The feasibility and value of extraoperative and adjuvant intraoperative stereoelectroencephalography in rolandic and perirolandic epilepsies.

OBJECTIVE: The objective of this study was to illustrate the feasibility and value of extra- and intraoperative stereoelectroencephalography (SEEG) in patients who underwent resection in rolandic and perirolandic regions. METHODS: The authors retrospectively reviewed all consecutive patients with at least 1 year of postoperative follow-up who underwent extra- and intraoperative SEEG monitoring between January 2015 and January 2017. RESULTS: Four patients with pharmacoresistant rolandic and perirolandic focal epilepsy were identified, who underwent conventional extraoperative invasive SEEG evaluations followed by adjuvant intraoperative SEEG recordings. Conventional extraoperative SEEG evaluations demonstrated ictal and interictal epileptiform activities involving eloquent rolandic and perirolandic cortical areas in all patients. Following extraoperative monitoring, patients underwent preplanned staged resections guided by simultaneous and continuous adjuvant intraoperative SEEG monitoring. Resections, guided by electrode contacts of interest in 3D boundaries, were performed while continuous real-time electrographic data from SEEG recordings were obtained. Staged approaches of resections were performed until there was intraoperative resolution of synchronous rolandic/perirolandic cortex epileptic activities. All patients in the cohort achieved complete seizure freedom (Engel class IA) during the follow-up period ranging from 18 to 50 months. Resection resulted in minimal neurological deficit; 3 patients experienced transient, distal plantar flexion weakness (mild foot drop). CONCLUSIONS: The seizure and functional outcome results of this highly preselected group of patients testifies to the feasibility and demonstrates the value of the combined benefits of both intra- and extraoperative SEEG recordings when resecting the rolandic and perirolandic areas. The novel hybrid method allows a more refined and precise identification of the epileptogenic zone. Consequently, tailored resections can be performed to minimize morbidity as well as to achieve adequate seizure control.

Is there a place for surgical treatment of nonpharmacoresistant epilepsy?

Epilepsy surgery has been shown to be the best possible treatment in well-defined and difficult-to-treat epilepsy syndromes, such as mesial temporal lobe epilepsy with unilateral hippocampal sclerosis, even early in the course of the disease if pharmacoresistance is proven. This review addresses the question if epilepsy surgery may be justified today even in nonpharmacoresistant cases. There are two possible groups of patients: first, there are epilepsy syndromes with a benign spontaneous course or with a potentially good treatment prognosis under appropriate antiepileptic drug (AED) treatment. Second, there are epilepsies with potentially worse AED treatment prognosis in which appropriate AED treatment has not yet been applied because of the short course of the disease, tolerability problems that prevented usually effective dosing, or adherence issues. In group one, the good spontaneous prognosis or the usually satisfying course under AED treatment in line with the commonly generalized underlying epileptogenesis does not suggest that epilepsy surgery is a realistic alternative, not even in cases with distinct focal clinical and/or electroencephalography (EEG) patterns like in Rolandic epilepsy with centrotemporal spikes. In the second group, the recent International League Against Epilepsy (ILAE) definition should allow assessment of individual pharmacoresistance early after the onset of the disease in order to avoid any delay. Concerns about a potential disease-specific or drug-specific cognitive decline that could be avoided in early surgery are speculative, a matter of controversial discussion, and certainly not relevant, if pharmacoresistance is consequently addressed in time according to the ILAE recommendations. One should also not forget that even in typically pharmacoresistant epilepsy syndromes that are suitable for surgical procedures, satisfying courses do exist that would not require early or any epilepsy surgery. Therefore, in almost any instance, epilepsy surgery as initial treatment or immediately after a first AED is still not recommended although, especially in cases with nonadherence to AEDs, it may be occasionally considered in order to outweigh the risks of ongoing seizures and epilepsy if surgery is not performed.

Epilepsy surgery of the rolandic and immediate perirolandic cortex: surgical outcome and prognostic factors.

OBJECTIVE: Herein we present a single-center retrospective study of patients who underwent epilepsy surgery for seizures arising from the sensorimotor (rolandic) cortex. The goal was to find prognostic factors associated with better seizure outcome and to evaluate both surgical and neurologic outcomes. PATIENTS, METHODS, AND MATERIALS: A total of 66 patients fulfilled eligibility criteria and were included in the study. Patients were divided into two groups for analysis: patients with resections within rolandic cortex (RO group; n = 46), and patients with resections in immediate perirolandic cortex and simultaneous sensorimotor multiple subpial transections (IPR group; n = 20). RESULTS: Favorable postoperative seizure outcome (International League Against Epilepsy [ILAE]; ILAE1-ILAE3) was achieved in 42 patients (64%), 39 (59%) of whom were completely seizure-free (ILAE1). The favorable seizure outcome in the RO group (72%) was better than in the IPR group (45%) (p = 0.04, relative risk [RR] 0.51 [0.28-0.94, 95% CI]). Eighteen patients (34%) had a postoperative permanent neurologic deficit. Independent predictors for excellent seizure outcome (ILAE1) after multivariate regression analysis were complete resection of the lesion (p < 0.001), pathology (p = 0.009), age at surgery (p = 0.03), and the absence of preoperative simple partial seizures (p = 0.01). SIGNIFICANCE: With a 64% favorable seizure outcome, surgery for intractable epilepsy arising from sensorimotor cortex is possible and can be worthwhile. The increased risk for postoperative neurologic deficits is higher than in other locations, and this must be discussed with patients in detail prior to surgery. Best postoperative results can be achieved in cases in which a complete resection is possible without damaging eloquent cortical areas.

The threshold of cortical electrical stimulation for mapping sensory and motor functional areas.

This study aimed to investigate the threshold of cortical electrical stimulation (CES) for functional brain mapping during surgery for the treatment of rolandic epilepsy. A total of 21 patients with rolandic epilepsy who underwent surgical treatment at the Beijing Institute of Functional Neurosurgery between October 2006 and March 2008 were included in this study. Their clinical data were retrospectively collected and analyzed. The thresholds of CES for motor response, sensory response, and after discharge production along with other threshold-related factors were investigated. The thresholds (mean ± standard deviation) for motor response, sensory response, and after discharge production were 3.48 ± 0.87, 3.86 ± 1.31, and 4.84 ± 1.38 mA, respectively. The threshold for after discharge production was significantly higher than those of both the motor and sensory response (both p<0.05). A negative linear correlation was found between the threshold of after discharge production and disease duration. Using the CES parameters at a stimulation frequency of 50 Hz and a pulse width of 0.2 ms, the threshold of sensory and motor responses were similar, and the threshold of after discharge production was higher than that of sensory and motor response.

Focal cortical dysplasia type IIb in the rolandic cortex: functional reorganization after early surgery documented by passive task functional MRI.

Surgery for seizures arising from the rolandic area can be performed effectively, and accurate mapping of eloquent regions may improve seizure and functional outcome. Noninvasive cortical mapping is, however, hardly feasible in young children. We studied two children with epileptogenic focal cortical dysplasia (FCD) type IIb in the rolandic area, in whom preoperative passive task functional MRI (fMRI) during sedation helped planning a tailored surgical approach. In one patient the dysplastic cortex was functionally activated. After complete lesionectomy both children exhibited motor impairment that readily improved. Repeat fMRI, performed after complete (Patient 1) or partial (Patient 2) recovery, demonstrated relocation of motor-related activations posterior to the area of resection. fMRI during sedation can be used to demonstrate postsurgical functional reorganization of the motor cortex in young children. There is interindividual variability in functional activation of FCD type IIb.

Preoperative functional mapping for rolandic brain tumor surgery: comparison of navigated transcranial magnetic stimulation to direct cortical stimulation.

BACKGROUND: Transcranial magnetic stimulation (TMS) is the only noninvasive method for presurgical stimulation mapping of cortical function. Recent technical advancements have significantly increased the focality and usability of the method. OBJECTIVE: To compare the accuracy of a 3-dimensional magnetic resonance imaging-navigated TMS system (nTMS) with the gold standard of direct cortical stimulation (DCS). METHODS: The primary motor areas of 20 patients with rolandic tumors were mapped preoperatively with nTMS at 110% of the individual resting motor threshold. Intraoperative DCS was available from 17 patients. The stimulus locations eliciting the largest electromyographic response in the target muscles ("hotspots") were determined for both methods. RESULTS: The nTMS and DCS hotspots were located on the same gyrus in all cases. The mean ± SEM distance between the nTMS and DCS hotspots was 7.83 ± 1.18 mm for the abductor pollicis brevis (APB) muscle (n = 15) and 7.07 ± 0.88 mm for the tibialis anterior muscle (n = 8). When a low number of DCS stimulations was performed, the distance between the nTMS and DCS hotspots increased substantially (r = -0.86 for APB). After the exclusion of the cases with < 15 DCS APB responses, the mean ± SEM distance between the hotspots was only 4.70 ± 1.09 mm for APB (n = 8). CONCLUSION: Peritumoral mapping of the motor cortex by nTMS agreed well with the gold standard of DCS. Thus, nTMS is a reliable tool for preoperative mapping of motor function.

Tailored resections for intractable rolandic cortex epilepsy in children: a single-center experience with 48 consecutive cases.

OBJECTIVE: A single-center experience with pediatric patients who underwent surgery for intractable rolandic epilepsy was reviewed with the aim of identifying putative factors that could influence postoperative seizure outcome in this population. METHODS: Clinical data of 48 patients under 18 years of age with diagnosis of intractable rolandic epilepsy who underwent surgery from January 1996 to September 2009 were reviewed. RESULTS: Patients' mean age at surgery was 9.9 ± 5.3 years; mean age at epilepsy onset was 3.9 years; mean seizure duration prior to surgery was 6 years; and mean follow-up was 5.1 years. The most frequent etiologies were cortical dysplasia, astrogliosis, tumors, tuberous sclerosis complex, and Sturge-Weber syndrome, which were observed in 20/48 (41.6%), 10/48 (20.8%), 10/48 (20.8%), 5/48 (10.4%), and 3/48 (6.2%) of the patients, respectively. After surgery, 20 patients (41.6%) showed neurological deficits, which in turn recovered within no longer than 6 months after surgery. Seizure outcome was classified as Engel class I in 29 (60.4%), Engel class II in 10 (20.8%), and Engel class III in 9 (18.8%) of the patients. The factors significantly related with seizure outcome were histological features (tumor versus non-tumor cases, p = 0.04) and lesion site (focal lesions versus non-focal lesions, p = 0.04). CONCLUSIONS: Tailored resection of rolandic cortex for intractable epilepsy can be safely performed in children. Accurate mapping of both functional cortex and epileptogenic areas may lead to improved seizure outcome. Tumor as well as focal lesions in hand and face motor areas are associated with good seizure outcome.

The combination of subdural and depth electrodes for intracranial EEG investigation of suspected insular (perisylvian) epilepsy.

PURPOSE: We present two methods of implantation for the investigation of suspected insular and perisylvian epilepsy that combine depth and subdural electrodes to capitalize on the advantages of each technique. METHODS: Retrospective study of all intracranial EEG studies that included insular electrodes from 2004-2010. Patients were divided according to the implantation scheme. The first method (type 1) consisted of a craniotomy, insertion of insular electrodes after microdissection of the sylvian fissure, orthogonal implantation of mesiotemporal structures with neuronavigation, and coverage of the adjacent lobes with subdural electrodes. The second method (type 2) consisted of magnetic resonance imaging (MRI)-stereotactic frame-guided depth electrode implantation into insula and hippocampus using sagittal axes, and insertion of subdural electrodes through burr holes to cover the adjacent lobes. The combined implantations were developed and performed by one neurosurgeon (AB). KEY FINDINGS: Nineteen patients had an intracranial study that sampled the insula, among other regions. Sixteen patients were implanted using the first method, which allowed a mean of 4, 5, 20, 15, and 42 contacts per patient to be positioned into/over the insular, mesial temporal, neocortical temporal, parietal, and frontal areas, respectively. The second method (three patients) allowed a mean of 8, 7, 16, 6, and 9 contacts per patient to sample the same areas, respectively. The four patients in whom transient neurologic deficits occurred were investigated with use of type 1 implantation. SIGNIFICANCE: Combined depth and subdural electrodes can be used safely to investigate complex insular/perisylvian refractory epilepsy. Choice of implantation scheme should be individualized according to presurgical data and the need for functional localization.

Epilepsy surgery outcome in coexisting symptomatic refractory focal epilepsy and benign focal epilepsy of childhood.

Epilepsy surgery may successfully treat refractory symptomatic focal epilepsy in patients with coexisting benign focal epileptiform discharges. Reported here is the outcome after resective epilepsy surgery in three children with pharmacoresistant lesional focal epilepsy in whom seizures of benign focal epilepsy of childhood had been recorded. Two patients had left temporal epilepsy due to a malformation of cortical development; one of these had dual pathology, with additional ipsilateral hippocampal sclerosis. One child had catastrophic left hemispheric epilepsy due to left hemimegalencephaly. Frequent, habitual seizures of symptomatic epilepsy resolved after surgery (follow-up duration, 32-55 months); however, rare benign focal seizures of childhood have continued. These cases demonstrate that lesional pharmacoresistant focal epilepsy can be successfully treated with resective epilepsy surgery even when coexisting with benign focal epilepsy of childhood. During postoperative follow-up, careful documentation of breakthrough seizures due to benign focal epilepsy of childhood is important, so that these patients are not labeled as surgical failures.

Surgical outcome following resection of rolandic focal cortical dysplasia.

OBJECTIVE: This study evaluates surgical outcomes in patients with balloon cell containing, focal cortical dysplasia (FCD type IIB) in the peri-rolandic region. We also address the question of, postoperative seizure worsening after an incomplete resection in this patient population. METHODS: We retrospectively reviewed the clinical data on patients with pathologically proven FCD, who underwent epilepsy surgery over a 12-year period. Seizure outcomes were compared between, patients with rolandic FCD and non-rolandic frontal FCD. RESULTS: Seventeen patients with rolandic and 22 patients with non-rolandic FCD were identified. Twelve patients were found to have type IIB lesions (8 rolandic). Seizure freedom at last follow up was, achieved in 59% of the rolandic subset of patients (including 75% seizure freedom in the rolandic type, IIB cases, as opposed to 44% of rolandic non-type IIB and 75% of type IIB outside the rolandic, region). New postoperative neurological deficits occurred in 59% of the rolandic subgroup and were, independent of pathology. Three patients (3/8) with incompletely resected rolandic type IIB lesions, developed status epilepticus postoperatively; all 3 patients became seizure free following a second, more extensive surgical resection. CONCLUSIONS: Patients with rolandic FCD can achieve a good surgical outcome after a complete, resection. However, an incomplete surgical resection in this patient population may lead to acute, postoperative seizure worsening.

Neurosurgical management of intractable rolandic epilepsy in children: role of resection in eloquent cortex. Clinical article.

OBJECT: The authors undertook this study to review their experience with cortical resections in the rolandic region in children with intractable epilepsy. METHODS: The authors retrospectively reviewed the medical records obtained in 22 children with intractable epilepsy arising from the rolandic region. All patients underwent preoperative electroencephalography (EEG), MR imaging, prolonged video-EEG recordings, functional MR imaging, magnetoencephalography, and in some instances PET/SPECT studies. In 21 patients invasive subdural grid and depth electrode monitoring was performed. Resection of the epileptogenic zones in the rolandic region was undertaken in all cases. Seizure outcome was graded according to the Engel classification. Functional outcome was determined using validated outcome scores. RESULTS: There were 10 girls and 12 boys, whose mean age at seizure onset was 3.2 years. The mean age at surgery was 10 years. Seizure duration prior to surgery was a mean of 7.4 years. Nine patients had preoperative hemiparesis. Neuropsychological testing revealed impairment in some domains in 19 patients in whom evaluation was possible. Magnetic resonance imaging abnormalities were identified in 19 patients. Magnetoencephalography was performed in all patients and showed perirolandic spike clusters on the affected side in 20 patients. The mean duration of invasive monitoring was 4.2 days. The mean number of seizures during the period of invasive monitoring was 17. All patients underwent resection that involved primary motor and/or sensory cortex. The most common pathological entity encountered was cortical dysplasia, in 13 children. Immediately postoperatively, 20 patients had differing degrees of hemiparesis, from mild to severe. The hemiparesis improved in all affected patients by 3-6 months postoperatively. With a mean follow-up of 4.1 years (minimum 2 years), seizure outcome in 14 children (64%) was Engel Class I and seizure outcome in 4 (18%) was Engel Class II. In this series, seizure outcome following perirolandic resection was intimately related to the child's age at the time of surgery. By univariate logistic regression analysis, age at surgery was a statistically significant factor predicting seizure outcome (p < 0.024). CONCLUSIONS: Resection of rolandic cortex for intractable epilepsy is possible with expected morbidity. Accurate mapping of regions of functional cortex and epileptogenic zones may lead to improved seizure outcome in children with intractable rolandic epilepsy. It is important to counsel patients and families preoperatively to prepare them for possible worsened functional outcome involving motor, sensory and/or language pathways.

Sequential analysis of fMRI images: A new approach to study human epileptic networks.

PURPOSE: The aim of this study was to introduce a new approach for analysis of functional magnetic resonance imaging (fMRI) data in order to illustrate the temporal development of the blood oxygenation level-dependent (BOLD) signal changes induced by epileptic seizures. METHOD: In order to sequentially analyze the fMRI images acquired during epileptic seizures, a continuous series of echo planar imaging (EPI) scans covering the complete period of a seizure was acquired. Data were segmented into 10-s blocks. Each block, representing a unique experimental condition, was contrasted with a neutral (no seizure) baseline condition. Visual comparison of the activations from one block to the next highlighted the course of activations and deactivations during the seizure event. This analysis was applied to three independent seizures of one patient with peri-rolandic epilepsy secondary to chronic encephalitis: one seizure before epilepsy surgery and two after unsuccessful tailored resection. Observations were compared to results from invasive subdural electroencephalography (EEG) monitoring, single-photon emission computed tomography (SPECT) coregistered to MRI (SISCOM), and independent component analysis (ICA), a model-free method of BOLD-signal analysis. RESULTS: The initial increase in BOLD signal occurred 10-40 s before clinical onset in the same location compared to the seizure-onset zone determined by invasive subdural evaluation and SISCOM. Sequential involvement of cortical and subcortical structures was in agreement with SISCOM, intracranial EEG recordings, and ICA results. DISCUSSION: In selected patients, sequential analysis of changes in BOLD signal induced by epileptic seizures might represent a useful approach for investigating the temporal development of brain activity during epileptic seizures, thereby allowing imaging of those cerebral structures involved in seizure generation and propagation.

Epilepsy surgery in children with seizures arising from the rolandic cortex.

PURPOSE: Medically intractable seizures arising from the sensorimotor (rolandic) cortex present a formidable challenge for epileptologists and epilepsy surgeons. The objective of this study was to evaluate the safety and efficacy of surgical treatment for seizures in this region. METHODS: We retrospectively reviewed the medical records of 24 children and adolescents who underwent surgical management of refractory rolandic epilepsy at our institution. RESULTS: Patient age at the time of surgery ranged between 1.9 and 19.2 years, and median postoperative follow-up was 2.3 years. Three patients had clear lesions on preoperative magnetic resonance imaging (MRI) that correlated with noninvasive electroencephalography (EEG) and underwent single-stage lesionectomies. Of the 21 patients who underwent invasive monitoring, 19 underwent therapeutic procedures, including lesionectomy, topectomy, and multiple subpial transections. Engel class I and II outcomes were seen in five of six patients (84.0%) after lesionectomy and in seven of nine patients (77.0%) and four of six patients (66.7%) after sensorimotor corticectomy or multiple subpial transections (MSTs), respectively. At last follow-up, neurologic deficits were observed following 50.0% (n = 3 of 6) of lesionectomies and 67.0% (n = 7 of 9) of topectomies; no neurologic deficits were seen in patients undergoing motor cortex MSTs. No correlation existed between seizure outcome and duration of epilepsy, age at diagnosis, age at surgery, histopathology, or the presence or absence of a discrete lesion. DISCUSSION: Surgery for appropriately selected children with intractable seizures arising from rolandic cortex was effective and associated with minimal neurologic morbidity in many cases. Engel I-II outcome was achieved in 77% of patients overall, and neurologic deficits were often mild and improved over time. Lesionectomy resulted in the best seizure control, followed by topectomy, and then MSTs.

[Diagnosis of sylvian and perisylvian epilepsies. Excitation symptomes of the insula reilii]. / Diagnostik sylvischer und perisylvischer Epilepsien. Exzitationssymptome der versunkenen Insel.

The insula is a hidden part in the cerebral cortex about which relatively little neurological research has been done. The present manuscript describes architectural and evolutionary aspects of the insula reilii as well as its function, towards a better understanding of seizure semiology. As the literature of such casuistry is poor, some own cases are presented. Seizure semiology, imaging, magnetoencephalographic reports, resective epilepsy surgery, radiosurgical treatments, and thermolesions are described. Magnetic source imaging as noninvasive treatment can deliver important information for the involvement of sylvian and perisylvian regions in focal pharmacoresistant epilepsies.

Clinical manifestations of insular lobe seizures: a stereo-electroencephalographic study.

PURPOSE: In this study, we report the clinical features of insular lobe seizures based on data from video and stereo-electroencephalographic (SEEG) ictal recordings and direct electric insular stimulation of the insular cortex performed in patients referred for presurgical evaluation of temporal lobe epilepsy (TLE). METHODS: Since our first recordings of insular seizures, the insular cortex has been included as one of the targets of stereo-electroencephalographic (SEEG) electrode implantation in 50 consecutive patients with TLE whose seizures were suspected to originate from, or rapidly to propagate to, the perisylvian cortex. In six, a stereotyped sequence of ictal symptoms associated with intrainsular discharges could be identified. RESULTS: This ictal sequence occurred in full consciousness, beginning with a sensation of laryngeal constriction and paresthesiae, often unpleasant, affecting large cutaneous territories, most often at the onset of a complex partial seizure (five of the six patients). It was eventually followed by dysarthric speech and focal motor convulsive symptoms. The insular origin of these symptoms was supported by the data from functional cortical mapping of the insula by using direct cortical stimulations. CONCLUSIONS: This sequence of ictal symptoms looks reliable enough to characterize insular lobe epileptic seizures (ILESs). Observation of this clinical sequence at the onset of seizures on video-EEG recordings in TLE patients strongly suggests that the seizure-onset zone is located not in the temporal but in the insular lobe; recording directly from the insular cortex should occur before making any decision regarding epilepsy surgery.

Functional variability of the human cortical motor map: electrical stimulation findings in perirolandic epilepsy surgery.

The purpose of this study was to assess the cortical representation of sensorimotor functions in patients undergoing perirolandic epilepsy surgery, focusing on somatotopy, mosaicism, and variability of function in relation to the classic motor homunculus. The authors studied 36 patients in whom intraoperative or extraoperative electrical cortical stimulation to map motor functions was performed. A computer program was devised to register electrode number, stimulation parameters, and response to each stimulus. Electrode position was represented graphically whenever a stimulus was delivered. A total of 43 maps from 36 patients were analyzed. The authors found variations in the organization of M1 (primary motor cortex) in seven patients (19.4%). Four patients (11.1%) presented mosaicism (overlapping of functional areas), two (5.6%) presented variability (inverted disposition of M1 functional areas), and one (2.8%) had both. The results of this study challenge the notion of orderly topographic relationships between the human sensorimotor functions and their representation in the primary motor cortex. These results confirm those of other studies with animals and humans using novel imaging techniques, suggesting that the motor homunculus may not always be considered a definite and absolute representation of M1.

Future aspects of epilepsy research.

This contribution in honour of Prof. Gerhard Pendl first reviews some recent studies on resected tissue, migrational disorders, and Rasmussen's Syndrome. These areas of basic research profit from recent advances of molecular biology and genetics. On the clinical side, some studies dealing with proton magnetic resonance spectroscopy are reviewed. In order to highlight the progress in clinical epilepsy research using modern methods of structural and functional imaging, functional outcome prediction is also reviewed. This kind of advanced clinical research is dealt with by discussing risk factor assessment associated with postsurgical decrements in memory. With regard to motor functions, we compare the yield of functional MR and intraoperative cortical stimulation in patients with lesions in or close to the Rolandic cortex. Progress in the field of advanced EEG analysis is reviewed in the context of "seizure prediction" and cognitive event-related potentials. Finally some of the new epilepsy treatment options, such as Gamma Knife treatment, where Prof. Pendl's group made pioneering contributions, are dealt with.

Seizure's outcome after cortical resections including the face and tongue rolandic areas in patients with refractory epilepsy and normal MRI submitted to subdural grids' implantation.

PURPOSE: To study the seizure's outcome in patients with refractory epilepsy and normal MRI submitted to resections including the rolandic cortex. METHODS: Four adult patients were studied. All patients had motor or somatosensory simple partial seizures and normal MRI and were submitted to subdural grids' implantation with extensive coverage of the cortical convexity (1 in the non-dominant and 3 in the dominant hemisphere). RESULTS: ECoG was able to define focal areas of seizures' onset in every patient. All patients were submitted to resection of the face and tongue motor and sensitive cortex; two patients had resections including the perirolandic cortex and 2 had additional cortical removals. Three patients are seizures' free and one had a greater then 90% reduction in seizure frequency. CONCLUSION: Resections including the face and tongue rolandic cortex can be safely performed even within the dominant hemisphere.

Malignant rolandic-sylvian epilepsy in children: diagnosis, treatment, and outcomes.

OBJECTIVE: To describe the diagnosis, treatment, and outcomes in children with malignant rolandic-sylvian epilepsy (MRSE), defined as a form of epilepsy characterized by sensorimotor seizures, medical refractoriness, normal MRI, frontocentrotemporal EEG spikes, rolandic-sylvian spike sources on magnetoencephalography (MEG), and cognitive problems. METHODS: A retrospective chart analysis of seven patients who had shown these characteristics and undergone extensive diagnostic testing, including MEG and intracranial video-EEG was performed. RESULTS: Interictal scalp EEG spikes were seen over the frontocentrotemporal regions bilaterally (6) and unilaterally (1). MEG showed spike sources in the perisylvian region in two patients (both bilateral) and in the perirolandic fissure in five (two bilateral). Three patients required bilateral subdural strips to lateralize seizures before electrocorticography. Final electrocorticograms showed an ictal onset zone around the rolandic (four cases) and rolandic-sylvian regions (three cases). Six patients showed neuropsychological deficits. After cortical excision and multiple subpial transection, three were seizure free and four had seizures rarely (30 months' mean follow-up). No child had a permanent deficit in sensorimotor or cognitive functions, although two showed exacerbation of preexisting attentional deficits. Tissue analysis showed definite evidence of neuronal migration disorders (3) and gliosis (2). CONCLUSIONS: MEG was helpful for localizing both malignant rolandic-sylvian neuronal activities and functional cortex. Successive neuropsychological assessments are necessary to detect cognitive deterioration and indicate remedial programming. If, after careful observation over at least 5 years, surgery is considered to control refractory seizures, intracranial video-EEG is needed to localize the epileptogenic zone.

Treatment in typical and atypical rolandic epilepsy.

Benign rolandic epilepsy is the most common epilepsy of childhood. Regarding seizure control typical and atypical rolandic epilepsies have a reasonable prognosis. In a recent prospective study STM was shown to control seizures in BECTS and STM is regarded as first line drug for this epilepsy in countries having access to this drug. Otherwise, CBZ is used most often and very effectively in BECTS. However, CBZ has the disadvantage to worsen clinical and EEG features up to the precipitation of CSWS in few patients. Epileptic seizures are often not the main problem in atypical rolandic epilepsies like CSWS or LKS and the amelioration of cognitive dysfunction by epileptic discharge is the prominent aim of an AED therapy. Steroids seem the have the best efficacy in these cases.