizure outcomes (66.6%). Positive functional outcomes are recorded in 80% of patients with 78% reporting improvement from the pre-surgical level. Five (7.2%) patients underwent shunt surgery. One mortality recorded. Hemispherotomy has excellent seizure outcomes. Early surgery in cortical malformations appears to be predictor of poorer seizure outcomes. HOPS score is a good tool to predict the seizure outcomes. Hemispherotomy is perceived to improve the Cognitive and functional performance.

Outcomes of resective surgery in pediatric patients with drug-resistant epilepsy: A single-center study from the Eastern Mediterranean Region.

Epilepsy surgery is widely accepted as an effective therapeutic option for carefully selected patients with drug-resistant epilepsy (DRE). There is limited data on the outcome of epilepsy surgery, especially in pediatric patients from the Eastern Mediterranean region. Hence, we performed a retrospective study examining the outcomes of resective surgery in 53 pediatric patients with focal DRE. Patients with focal DRE who had undergone epilepsy surgery were included in the present study. All patients underwent a comprehensive presurgical evaluation. Postoperative seizure outcomes were classified using the Engel Epilepsy Surgery Outcome Scale. After surgery, 33 patients (62.2%) were Class I according to the Engel classification of surgical outcomes; eight patients (15.0%) were Class II, 11 (20.7%) were Class III, and one (1.8%) was Class IV. The relationships of presurgical, surgical, and postsurgical clinical variables to seizure outcomes were compared. Older age at seizure onset, older age at the time of surgery, the presence of focal to bilateral tonic-clonic seizures, seizure duration over 2 minutes, unsuccessful treatment with three or fewer antiseizure medications, lesions confined to one lobe (as demonstrated via magnetic resonance imaging [MRI]), surgical site in the temporal lobe, and histopathology including developmental tumors were significantly linked to an Engel Class I outcome. A univariate analysis of excellent surgical outcomes showed that lateralized semiology, localized interictal and ictal electroencephalogram (EEG) discharges, lateralized single-photon emission computed tomography and positron emission tomography findings, and temporal lobe resections were significantly related to excellent seizure outcomes. The results of our study are encouraging and similar to those found in other centers around the world. Epilepsy surgery remains an underutilized treatment for children with DRE and should be offered early.

Central quadrantotomy for intractable childhood epilepsy: operative technique and functional neuroanatomy.

Refractory subhemispheric epilepsy has been traditionally treated by resection. The last few decades have seen the emergence of disconnective techniques, for both hemispheric and subhemispheric disease. The aim of this study was to describe the technique for a disconnective surgery for large epileptogenic lesions involving the central (perirolandic cortices), parietal, and occipital lobes. This junctional cortex within the hemisphere (in contrast to anterior and posterior quadrantotomies) presents unique challenges when contemplating a complete disconnection of the region. The surgical technique is achieved through six distinct steps: fronto-central, inferior frontoparietal, lateral temporo-occipital, medial frontal, basal temporo-occipital, and posterior parasagittal callosal disconnections. The functional neuroanatomy of each step is described, along with cadaveric dissections. The authors describe this technique and include a case description of a young girl who presented with childhood-onset intractable epilepsy associated with cognitive stagnation. The postoperative seizure outcome in this patient remains excellent at 2 years' follow-up, with gains in cognition and behavior. Excellent seizure outcomes can be achieved if the network encompassing the entire epileptogenic cortex is disconnected while ensuring preservation of fiber systems that link functionally eloquent uninvolved cortices adjacent to the central quadrant.

Hemispherotomy in Infants with Hemimegalencephaly: Long-Term Seizure and Developmental Outcome in Early Treated Patients.

Hemimegalencephaly (HME) is a rare brain congenital malformation, consisting in altered neuronal migration and proliferation within one hemisphere, which is responsible for early onset drug-resistant epilepsy. Hemispherotomy is an effective treatment option for patients with HME and drug-resistant epilepsy. Surgical outcome may be variable among different surgical series, and the long-term neuropsychological trajectory has been rarely defined using a standardized neurocognitive test. We report the epileptological and neuropsychological long-term outcomes of four consecutive HME patients, operated on before the age of three years. All patients were seizure-free and drug-free, and the minimum follow-up duration was of five years. Despite the excellent post-surgical seizure outcome, the long-term developmental outcome is quite variable between patients, ranging from mild to severe intellectual disabilities. Patients showed improvement mainly in communication skills, while visuo-perceptive and coordination abilities were more impaired. Epileptological outcome seems to be improved in early treated patients; however, neuropsychological outcome in HME patients may be highly variable despite early surgery.

Precision medicine in pediatric temporal epilepsy surgery: optimization of outcomes through functional MRI memory tasks and tailored surgeries.

The goal of epilepsy surgery is both seizure cessation and maximal preservation of function. In temporal lobe (TL) cases, the lack of functional MRI (fMRI) tasks that effectively activate mesial temporal structures hampers preoperative memory risk assessment, especially in children. This study evaluated pediatric TL surgery outcome optimization associated with tailored resection informed by an fMRI memory task. The authors identified focal onset TL epilepsy patients with 1) TL resections; 2) viable fMRI memory scans; and 3) pre- and postoperative neuropsychological (NP) evaluations. They retrospectively evaluated preoperative fMRI memory scans, available Wada tests, pre- and postoperative NP scores, postoperative MRI scans, and postoperative Engel class outcomes. To assess fMRI memory task outcome prediction, the authors 1) overlaid preoperative fMRI activation onto postoperative structural images; 2) classified patients as having "overlap" or "no overlap" of activation and resection cavities; and 3) compared these findings with memory improvement, stability, or decline, based on Reliable Change Index calculations. Twenty patients met the inclusion criteria. At a median of 2.1 postoperative years, 16 patients had Engel class IA outcomes and 1 each had Engel class IB, ID, IIA, and IID outcomes. Functional MRI activation was linked to NP memory outcome in 19 of 20 cases (95%). Otherwise, heterogeneity characterized the cohort. Functional MRI memory task activation effectively predicted individual NP outcomes in the context of tailored TL resections. Patients had excellent seizure and overall good NP outcomes. This small study adds to extant literature indicating that pediatric TL epilepsy does not represent a single clinical syndrome. Findings support individualized surgical intervention using fMRI memory activation to help guide this precision medicine approach.

Lateral versus vertical hemispheric disconnection for epilepsy: a systematic review and meta-analysis.

Lateral periinsular hemispherotomy (LPH) and vertical parasagittal hemispherotomy (VPH) are the most popular disconnective techniques for intractable epilepsies associated with unilateral hemispheric pathologies. The authors aimed to investigate possible differences in seizure outcome and complication rates between patients who underwent LPH and VPH. A comprehensive literature search of PubMed and Embase identified English-language articles published from database inception to December 2019 that reported series (minimum 12 patients with follow-up ≥ 12 months) on either LPH or VPH. Pooled rates of seizure freedom and complications (with a particular focus on hydrocephalus) were analyzed using meta-analysis to calculate both fixed and random effects. Heterogeneity (Cochran's Q test) and inconsistency (fraction of Q due to actual heterogeneity) were also calculated. Twenty-five studies were included. Data from 825 patients were available for seizure outcome analysis (583 underwent LPH and 242 underwent VPH), and data from 692 patients were available for complication analysis (453 underwent LPH and 239 underwent VPH). No differences were found in the pooled rates of Engel class I seizure outcome between patients who underwent LPH (80.02% and 79.44% with fixed and random effects, respectively) and VPH (79.89% and 80.69% with fixed and random effects, respectively) (p = 0.953). No differences were observed in the pooled rates of shunted hydrocephalus between patients who underwent LPH (11.34% and 10.63% with fixed and random effects, respectively) and VPH (11.07% and 9.98% with fixed and random effects, respectively) (p = 0.898). Significant heterogeneity and moderate inconsistency were determined for hydrocephalus occurrence in patients who underwent both LPH and VPH. LPH and VPH techniques present similar excellent seizure outcomes, with comparable and acceptable safety profiles.

Seizure Outcomes and Reoperation in Surgical Rasmussen Encephalitis Patients.

Rasmussen encephalitis (RE) is a rare inflammatory disease affecting one hemisphere, causing progressive neurological deficits and intractable seizures. To report long-term seizure outcomes, reoperations, and functional outcomes in patients with RE who underwent hemispherectomy at our institution. Retrospective review was performed for all patients with RE who had surgery between 1998 and 2020. We collected seizure history, postoperative outcomes, and functional data. Imaging was independently reviewed in a blinded fashion by 2 neurosurgeons and a neuroradiologist. We analyzed 30 patients with RE who underwent 35 hemispherectomies (5 reoperations). Using Kaplan-Meier analysis, seizure-freedom rate was 81.5%, 63.6%, and 55.6% at 1, 5, and 10 years after surgery, respectively. Patients with shorter duration of hemiparesis preoperatively were less likely to be seizure-free at follow-up (P = .011) and more likely to undergo reoperation (P = .004). Shorter duration of epilepsy (P = .026) and preoperative bilateral MRI abnormalities (P = .011) were associated with increased risk of reoperation. Complete disconnection of diseased hemisphere on postoperative MRI after the first operation improved seizure-freedom (P = .021) and resulted in fewer reoperations (P = .034), and reoperation resulted in seizure freedom in every case. Obtaining complete disconnection is critical for favorable seizure outcomes from hemispherectomy, and neurosurgeons should have a low threshold to reoperate in patients with RE with recurrent seizures. Rapid progression of motor deficits and bilateral MRI abnormalities may indicate a subpopulation of patients with RE with increased risk of needing reoperation. Overall, we believe that hemispherectomy is a curative surgery for the majority of patients with RE, with excellent long-term seizure outcome.

Epilepsy surgery for drug-resistant temporal lobe epilepsy in over-50 year-olds: Seizure outcome, surgical complications and neuropsychological outcome

ObjectiveSurgery is an effective treatment for drug-resistant temporal-lobe epilepsy (TLE), but is still underutilized for older patients because of a perceived higher rate of perioperative complications, cognitive decline and worse seizure outcome. MethodsWe retrospectively screened all patients operated on in our institution for drug-resistant TLE between 2007 and 2019. Data of patients aged ≥50 years versus <50 years at surgery were compared. The primary endpoint was freedom from disabling seizure (Engel I) at 2 years postoperatively. ResultsIn patients aged ≥50 years (n=19), mean age at surgery was 54.9 years and mean disease duration was 36.6 years. At 2 years postoperatively, rates of Engel I seizure outcome were not significantly different between the two groups (73.9% in the <50 years group versus 94.4% in the ≥50 years group). Although surgical complications were significantly (47.4%) in the older patients, neurological deficit was permanent in only 5.3% of cases. At 1 year postoperatively, neuropsychological outcome did not significantly differ between the two groups. ConclusionsPatients aged ≥50 years had an excellent seizure outcome at 2 years postoperatively. Early postoperative complications were more frequent in patients aged ≥50 years but were mostly transient. Cognitive outcome was similar to that in younger patients. These findings strongly suggest that age ≥50 years should not be an exclusion criterion for resective epilepsy surgery in patients with drug-resistant TLE.

Pediatric hemispherectomy outcome: Adaptive functioning, intelligence, and memory

ObjectiveOur purpose was to characterize neuropsychological evaluation (NP) outcome following functional hemispherectomy in a large, representative cohort of pediatric patients. MethodsWe evaluated seizure and NP outcomes and medical variables for all post-hemispherectomy patients from Seattle Children’s Hospital epilepsy surgery program between 1996 and 2020. Neuropsychological evaluation outcome tests used were not available on all patients due to the diversity of patient ages and competency that is typical of a representative pediatric cohort; all patients had at least an adaptive functioning or intelligence measure, and a subgroup had memory testing. ResultsA total of 71 hemispherectomy patients (37 right; 34 females) yielded 66 with both preoperative (PREOP) plus postoperative (POSTOP) NPs and 5 with POSTOP only. Median surgery age was 5.7 (IQR 2–9.9) years. Engel classification indicated excellent seizure outcomes: 59 (84%) Class I, 6 (8%) Class II, 5 (7%) Class III, and 1 (1%) Class IV. Medical variables – including seizure etiology, surgery age, side, presurgical seizure duration, unilateral or bilateral structural abnormalities, secondarily generalized motor seizures – were not associated with either Engel class or POSTOP NP scores, though considerable heterogeneity was evident. Median PREOP and POSTOP adaptive functioning (PREOP n = 45, POSTOP n = 48) and intelligence (PREOP n = 29, POSTOP n = 36) summary scores were exceptionally low and did not reveal group decline from PREOP to POSTOP. Fifty-five of 66 (85%) cases showed stability or improvement. Specifically, 5 (8%) improved; 50 (76%) showed stability; and 11 (16%) declined. Improve and decline groups showed clinically interesting, but not statistical, differences in seizure control and age. Median memory summary scores were low and also showed considerable heterogeneity. Overall median PREOP to POSTOP memory scores (PREOP n = 16, POSTOP n = 24) did not reveal declines, and verbal memory scores improved. Twenty six percent of intelligence and 33% of memory tests had verbal versus visual-spatial discrepancies; all but one favored verbal, regardless of hemispherectomy side. SignificanceThis large, single institution study revealed excellent seizure outcome in 91% of all 71 patients plus stability and/or improvement of intelligence and adaptive functioning in 85% of 66 patients who had PREOP plus POSTOP NPs. Memory was similarly stable overall, and verbal memory improved. Medical variables did not predict group NP outcomes though heterogeneity argues for further research. This study is unique for cohort size, intelligence plus memory testing, and evidence of primacy of verbal over visual-spatial development, despite hemispherectomy side. This study reinforces the role of hemispherectomy in achieving good seizure outcome while preserving functioning.

Two-trajectory laser amygdalohippocampotomy: Anatomic modeling and initial seizure outcomes.

Laser interstitial thermal therapy (LITT) for mesial temporal lobe epilepsy (mTLE) is typically performed with one trajectory to target the medial temporal lobe (MTL). MTL structures such as piriform and entorhinal cortex are epileptogenic, but due to their relative geometry, they are difficult to target with one trajectory while simultaneously maintaining adequate ablation of the amygdala and hippocampus. We hypothesized that a two-trajectory approach could improve ablation of all relevant MTL structures. First, we created large-scale computer simulations to compare idealized one- vs two-trajectory approaches. A two-trajectory approach was then validated in an initial cohort of patients. We used magnetic resonance imaging (MRI) from the Human Connectome Project (HCP) to create subject-specific target structures consisting of hippocampus, amygdala, and piriform/entorhinal/perirhinal cortex. An algorithm searched for safe potential trajectories along the hippocampal axis (catheter one) and along the amygdala-piriform axis (catheter two) and compared this to a single trajectory optimized over all structures. The proportion of each structure ablated at various burn radii was evaluated. A cohort of 11 consecutive patients with mTLE received two-trajectory LITT; demographic, operative, and outcome data were collected. The two-trajectory approach was superior to the one-trajectory approach at nearly all burn radii for all hippocampal subfields and amygdala nuclei (p<.05). Two-laser trajectories achieved full ablation of MTL cortical structures at physiologically realistic burn radii, whereas one-laser trajectories could not. Five patients with at least 1year of follow-up (mean=21.8months) experienced Engel class I outcomes; 6 patients with less than 1year of follow-up (mean=6.6months) are on track for Engel class I outcomes. Our anatomic analyses and initial clinical results suggest that LITT amygdalohippocampotomy performed via two-laser trajectories may promote excellent seizure outcomes. Future studies are required to validate the long-term clinical efficacy and safety of this approach.

Surgical outcomes for medically refractory epilepsy secondary to posterior cortex ulegyria as sequelae of perinatal insults

BackgroundTo study surgical outcomes in pharmaco-resistant epilepsy associated with posterior cortex ulegyria secondary to perinatal insults. MethodsA cohort was analysed for clinico-radiological charectaristics, surgical interventions and seizure outcomes. ObservationsA total of 38 patients underwent surgery, divided as group A - curative surgeries (n = 20) and group B - palliative surgeries (n = 18). Mean age of onset of epilepsy in group A was 5.2 ± 3.4 years against 2.7 ± 2.4 years in group B (p < 0.01). Electroclinical Lennox Gastaut Syndrome was encountered in 9/20 patients in group A, against all 18 patients in group B. Disabling reflex epilepsy was seen in 10 (26 %) patients.Interictal electrophysiology localized in the posterior cortex in all patients in group A, but ictal onsets contributed in only 7/20 patients. Nine patients from group A had unilateral parieto-occipital ulegyria while bilateral in 11/20 patients, and 16/18 from group B. Group A patients underwent parieto-occipital resection (n = 10) and temporo-parieto-occipital disconnection (n = 10) while group B underwent complete corpus callosotomy (n = 18).In group A, Engel Ia outcome was achieved in 15/20 patients (75 %) at mean follow up of 23.5 ± 7.9 months. Group B patients experienced cessation of head drops in all 18 patients, with two-third reduction in seizure frequency at 29.2± 12.4 months of mean follow up. Reflex seizures responded completely in both groups. ConclusionsEpilepsy surgeries for posterior cortex ulegyria results in excellent seizure outcomes. Corpus callosotomy appears highly effective as a palliation for head drop as well as disabling reflex seizures in a well selected cohort.

Operative variations in temporal lobe epilepsy surgery and seizure and memory outcome in 226 patients suffering from hippocampal sclerosis

ABSTRACT Objective: The aim of this retrospective cohort study was to assess seizure and memory outcomes following temporal lobe surgery in patients suffering from medically refractory temporal lobe epilepsy (TLE) and hippocampal sclerosis (HS). Methods: A retrospective monocentric data analysis was performed in consecutive patients who were operated on during 2002–2018. In the first decennium, standard temporal lobe resections (TLR) were predominately performed, and later, antero-temporal lobe resections (ATLR) were mainly performed. Seizure and memory outcomes over time were assessed according to ILAE/Engel classification and the Berlin Amnesia Test (BTA), respectively. Results: Altogether, 231 surgeries were performed on 226 patients (mean age, 40 years [range, 10–68 years]; male: female, 1:1.4; mean seizure duration, 25 years; and mean follow-up duration, 4.75 years [range, 1–16]). Recently, outcomes of 78.3% of the patients in the total cohort were classified as Engel class I, with 54.9% of patients being completely seizure free. The recent cohort of ATLR since 2012 showed significant more completely seizure-free patients than before 2012 (Engel IA 46.6% versus 67.7%, p < 0.0025, χ 2), although the Kaplan Meier analysis of all patients favors TLR for better seizure outcome (61% ATLR vs 73% TLR seizure free after 5 yrs, log rank p < 0.001). Verbal memory improved significantly in non-dominant patients. Minor neurological complications were noted (permanent severe complications, 0.4%; temporary severe complications, 4.8%). Conclusion: Significant improvements in seizure and memory outcomes were observed over time, with surgical technique and seizure duration as important prognostic factors. Early admittance for surgery may favor an excellent seizure outcome in patients undergoing temporal lobe resection for HS.

Insular seizures and epilepsies: Ictal semiology and minimal invasive surgery.

The increased identification of seizures with insular ictal onset, promoted by the international development of stereo-electroencephalography (SEEG), has led to the recent description of larger cohorts of patients with insular or insulo-opercular epilepsies than those previously available. These new series have consolidated and extended our knowledge of the rich ictal semiology and diverse anatomo-clinical correlations that characterized insular seizures. In parallel, some experiences have been gained in the surgical treatment of insular epilepsies using minimal invasive procedures. The large majority of patients present with auras (mostly somatosensory and laryngeal) and motor signs (predominantly elementary and orofacial), an underlying focal cortical dysplasia, and an excellent postoperative seizure outcome. Many other subjective and objective ictal signs, known to occur in other forms of epilepsies, are also observed and clustered in five patterns, reflecting the functional anatomy of the insula and its overlying opercula, as well as preferential propagation pathways to frontal or temporal brain regions. A nocturnal predominance of seizure is frequently reported, whereas secondary generalization is infrequent. Some rare ictal signs are highly suggestive of an insular origin, including somatic pain, reflex seizures, choking spells, and vomiting. Minimal invasive surgical techniques have been applied to the treatment of insular epilepsies, including Magnetic Resonance Imaging-guided laser ablation (laser interstitial thermal therapy (LITT)), radiofrequency thermocoagulation (RFTC), gamma knife radiosurgery, and responsive neurostimulation. Rates of seizure freedom (about 50%) appear lower than that reported with open-surgery (about 80%) with yet a significant proportion of transient neurological deficit for LITT and RFTC. Significant progress has been made in the identification and surgical treatment of insular and insulo-opercular epilepsies, including more precise anatomo-clinical correlations to optimally plan SEEG investigations, and experience in using minimal invasive surgery to reduce peri-operative morbidity.

Multiple hippocampal transections for refractory pediatric mesial temporal lobe epilepsy: seizure and neuropsychological outcomes.

Temporal lobe epilepsy (TLE) is the most common focal epilepsy across adult and pediatric age groups. It is also the most amenable to surgery, with excellent long-term seizure outcome. Most TLE cases have an epileptogenic zone in the mesial temporal structures, namely the hippocampus. Resecting the dominant hippocampus has been shown to be associated with significant verbal memory deficits, especially in patients with intact verbal memory scores presurgically. Multiple hippocampal transection (MHT) is a relatively new surgical technique designed to interrupt the longitudinal hippocampal circuitry involved in seizure propagation yet preserve the circular fibers involved in memory function. This technique has been used to treat mesial TLE in both dominant- and nondominant-hemisphere cases, almost exclusively in adults. It has been applied to normal and sclerotic hippocampi. In this study, information on 3 pediatric patients who underwent MHT for mesial TLE at Children's Wisconsin between 2017 and 2018 is included. Clinical, electroencephalographic, and neuropsychological features and outcomes are described in detail. MRI revealed a tumor in the amygdala with a normal hippocampus in 1 patient and hippocampal sclerosis in 2 patients. All patients underwent stereoelectroencephalography confirming the involvement of the hippocampus in seizure onset. MHTs were completed under intraoperative monitoring, with amygdala and temporal tip resection in all patients due to early spread to these regions. All patients had excellent seizure outcomes at 1 year, and 2 of the 3 patients remain seizure free at last follow-up (range 20-36 months), all with stable or improved neuropsychological profiles, including verbal memory. MHT is a relatively new surgical procedure designed to preserve essential memory circuitry while disrupting seizure propagation pathways in the hippocampus. A growing body of literature shows good seizure and neuropsychological results, but mainly in adults. This is the first series of MHTs used exclusively in children at one medical center, showcasing excellent seizure control and preservation of neuropsychological functioning. One of the patients is also the first described to have MHT in the setting of an amygdalar tumor abutting the hippocampus, further expanding the pathological setting in which MHT can be used effectively.

Clinical features and electroclinical evolution in 22 cases with epileptic spasms without hypsarrhythmia.

This study aimed to investigate the general presentation of epileptic spasms without hypsarrhythmia (ESwoH) and retrospectively determine whether there are differences in treatment effects related to ACTH therapy, long-term seizure outcome, and evolution of EEG features according to pre-treatment EEG patterns. According to the pattern of background activity, we divided our cohort into two groups: Group 1: normal background activity or with localized intermittent slow waves; Group 2: intermittent slow waves appearing generalized or in two or more lobes. Subjects included 22 children (Group 1: n=10; Group 2: n=12) diagnosed with ESwoH who received treatment from 2007 to 2017. The median age at onset of epileptic spasms was 5.5 months and the follow-up period lasted for 40.5 months. ACTH therapy was performed for seven patients from Group 1 and eight patients from Group 2. Only one patient from Group 2 responded to ACTH. Patients receiving effective treatments at early stages had excellent seizure outcome. Refractory cases included six patients in Group 1 and eight patients in Group 2; subsequent follow-up EEGs indicated hypsarrhythmia in one patient in Group 1 (17%) and six patients (75%) in Group 2, including one patient whose EEG pattern indicated progression to Lennox-Gastaut syndrome. Overall, ACTH is ineffective for patients with epileptic spasms without hypsarrhythmia. The EEG may indicate possible future development of hypsarrhythmia if epileptic spasms are resistant to treatment, especially in patients with diffuse slow waves on pre-treatment EEG. The efficacy of treatment introduced at early stages from onset may predict long-term seizure outcome.

Examining the Utility of Resective Epilepsy Surgery in Children With Electrical Status Epilepticus in Sleep: Long Term Clinical and Electrophysiological Outcomes.

Background: Electrical Status Epilepticus in Sleep (ESES) is an epileptic encephalopathy syndrome characterized by infrequent clinical seizures and prominent interictal burden during slow wave sleep associated with cognitive deficits and behavioral dysfunction. Medical treatment with anti-epileptic drugs is often unsuccessful. Resective surgery may be a valuable option in carefully selected patients. This case series aims to describe the indications, long term results and utility of resective surgery for ESES.Methods: Information on 14 patients who underwent surgery for epilepsy and ESES at the Children's Hospital of Wisconsin between 2007 and 2017 is included. Clinical, electrographic and neuropsychological features and outcomes are described in detail.Results: The most common pathology was encephalomalacia due to perinatal middle cerebral artery stoke (5/14). Twelve patients had imaging findings of perinatal pathologies; however, two patients had normal magnetic resonance imaging. Surgery was performed to control refractory epilepsy in eight patients. Six patients had no clinical seizures for 1–6 years prior to surgery, one of which had no known clinical seizures at all. All showed cognitive declines (6/14) or impairment (8/14) on neuropsychological assessments, and surgery was suggested to minimize further cognitive declines. The most common surgical procedure was hemispherotomy (10/14). Temporo-parieto-occipital disconnection, frontal lobectomy, parieto-occipital resection, and limited corticectomy were also used, with good outcomes for the first three procedures. Clinical follow up mean was 4.4 years and 12 patients had excellent seizure outcome. Electroencephalography (EEG) follow up mean was 3 years and ESES resolved in 12/14 patients. All patients completed post-surgical neuropsychological evaluation with mean follow-up of 17.46 months.Conclusions: Resective surgery is an effective treatment for selected cases of ESES, producing long term seizure freedom, resolution of ESES and stabilization of cognitive and behavioral functioning in most patients. Our case series is the largest single center cohort description addressing resective surgery for ESES. Outcomes in this sample suggest that good long-term seizure, EEG and cognitive/behavioral outcomes can be achieved in patients with normal brain imaging and in limited lobar or multi-lobar resections. Moreover, patients with ESES and very infrequent clinical seizures can benefit from surgery with stabilization of cognitive and behavioral functioning.

Neuronavigation-assisted surgical treatments for medically refractory epilepsy: Single-hospital experience with 4 surgical approaches

ObjectiveSurgical treatment should be considered for patients with medically refractory epilepsy, and neuronavigation may benefit and reduce the technical difficulties during surgery. In this study, we aimed to report our single-hospital experience of incorporating neuronavigation for treating patients with medically refractory epilepsy using 4 types of surgery. Patients and methodsPatients who were diagnosed as medically refractory epilepsy and received neuronavigation-assisted surgery were included in this retrospective analysis. The type of surgery was decided by the surgery committee after careful evaluation and discussion, including temporo-parietal-occipital (TPO) disconnection, anterior subtotal callosal section, functional hemispherectomy and resection of the epileptogenic zone(s). Postoperative seizure outcome at the last visit was evaluated using Engel classification. ResultsA total of 173 patients with medically refractory epilepsy who were treated surgically under the assistance of neuronavigation were included. The majority type of surgery was resection of epileptic zone, n = 104 (60.12%). An excellent seizure outcome, Engel Class I was found in 50.86% of the patients, followed by 23.12% patients with a good outcome of Engel Class II. ConclusionOverall more than half of the patients could have excellent seizure outcome of Engel Class I, the postoperative complications were manageable. These results indicated that the applicability of neuronavigation, and the use of neuronavigation provides good efficacy and safety for all kinds of surgical procedures for patients with medically refractory epilepsy.

Surgical Treatment of Extratemporal Epilepsy: Results and Prognostic Factors.

Surgery is a widely accepted option for the treatment of pharmacoresistant epilepsies of extratemporal origin. To analyze clinical and epileptological results and to provide prognostic factors influencing seizure outcome. This retrospective single-center study comprises a consecutive series of 383 patients, most of whom had an identifiable lesion on MRI, who underwent resective surgery for extratemporal epilepsy. Data including diagnostic modalities, surgical treatment, histopathology, prognostic factors, and epileptological outcome were analyzed. Resective procedures were located as follows: frontal (n = 183), parietal (n = 44), occipital (n = 24), and insular (n = 24). In 108 cases resection included more than 1 lobe. Histopatholological evaluation revealed focal cortical dysplasias (n = 178), tumors (n = 110), cavernomas (n = 27), gliosis (n = 42), and nonspecific findings (n = 36). A distinct epileptogenic lesion was detected in 338 (88.7%) patients. After a mean follow-up of 54 mo, 227 (62.5%) patients remained free from disabling seizures (Engel class I), and 178 (49%) were completely seizure free (Engel class Ia). There was no perioperative mortality. Permanent morbidity was encountered in 46 cases (11.8%). The following predictors were significantly associated with excellent seizure outcome (Engel I): lesion visible on magnetic resonance imaging (MRI; P = .02), noneloquent location (P = .01), complete resection of the lesion (P = .001), absence of epileptic activity postoperatively (P = .001), circumscribed histological findings (P = .001), lower age at surgery (P = .008), and shorter duration of epilepsy (P = .02). Surgical treatment of extratemporal epilepsy provides satisfying epileptological results with an acceptable morbidity. Best results can be achieved in younger patients with circumscribed MRI lesions, which can be resected completely.

Epilepsy Surgery for Children With Low-Grade Epilepsy-Associated Tumors: Factors Associated With Seizure Recurrence and Cognitive Function

ObjectiveLow-grade epilepsy-associated tumors (LEATs) are associated with childhood seizures that are typically drug-resistant, necessitating surgical interventions. In this study, we aimed to investigate the efficacy of surgical intervention in children with LEATs and to identify factors associated with seizure and cognitive outcomes. MethodsWe reviewed 58 children less than 18 years of age who underwent epilepsy surgery due to histopathologically confirmed LEATs and had a minimum postoperative follow-up duration of 24 months. ResultsOf the 58 patients who were followed for a median duration of 5.6 (IQR 3.2 to 10.0) years, 51 (87.9%) were seizure-free after surgery. In univariate analysis, shorter epilepsy duration, fewer antiepileptic drugs at time of surgery, gross total resection, and unilobar tumor involvement were associated with seizure freedom. In multivariate analysis, gross total resection was independently associated with seizure freedom. The preoperative and postoperative full-scale intelligence quotient (FSIQ) scores were 78.9 ± 27.1 and 80.9 ± 28.7, respectively. In univariate analysis, younger age at seizure onset, longer epilepsy duration, more antiepileptic drugs at time of surgery, multilobar tumor involvement, and presence of generalized epileptic discharges were associated with lower preoperative FSIQ. In multivariate analysis, longer epilepsy duration was independently associated with lower preoperative FSIQ scores. Postoperative FSIQ scores were significantly influenced by preoperative FSIQ scores. ConclusionsEpilepsy surgery for LEATs in children resulted in excellent seizure outcome. Gross total resection was the only independent factor associated with favorable seizure outcome. Preoperative and postoperative cognitive abilities were significantly influenced by epilepsy duration, so early surgical intervention should be considered.