A retrospective study comparing preoperative evaluations and postoperative outcomes in paediatric and adult patients undergoing surgical resection for refractory epilepsy

PurposeTo review and compare the preoperative characteristics and postsurgical outcomes in paediatric and adult patients who underwent surgical resections from 2001 to 2009.MethodsCombined data from noninvasive measures such as ictal semiology, interictal/ictal scalp EEGs, MRI and SPECT were utilised to identify the epileptogenic zones (EZ). When noninvasive investigations produced inconclusive or inconsistent findings, patients underwent intracranial EEG monitoring. Resective micro-surgical procedures were conducted according to the results of the anatomo-electro-clinical investigations and were carried out to remove the EZ. We then followed up 222 paediatric (≤18 years old) and 100 adult patients (≥19 years old) for 1–9 years postoperatively.ResultsThe mean age of seizure onset in paediatric group was significantly lower than that in adult group. 95 (43%) of the paediatric and 42 (42%) of the adult patients required long-term intracranial EEG recording. 54 (24.3%) of the paediatric and 62 (62%) of the adult patients were found to have temporal lobe epilepsy (TLE), while 149 (67.1%) of the paediatric and 37 (37.0%) of the adult patients had extra-temporal lobe epilepsy (ETLE) (p = 0.000). 19 (8.6%) of the paediatric patients and 1 (1%) adult patient had hemispheric lesions (p = 0.009). 148 (66.7%) of the paediatric and 61 (61.0%) of the adult patients were seizure-free during the follow-up period. 17 of 19 (89.5%) children who underwent hemispherectomy were seizure-free. In both paediatric and adult groups, the surgical outcome for patients with TLE was significantly better than that of patients with ETLE (p = 0.018 in children, p = 0.029 in adults). Both the location of EZs and seizure-free ratio were significantly different (p < 0.001) between the preadolescent (≤12 years old) and adolescent (13–18 years old) group. Hippocampal sclerosis was the most common pathologic finding in patients with TLE in both groups, and was followed by focal cortical dysplasia. In patients with TLE, the proportion of tumour was significantly higher in the paediatric than the adult group (25.9% vs. 10%, p = 0.021).ConclusionPaediatric patients with refractory seizures had more extratemporal or hemispheric resectable epileptogenic foci and fewer temporal foci than adults. Our study demonstrates that resective surgery is an effective and safe early intervention in strictly selected paediatric patients with refractory epilepsy.     

Is ‘burned-out hippocampus’ syndrome a distinct electro‐clinical variant of MTLE-HS syndrome?

AimTo study the clinical, electrophysiological and imaging characteristics of patients with unilateral mesial temporal lobe epilepsy (MTLE) with contralateral ictal onset on scalp EEG, viz. ‘burned-out hippocampus’ syndrome (MTLE-BHS).MethodsMTLE-BHS was defined as TLE with unilateral hippocampal sclerosis (HS) without any dual pathology on MRI and contralateral ictal onset on scalp EEG, unlike in classical hippocampal sclerosis (HS). Consecutive “MTLE-BHS” patients evaluated at our Centre for Comprehensive Epilepsy Care from January 2005 to July 2014 were studied. Twenty-five cases of classic MTLE-HS operated during the same period were also analyzed for comparison.ResultsSeventeen patients were diagnosed to have MTLE-BHS. Mean age of seizure onset was 9.5 ± 7.7 years and the mean duration of epilepsy was18.2 ± 7.3 years. Epigastric aura was more common in MTLE-HS and fear, secondary generalized seizures and temporal polar changes on MRI were more prevalent in the MTLE-BHS subgroup. In the latter group, five (29%) exhibited seizure semiology and 2 (12%) had interictal discharges discordant to the side of MTS. Eight (47%) patients in the MTLE-BHS sub-group had normal medial temporal volume on Scheltens scale. Eight patients among MTLE-BHS underwent surgery (4 following intracranial monitoring that localized to the side of HS) with Engel class I outcome at 1 year follow-up in 6 and Engel class II outcome in 2.ConclusionAttenuation of ipsilateral fast ictal rhythms on scalp EEG as well as neocortical changes are likely to be deterministic factors for MTLE-BHS as opposed to the severity of hippocampal atrophy. Considering good post-operative outcomes, intracranial monitoring for surgical selection is not mandatory in MTLE-BHS despite discordant semiology and ictal onset, in the presence of inter-ictal, functional imaging and neuropsychology data concordant to the side of HS.     

Orbitofrontal epilepsy: Case series and review of literature

BackgroundOrbitofrontal epilepsy (OFE) is less known and is poorly characterized in comparison with temporal lobe epilepsy, partly because it is rare and possibly because it is unrecognized and therefore underestimated.ObjectiveThis paper aimed to better characterize seizure semiology, presurgical findings, and surgical outcomes in patients with OFE.MethodsWe retrospectively reviewed all confidently established OFE cases from six Canadian epilepsy monitoring units between 1988 and 2014, and in the literature between 1972 and 2017. Inclusion criteria were identification of an epileptogenic lesion localized in the OFC or if the patient was seizure-free after surgical removal of the OFC in nonlesional cases.ResultsSixteen cases were identified from our databases. Fifty percent had predominantly sleep-related seizures; 56% had no aura (the remaining had nonspecific or vegetative auras), and 62.5% featured hypermotor (mostly hyperkinetic) behaviors. Interictal epileptiform discharges over frontal and temporal derivations always allowed lateralization. Magnetic resonance imaging (MRI) identified an orbitofrontal lesion in 8/16, positron emission tomography (PET) identified a hypometabolism extending outside the orbital cortex in 4/9, ictal single-photon emission computed tomography (SPECT) identified an orbital hyperperfusion in 1/5, magnetoencephalography (MEG) identified lateral orbital sources in 2/4, and intracranial electroencephalography (EEG) identified an orbitofrontal onset in 9/10. Fourteen patients underwent surgery, all reaching a favorable outcome (71.4% Engel 1; 28.6% Engel 2; mean FU = 5.6 years). Pre- and postoperative neuropsychological assessments revealed heterogeneous findings. Our review of literature identified 71 possible cases of OFE, 32 with confident focus localization. Extracted data from these cumulated cases supported observations made from our case series.ConclusionsOrbitofrontal epilepsy should be suspected with sleep-related, hyperkinetic seizures with no specific aura, and frontotemporal interictal discharges. Several patients have nonmotor seizures with or without auras which may resemble temporal lobe seizures. Postoperative seizure outcome was favorable, but there is inherent bias as we only included patients with a seizure-free outcome if the MRI was negative. A larger study is required to address identified gaps in knowledge such as identifying discriminative features between medial and lateral OFE, evaluating the value of more recent diagnostic tools, and assessing the neuropsychological outcome of orbital epilepsy surgery.     

Outcomes of resective surgery in children and adolescents with focal lesional epilepsy: The experience of a tertiary epilepsy center

ObjectiveThis study aimed to investigate the efficacy of resective surgery in children with focal lesional epilepsy by evaluating the predictive value of pre- and postsurgical factors in terms of seizure freedom.MethodsThis study included 61 children aged between 2 and 18 years who were admitted to the pediatric video-EEG unit for presurgical workup. Each patient was evaluated with a detailed history, video-EEG, neuroimaging, and postsurgical outcomes according to Engel classification to predict postsurgical seizure freedom. All the possible factors including history, etiology, presurgical evaluation, surgical procedures, and postsurgical results were analyzed for their predictive value for postoperative seizure freedom.ResultsOf the 61 patients, 75% were diagnosed as having temporal lobe epilepsy (TLE), and 25% were diagnosed with extra-TLE. Two years after the surgery, 78.6% were seizure-free, of which 89% had TLE, and 50% had extra-TLE (p < 0.05). Patients were more likely to have a favorable outcome for seizure freedom if they had rare seizure frequency, focal EEG findings, and focal seizures; had a temporal epileptogenic zone; or had TLE and hippocampal sclerosis. On the other hand, patients were more likely to have unfavorable results for seizure freedom if they had younger age of seizure onset, frequent seizures before the surgery, a frontal or multilobar epileptogenic zone, secondarily generalized seizures, extra-TLE with frontal lobe surgery, or focal cortical dysplasia.SignificanceResective surgery is one of the most effective treatment methods in children with intractable epilepsy. A history of young age of seizure onset, frequent seizures before surgery, secondarily generalized seizures, a multilobar epileptogenic zone, frontal lobe surgery, and focal cortical dysplasia (FCD) are the most important predictive factors indicating that a patient would continue having seizures after surgery. On the other hand, focal seizure semiologies, temporal lobe localization, and hippocampal sclerosis indicate that a patient would have better results in terms of seizure freedom.     

Insular and insulo-opercular epilepsy in childhood: An SEEG study

PurposeIn recent years, there have been series analysing the electro-clinical correlations of insular epilepsy in adult populations. In contrast, the ictal semiology in children with insular epilepsy is poorly described. Considering that early and successful surgery may greatly improve the cognitive outcome and quality of life, it is worthwhile to deepen our knowledge of insular epilepsy in children.MethodsWe retrospectively evaluated ten children with drug-resistant focal insular epilepsy who had been consecutively explored with stereoelectroencephalography (SEEG), followed by individually tailored resective surgery that included part of the insula in all cases. A detailed anatomo-electro-clinical analysis of non-invasive EEG and SEEG data was performed. At least one of the electrodes explored the insular cortex. SEEG analysis confirmed that the insular cortex was included in the ictal onset zone.ResultsEpilepsy onset was mostly during the first year of life, characterized by subtle seizures as well as spasms and myoclonic seizures. Later on, neurovegetative signs and asymmetric tonic and hypermotor seizures (HMS) dominated the ictal semiology. The epileptogenic zone was frequently wider than insular with frontal and central predominance. In eight patients, the tailored resection included a lesion. In seven patients, an Engel class 1 outcome as well as neuropsychological and behavioural improvement was obtained.ConclusionsSEEG is feasible and useful in children with drug-resistant insular epilepsy which is often characterized by autonomic symptoms as the initial symptoms and should be suspected in cases with HMS, asymmetric tonic seizures and even asymmetric spasms. Early propagation is mostly frontal and central. Analysis of a larger population is required to refine these findings.     

Differences between mesial and neocortical magnetic-resonance-imaging-negative temporal lobe epilepsy

ObjectiveThe aim of this study was to assess clinical and electrophysiological differences within a group of patients with magnetic-resonance-imaging-negative temporal lobe epilepsy (MRI-negative TLE) according to seizure onset zone (SOZ) localization in invasive EEG (IEEG).MethodsAccording to SOZ localization in IEEG, 20 patients with MRI-negative TLE were divided into either having mesial SOZ–mesial MRI-negative TLE or neocortical SOZ–neocortical MRI-negative TLE. We evaluated for differences between these groups in demographic data, localization of interictal epileptiform discharges (IEDs), and the ictal onset pattern in semiinvasive EEG and in ictal semiology.ResultsThirteen of the 20 patients (65%) had mesial MRI-negative TLE and 7 of the 20 patients (35%) had neocortical MRI-negative TLE. The differences between mesial MRI-negative TLE and neocortical MRI-negative TLE were identified in the distribution of IEDs and in the ictal onset pattern in semiinvasive EEG. The patients with neocortical MRI-negative TLE tended to have more IEDs localized outside the anterotemporal region (p = 0.031) and more seizures without clear lateralization of ictal activity (p = 0.044). No other differences regarding demographic data, seizure semiology, surgical outcome, or histopathological findings were found.ConclusionsAccording to the localization of the SOZ, MRI-negative TLE had two subgroups: mesial MRI-negative TLE and neocortical MRI-negative TLE. The groups could be partially distinguished by an analysis of their noninvasive data (distribution of IEDs and lateralization of ictal activity). This differentiation might have an impact on the surgical approach.     

Temporal lobe surgery in medically refractory epilepsy: A comparison between populations based on MRI findings

IntroductionHigh resolution MRI findings suggestive of mesial temporal sclerosis (MRI-MTS) correlate with good outcome after surgery. However, a large group of patients present with normal brain MRI (N-MRI) and temporal lobe epilepsy (TLE). We aim to compare pre-operative ictal EEG patterns in predicting surgical outcomes in the population with MRI-MTS vs. N-MRI after selective anterior-mesial temporal lobe (AMTL) resection.Methods241 patients with unilateral anterior ictal EEG findings underwent selective AMTL resection. 143 MRI-MTS and 98 N-MRI patients were identified. Outcome was based on the modified Engel classification, ictal EEG pattern at seizure onset, demographics and MRI findings.ResultsSeizure-free outcome was seen in the MRI-MTS in 79% of patients, compared to 59.1% (p < .005) of the N-MRI group. No significant difference was identified in ictal EEG patterns at presentation between groups. Class I outcome was achieved in 78.9% of patients that had theta rhythm and MRI-MTS compared to 57.9% of patients that had theta rhythm and N-MRI (p < 0.05).Discussion and conclusionSurgical treatment for mesial TLE is effective. Positive MRI suggestive of mesial temporal sclerosis (MTS) predicts better seizure control after surgery. Theta rhythm is the most common ictal pattern and seems to carry the best prognosis for TLE. However, a well-selected group of patients with N-MRI will benefit from surgical intervention, and similar outcome to MRI-MTS patients can be achieved if delta ictal EEG pattern is presented at initial onset. Early referral to an epilepsy center cannot be emphasized enough, even in situations when high-resolution brain MRI is normal.     

Surgical treatment of hypermotor seizures originating from the temporal lobe

PurposeTo describe the characteristics of electroclinical manifestations in patients with hypermotor seizures (HMSs) originating from the temporal lobe.MethodsWe retrospectively reviewed the data of patients who underwent surgical treatments for seizure to identify patients with HMSs of temporal origin. We systematically reviewed patient seizure histories, imaging reports, video-EEG monitoring data, operative records and pathological findings.ResultsEight of the 9 patients reported auras. The ictal behavior included marked agitation in 5 patients and mild agitation in 4 patients. All of the 9 patients exhibited stiffness or dystonia of the upper limb or contralateral limbs during ictus. Seven of the 9 patients completed intracranial recording and at least 3 seizures were recorded for each patient. The intracranial recordings showed ictal activity originating from mesial temporal lobe in 6 patients and the lateral temporal lobe in 1 patient. The time interval of ictal propagation from the temporal to frontal lobe was 15.0 ± 8.3 s. While the time interval from EEG origination to the beginning of hypermotor behavior was 21.0 ± 8.1 s. Brain MRIs revealed hippocampal sclerosis in 3, neoplastic lesion in 1, and normal images in the remaining 5 patients. Patients were followed for 1–5 years after the anterior temporal lobectomy; 7 patients remained seizure-free throughout follow-up.ConclusionSome HMSs can originate from the temporal lobe. In carefully selected patients, surgical resection may lead to good outcomes.     

Lesion focused stereotactic thermo-coagulation of focal cortical dysplasia IIB: A new approach to epilepsy surgery?

PurposeOver the last few decades, preferred epilepsy surgical approaches have developed from standard lobectomies to individually tailored resections. Yet, it remains uncertain how small surgical interventions can be without compromising the success of the surgery. Particularly in patients with epileptogenic lesions identified by magnetic resonance imaging (MRI), it is unresolved whether resection or destruction of the lesion suffices to eliminate the epileptogenic zone. We present a minimally invasive surgical approach that may provide insight into this issue.MethodsTwo patients with pharmacoresistant epilepsy due to focal cortical dysplasia type IIB demonstrated by 3 Tesla MRI were treated with lesion-focused stereotactic radio-frequency thermo-coagulation. In both patients, pathognomonic epileptic discharges were recorded from the lesion via a stimulation device prior to coagulation. In one patient, the suspected proximity of the lesion to the pyramidal tract was verified by eliciting motor evoked potentials from the depths of the lesion.ResultsFollowing complete and near complete lesion destruction, seizures (several per day or per week, respectively) ceased in patients A and B, with no seizures for 12 months and 5 months, respectively, at the time of this publication. Neither patient acquired persistent postoperative neurological deficits.ConclusionThe fact that seizure activity stopped after destruction of small bottom-of-sulcus dysplasias implies that in these cases, the epileptogenic zone and the epileptogenic lesion may overlap. If future studies can replicate this finding, focused lesion destruction could be a further development of individually tailored epilepsy surgery. The technique described here is especially suited for high-precision surgery close to eloquent brain structures.     

Invasive explorations in children younger than 3years

PurposeIn children with drug-resistant focal epilepsy who are candidates for surgery, invasive exploration is sometimes required. However, this is being controversially discussed for children younger than 3 years. The question of its necessity, feasibility and its risks is often raised, since it concerns primarily lesional epilepsy and a lesionectomy might be proposed right away. However, this attitude does not take into account the specificities of epilepsy at this age, including poor specificity of electroclinical semiology and the ongoing myelination challenging the interpretation of magnetic resonance imaging (MRI).MethodsWe retrospectively studied the records of children with drug-resistant epilepsy who were younger than 3 years of age at the time of their invasive exploration at our institution from 2000 to 2009. We reviewed the clinical, imaging and electrophysiological data, and included post-operative outcome for those who underwent surgery.Key findings26 Children met the inclusion criteria. All had drug-resistant epilepsy that started at an average of 5.2 months (range 0–20 months) with multiple daily seizures in all and developmental delay in 16. The average age at the time of exploration was 21.8 months (range 5–35). In 20 children, subdural electrodes in combination with two or three depth electrodes were implanted, and in six children aged over 2 years a stereo-electro-encephalography (SEEG) was performed. SEEG was considered technically difficult to achieve before the age of 2 years. The tolerance of invasive exploration was good with a 3% morbidity consisting of one subdural hematoma during exploration by subdural electrodes, evacuated without any particular sequelae. In 25 patients, the exploration permitted to propose a focal resection. The surgical intervention was in the frontal lobe in 12 cases, the parietal lobe in six, the occipital lobe in two patients, and the temporal lobe in one child who underwent an additional resection. Four children had a resection of two or three lobes. Five underwent a second surgery, following a second invasive exploration. Histologically, the resected tissue revealed focal cortical dysplasia in 21 cases (including three patients with tuberous sclerosis), two post-ischemic lesions, one dysembryoplastic neuroepithelial tumor, and one gangliglioma associated with dysplasia. The mean postoperative follow-up period was 51 months (range 4–110). For the children operated on twice, follow-up was counted from the second surgery on. Seventeen children (68%) had an outcome of Engel class 1. In five (20%), seizure frequency was significantly improved (Engel class 3). In two of three patients without improvement in seizure frequency (Engel class 4), a new SEEG is planned and the third is presently a candidate for hemispherotomy.SignificanceInvasive exploration is feasible, well tolerated and carries a low morbidity in children under 3 years of age. At this age, it is indicated for drug-resistant lesional epilepsy associated with developmental delay. It permits delineating the lesion, which is not possible with MRI. The choice of the technique is in part age-dependent. The discussion of its indication arises in the same way as in the older child.     

Do sphenoidal electrodes aid in surgical decision making in drug resistant temporal lobe epilepsy?

ObjectiveThe utility of sphenoidal electrodes (SPh) in analyzing interictal epileptiform discharges (IEDs) and ictal electrography remains controversial, despite its widespread use.MethodsOne hundred and twenty-two consecutive patients with presumed temporal lobe epilepsy (TLE) who underwent presurgical evaluation were prospectively studied. SPh and Silverman’s electrodes were placed, in addition to routine electrodes in 10–20 international system. IEDs and ictal electroencephalography (EEG) were analyzed separately in bipolar and referential montages. The proportion of patients selected for surgery after adjusting for SPh placement based on the earlier ictal onset and IEDs were analyzed.ResultsOf the 8701 IEDs in SPh, only 65% were seen over the scalp bipolar montage; 1392 (16%) IEDs were confined to SPh electrodes, and were not seen at scalp bipolar montage (p < 0.001). Spike amplitudes were highest at SPh (p < 0.001). Of the 592 seizures analyzed, 62 (61%) had simultaneous SPh and scalp onset, while in 26 (25%) SPh onset preceded the scalp.ConclusionsOut of the 35 patients with unilateral mesial temporal sclerosis (MTS) with additional neocortical changes and/or non-lateralized bitemporal IEDs and/or diffuse ictal onset (group 1), 27 were selected for surgery (77%). About 7% was selected for surgery in this group by SPh placement. Also, in patients with bilateral MTS (group 2), 25% (5/20) were chosen for anterior temporal lobectomy, SPh provided an additional benefit in 11% (p < 0.001). Patients with normal magnetic resonance imaging (group 3) and temporal plus epilepsy (group 4) had a lower surgical yield, only 12% and 9.5% could undergo surgery. They were denied surgical candidacy with SPh (p < 0.001).SignificanceOne-third of patients after SPh placement were selected for resective surgery obviating the need for invasive monitoring. The maximum yield was noted in unilateral MTS (associated with additional neocortical features or non-lateralized bilateral temporal interictal IEDs or diffuse ictal onset in scalp EEG) and in bilateral MTS. Those with normal MRI/temporal plus epilepsy could be excluded from direct resective surgery.     

Visual and semiautomated evaluation of epileptogenicity in focal cortical dysplasias — An intracranial EEG study

IntroductionThe aim of the study was the evaluation of the added value of depth to subdural electrodes in delineating epileptogenicity of focal cortical dysplasias (FCDs) and to test the Epileptogenicity Index (EI) in this setting.Material and methodsFifteen patients with FCD underwent iEEG with subdural and depth electrodes. Visual/EI analysis was performed in up to three habitual seizures per patient.ResultsVisual analysis: Grid onset seizures (n = 10) started in electrodes overlying the lesion in 7 and remote from it in 3 cases. Depth onset seizures (n = 7) affected only intralesional contacts in 4, intra- and extralesional in 2, and exclusively extralesional in 1 patient. Seizures started in depth and grid contacts simultaneously in 2 cases.EI analysis: The EI completely confirmed visual localization of seizure onset in 8 cases and depicted ictal onset-time accurately in 13. Beta/gamma ictal patterns were most reliably captured.Impact on surgical decision: Resection outline differed from MRI lesion in 7 patients based on grid and in three based on depth electrode information.DiscussionIn FCD, seizures can be generated within gyral/deep tissue appearing normal on imaging.ConclusionInvestigating FCD with subdural and depth electrodes is efficient to outline the seizure onset zone. The EI is a helpful additional tool to quantify epileptogenicity. Specific ictal patterns are prerequisite for reliable results.     

Semiology of hypermotor (hyperkinetic) seizures

BackgroundHypermotor seizures (HMSs) consist of complex movements involving proximal segments of the limbs and trunk that appear violent and inappropriate for the situation.MethodsWe analyzed hypermotor seizure videos in seizure-free patients (Engel class I) following resective epilepsy surgery. After completion of video analysis, we reviewed EEG and neuroimaging data.ResultsSearch of our epilepsy surgery database yielded 116 patients classified as having hypermotor seizures between 1996 and 2013. From this subset, 17/31 (55%) patients had been seizure-free for > 6 months (mean follow-up: 3.3 years). Mean seizure duration was 35 s (range: 6–91 s), of which the HM phase lasted a mean of 22 s (range: 3–53 s). In 16 patients (95%), hypermotor activity was seen at or within 10 s of clinical seizure onset.Type I semiology occurred in 6 patients, type II semiology in 10 patients, and 1 patient exhibited features of both. Type I and type II semiologies were noted in patients who had frontal lobe as well as extrafrontal resections. Nonversive head and body turning occurred in 10 patients (ranging from 90° to 270°) which was ipsilateral to the side of resection in all patients and seen both in frontal and extrafrontal resections. Six out of eleven patients with abnormal MRI and 4/6 patients with nonlesional MRI underwent invasive EEG evaluation. Eight patients (47%) had frontal lobe resection, 4/17 (23%) patients had temporal lobe resection, and one patient each had parietal lobe, insular, temporoparietooccipital, or motor sparing resection; 1 patient had functional hemispherectomy.ConclusionHypermotor semiology typically occurs at or within 10 s after seizure onset. Ipsilateral head/body turning appears to be of lateralizing value whereas asymmetry of limb movement was not lateralizing. Hypermotor semiology is most often seen in frontal lobe epilepsy but may occur in seizures arising from other locations.     

MEG-based identification of the epileptogenic zone in occult peri-insular epilepsy

IntroductionPresurgical work-ups of patients with pharmacoresistant epileptic seizures can require multiple diagnostic methods if magnetic resonance imaging (MRI) combined with video-EEG monitoring fails to show an epileptogenic lesion. Yet, the added value of available methods is not clear. In particular, only a minority of epilepsy centres apply magnetoencephalography (MEG). This study explores the potential of MEG for patients whose previous sophisticated work-ups missed deep-seated, peri-insular epileptogenic lesions.Patients and methodsThree patients with well documented, frequent, stereotypical hypermotor seizures without clear focus hypotheses after repeated presurgical work-ups including video-EEG-monitoring, 3 Tesla (3 T) magnetic resonance imaging (MRI), morphometric MRI analysis, PET and SPECT were referred to MEG source localisation.ResultsIn two out of three patients, MEG source localisation identified very subtle morphological abnormalities formerly missed in MRI or classified as questionable pathology. In the third patient, MEG was not reliable due to insufficient detection of epileptic patterns. Here, a 1 mm × 1 mm × 1 mm 3 T fluid-attenuated inversion recovery (FLAIR) MRI revealed a potential epileptogenic lesion. A minimal invasive work-up via lesion-focused depth electrodes confirmed the intralesional seizure onset in all patients, and histology revealed dysplastic lesions. Seizure outcomes were Engel 1a in two patients, and Engel 1d in the third.DiscussionMEG can contribute to the identification of epileptogenic lesions even when multiple previous methods failed, and when the lesions are located in deep anatomical structures such as peri-insular cortex. For epilepsy centres without MEG capability, referral of patients with cryptogenic focal epilepsies to centres with MEG systems may be indicated.     

Epilepsy surgery; indication and the latest treatment

In order to obtain the optimal results (being free from seizures and functional deficits) in epilepsy surgery from the viewpoint of restorative functional neurology, it is essential to define both epileptogenic and functional zones precisely. Several important concerns are listed as follows for neurologists who care and introduce surgical candidates with intractable epilepsy from their clinic to the epilepsy surgery centers; 1) intractability of seizures, 2) decreased verbal memory after surgery in patients with left mesial temporal lobe epilepsy, 3) surgical outcome of patients with neocortical epilepsy without MRI abnormalities, 4) possible dissociation between ictal onset zone and ictal semiology, and 5) interventional neurophysiology which enhances endogenous inhibitory networks in the brain in an externally triggered or internally driven way.     

Spindle frequency activity may provide lateralizing information in drug-resistant nocturnal mesial frontal lobe epilepsy: A pilot study on the contribution of sleep recordings

PurposeNocturnal frontal lobe epilepsy (NFLE) is characterized by sleep-related paroxysmal motor attacks occurring almost exclusively during non-REM sleep. Surgical treatment may relieve symptoms in drug-resistant patients. However, the identification of the epileptogenic zone, the region to be resected, is frequently challenging because of the absence of lateralizing and localizing information and the lack of informative EEG correlates. The aim of this study was to find asymmetries in the ictal activity that could provide information on the lateralization of the epileptogenic zone.MethodWe retrospectively analyzed the sleep EEG of four patients recorded prior to surgical intervention. The epileptogenic zone was known, as these patients had subsequently undergone successful surgery after bilateral intracerebral stereo-EEG investigation. Sleep EEG during the ictal phase was compared with sleep EEG during the pre-ictal phase.ResultsIn all patients, electrical sources of sigma activity (12–16 Hz) exhibited increased activity during the ictal phase which was higher in the epileptogenic hemisphere. Conversely, increased delta activity (1–4 Hz) was predominant contralateral to the epileptogenic focus in three of four patients.ConclusionSigma activity may have a predictive role in the lateralization of the epileptogenic zone and be useful during the pre-surgical evaluation of patients with NFLE.     

Intralesional recordings and epileptogenic zone in focal polymicrogyria

PURPOSE: Polymicrogyria (PMG) is recognized as an epileptogenic lesion but few data concerning organization of the epileptogenic zone (EZ) are available. METHODS: We analyzed the distribution of the EZ according to Stereo-EEG (SEEG) with intralesional recordings in four patients evaluated for intractable partial epilepsy associated with focal unilateral PMG, involving the posterior temporal region in two, the perisylvian area in one and the temporoparietal junction in the other. All had ictal scalp EEG, high-resolution structural and functional MRI, fluorodeoxyglucose positron emission tomography (FDG-PET), and SEEG. For each patient, several depth electrodes were implanted both within the PMG and in extralesional areas. RESULTS: In three patients, the PMG displayed high-frequency spiking activity. However, interictal and ictal recordings demonstrated a large epileptogenic network, which was more widespread than the PMG, including the mesial temporal structures in two. In another patient, interictal spiking and seizure onset site were located within the hippocampus and outside of the PMG, although it was rapidly involved during seizure spread. Overall, EZ was considered to be larger than the PMG in all patients although hypometabolic areas detected by PET were concordant with EZ. Three patients underwent extensive surgery including the PMG and are seizure free with a follow-up >2 years. DISCUSSION: Although intralesional recordings demonstrated intrinsic epileptogenicity in PMG, our data provide evidence that unilateral focal PMG belongs to a large epileptogenic network extending beyond the MRI lesion. SEEG may be helpful for planning surgery with favorable outcome, providing large resections are feasible, even in apparently focal PMG.     

“Eating” epilepsy revisited- an electro-clinico-radiological study

This study aimed to evaluate the clinical, video electroencephalographic and MRI attributes of patients with eating epilepsy (EE). Consecutive patients who were diagnosed with EE and underwent potential pre-surgical work-up from 2003 to 2012 formed the study cohort. Their electro-clinico-radiological and seizure outcome data were obtained from our prospectively maintained medical records. Out of 7094 patients who underwent evaluation for refractory seizures, 47 patients satisfied the criteria for EE. Twenty-three (48.9%) had exclusive EE; the remainder had a combination of predominantly eating-induced and unprovoked seizures with no differences noted in timing of seizures in relation to meals. Lesional epilepsy was seen in 34% of patients, with posterior cortex (PC; posterior temporo-parieto-occipital) predominance. In MRI negative patients, PC interictal epileptiform discharges were present in 34.4% of patients and multifocal in 20.6% of patients compared to the MRI positive group with 12.5% and 6.5%, respectively (p = 0.003). Among 24 patients (51.1%) with co-existent unprovoked seizures, developmental delay and PC ictal onset was more prevalent (p = 0.013 and 0.029) as compared to exclusive EE. The seizure frequency and outcome did not significantly differ between patients with or without MRI abnormality. Two patients underwent anterior temporal lobectomy, with persistence of their eating seizures postoperatively. EE is a complex reflex epilepsy of cryptogenic and symptomatic etiology. As opposed to the traditionally implied temporo-limbic mechanisms behind epileptogenesis, a multilobar network originating from the PC receiving sensory and visual inputs linked to the limbic-opercular pathways represents a plausible mechanism. Surgical selection should be diligent and cautious in this group of patients.     

Presurgical evaluation for partial epilepsy: relative contributions of chronic depth-electrode recordings versus FDG-PET and scalp-sphenoidal ictal EEG

One hundred fifty-three patients with medically refractory partial epilepsy underwent chronic stereotactic depth-electrode EEG (SEEG) evaluations after being studied by positron emission tomography (PET) with 18F-fluorodeoxyglucose (FDG) and scalp-sphenoidal EEG telemetry. We carried out retrospective standardized reviews of local cerebral metabolism and scalp-sphenoidal ictal onsets to determine when SEEG recordings revealed additional useful information. FDG-PET localization was misleading in only 3 patients with temporal lobe SEEG ictal onsets for whom extratemporal or contralateral hypometabolism could be attributed to obvious nonepileptic structural defects. Two patients with predominantly temporal hypometabolism may have had frontal epileptogenic regions, but ultimate localization remains uncertain. Scalp-sphenoidal ictal onsets were misleading in 5 patients. For 37 patients with congruent focal scalp-sphenoidal ictal onsets and temporal hypometabolic zones, SEEG recordings never demonstrated extratemporal or contralateral epileptogenic regions; however, 3 of these patients had nondiagnostic SEEG evaluations. The results of subsequent subdural grid recordings indicated that at least 1 of these patients may have been denied beneficial surgery as a result of an equivocal SEEG evaluation. Weighing risks and benefits, it is concluded that anterior temporal lobectomy is justified without chronic intracranial recording when specific criteria for focal scalp-sphenoidal ictal EEG onsets are met, localized hypometabolism predominantly involves the same temporal lobe, and no other conflicting information has been obtained from additional tests of focal functional deficit, structural imaging, or seizure semiology.     

Neural network underlying ictal pouting (“chapeau de gendarme”) in frontal lobe epilepsy

In order to determine the anatomical neural network underlying ictal pouting (IP), with the mouth turned down like a “chapeau de gendarme”, in frontal lobe epilepsy (FLE), we reviewed the video-EEG recordings of 36 patients with FLE who became seizure-free after surgery. We selected the cases presenting IP, defined as a symmetrical and sustained (> 5 s) lowering of labial commissures with contraction of chin, mimicking an expression of fear, disgust, or menace. Ictal pouting was identified in 11 patients (8 males; 16–48 years old). We analyzed the clinical semiology, imaging, and electrophysiological data associated with IP, including FDG-PET in 10 and SEEG in 9 cases. In 37 analyzed seizures (2–7/patient), IP was an early symptom, occurring during the first 10 s in 9 cases. The main associated features consisted of fear, anguish, vegetative disturbances, behavioral disorders (sudden agitation, insults, and fighting), tonic posturing, and complex motor activities. The epileptogenic zone assessed by SEEG involved the mesial frontal areas, especially the anterior cingulate cortex (ACC) in 8 patients, whereas lateral frontal onset with an early spread to the ACC was seen in the other patient. Ictal pouting associated with emotional changes and hypermotor behavior had high localizing value for rostroventral “affective” ACC, whereas less intense facial expressions were related to the dorsal “cognitive” ACC. Fluorodeoxyglucose positron emission tomography demonstrated the involvement of both the ACC and lateral cortex including the anterior insula in all cases. We propose that IP is sustained by reciprocal mesial and lateral frontal interactions involved in emotional and cognitive processes, in which the ACC plays a pivotal role.