Surgery for drug‐resistant tuberous sclerosis complex‐associated epilepsy: who,when, and what

Objective: Tuberous sclerosis complex (TSC) is a multisystem genetic disorder associated with refractory early‐onset epilepsy. Current evidence supports surgery as the intervention most likely to achieve long‐term seizure freedom, but no specific guidelines are available on TSC pre‐surgical workup. This critical review assesses which TSC patients are suitable for surgical treatment, when pre‐surgical evaluation should start, and what degree of surgical resection is optimal for postsurgical outcome. Methods: We searched for publications from 2000 to 2020 in Pubmed and Embase using the terms “tuberous sclerosis,” “epilepsy,” and “epilepsy surgery”. To evaluate postsurgical seizure outcome, we selected only studies with at least one year of follow‐up. Results: Overall, we collected data on 1,026 patients from 34 studies. Age at surgery ranged from one month to 54 years. Mean age at surgery was 8.41 years. Of the diagnostic non‐invasive pre‐surgical tools, MRI and video‐EEG were considered most appropriate. Promising data for epileptogenic tuber detection is provided from invasive SEEG studies. Data on surgery and related outcome were available for 769 patients. Seizure freedom was seen in 64.4% of patients who underwent tuberectomy, 68.9% treated with lobectomy and 65.1% with multilobar resection. The most effective surgical approach was lobectomy, even though more recently tuberectomy associated with the resection of the perituberal area seems to be the best approach to reach seizure freedom. Published postsurgical seizure freedom rates in patients with TSC were between 65% and 75%, but reduced to 48%‐57% over longer follow‐up periods. Early surgery might positively affect neurodevelopmental trajectory in some patients, even though data on cognitive outcome are still to be confirmed with longitudinal studies. Significance: Considering the strong correlation between epilepsy duration and neurocognitive outcome, all patients with TSC ought to be referred early to a dedicated epilepsy centre for individually tailored pre‐surgical evaluation by a multi‐disciplinary epilepsy surgery team.     

A 16‐year‐old girl with focal seizures and impaired awareness: divergent non‐invasive data related to a diffuse epileptogenic network

We describe a patient with medically refractory focal epilepsy who presented with divergent non‐invasive data, with MRI revealing hippocampal sclerosis and EEG indicating involvement of the occipital lobe. A localized corticectomy over the occipital convexity was performed based on intracranial EEG recording. The patient was seizure‐free after four years of follow‐up. Electroclinical hypotheses and challenges of defining the epileptogenic network are discussed.     

Ictal SPECT is useful in localizing the epileptogenic zone in infants with cortical dysplasia

Aims. To assess the localizing value of ictal SPECT in very young epilepsy surgery candidates when cerebral haemodynamic responses are known to be immature. Methods. We retrospectively studied 13 infants with intractable focal epilepsy caused by focal cortical dysplasia (FCD). Completeness of resection of the (1) ictal SPECT hyperperfusion zone and (2) cerebral cortex with prominent ictal and interictal abnormalities on intracranial EEG (ECoG or long‐term invasive monitoring) and the MRI lesion, when present, were correlated with postoperative seizure outcome. Results. All five patients with complete resection of the ictal SPECT hyperperfusion zone were seizure‐free compared to only one of eight patients with incomplete or no excision of hyperperfusion zones (p=0.00843). Similar results were noted for the MRI/iEEG‐defined epileptogenic region; five of six patients with complete removal were seizure‐free, whereas only one of seven incompletely resected patients was seizure‐free (p=0.02914). All four patients who underwent complete resection of both regions were seizure‐free compared to none of the six with incomplete resection (p=0.01179). Conclusion. Despite age‐related differences in cerebral perfusion, ictal SPECT provides useful localization data in infants with FCD. Complete resection of the hyperperfused regions is a strong predictor of favourable outcome. The added information may alleviate the need for invasive EEG evaluations in some patients.     

What is needed for resective epilepsy surgery from a neurosurgical point of view?

Invasive versus non-invasive epileptogenic zone definition was analyzed in a series of 89 patients operated on for drug-resistant epilepsy. In the group of 69 cortical resections, 26% needed invasive recordings, 13.5% when foreign-tissue lesions had been detected by MRI and 32% when were absent. In this last group temporal resections had invasive EEG recordings in 23.5% versus 54.5% when the epileptogenic zone was extratemporal. In a group of 43 temporal resections with more than one year follow-up MRI has detected some abnormality in 84%. Excluding focal lesions, MRI detected hippocampal/temporal lobe atrophy in 66% of the cases in agreement with other noninvasive tests and in 4% contralateral to the epileptogenic zone located by subdural strips. The outcome analysis showed that 85% of the patients with MRI-EEG agreement were seizure free in contrast to only 43% when MRI was non-lateralizing. Future studies has to be oriented to better understand the epileptic process of patients without MRI abnormalities.     

Intracranial investigation of a patient with nodular heterotopia and hippocampal sclerosis: dealing with a dual pathology

The pre‐operative assessment and surgical management of patients with dual pathology is challenging. We describe a patient with drug‐resistant focal epilepsy with hippocampal sclerosis and extensive periventricular nodular heterotopia in the same hemisphere. The semiology, scalp EEG, and imaging were divergent, but the presence of focal interictal and ictal epileptic discharges of the putative ictal onset zone resulted in successful localization of the epileptogenic zone. A less aggressive resection was performed based on intracranial EEG recording. The patient has been seizure‐free for three years since resection. Electroclinical hypotheses and challenges in defining the epileptogenic network are discussed.     

Drug‐resistant parietal lobe epilepsy: clinical manifestations and surgery outcome

Aim. We reviewed a large surgical cohort to investigate the clinical manifestations, EEG and neuroimaging findings, and postoperative seizure outcome in patients with drug‐resistant parietal lobe epilepsy (PLE). Methods. All drug‐resistant PLE patients, who were investigated for epilepsy surgery at Jefferson Comprehensive Epilepsy Center between 1986 and 2015, were identified. Demographic data, seizure data, EEG recordings, brain MRI, pathological findings, and postsurgical seizure outcome were reviewed. Results. In total, 18 patients (11 males and seven females) were identified. Sixteen patients (88%) had tonic‐clonic seizures, 12 (66%) had focal seizures with impaired awareness, and 13 (72%) described auras. Among 15 patients who had brain MRI, 14 patients (93%) had parietal lobe lesions. Only three of 15 patients (20%) who had interictal scalp EEG recordings showed parietal interictal spikes. Of 12 patients with available ictal surface EEG recordings, only three patients (25%) had parietal ictal EEG onset. After a mean follow‐up duration of 8.6 years, 14 patients (77.7%) showed a favourable postoperative seizure outcome. Conclusion. In patients with PLE, semiology and EEG may be misleading and brain MRI is the most valuable tool to localize the epileptogenic zone. Postsurgical seizure outcome was favourable in our patients with drug‐resistant parietal lobe epilepsy.     

Lesion‐negative anterior cingulate epilepsy

MRI‐negative anterior cingulate epilepsy is a rare entity. Herein, we describe a case of MRI and functional imaging‐negative intractable frontal lobe epilepsy in which, initially, secondary bilateral synchrony of surface and intracranial EEG and non‐lateralizing semiology rendered identification of the epileptogenic zone difficult. A staged bilateral stereotactic EEG exploration revealed a very focal, putative ictal onset zone in the right anterior cingulate gyrus, as evidenced by interictal and ictal high‐frequency oscillations (at 250 Hz) and induction of seizures from the same electrode contacts by 50‐Hz low‐intensity cortical stimulation. This was subsequently confirmed by ILAE class 1 outcome following resection of the ictal onset and irritative zones. Histopathological examination revealed focal cortical dysplasia type 1b (ILAE Commission, 2011) as the cause of epilepsy. The importance of anatomo‐electro‐clinical correlation is illustrated in this case in which semiological and electrophysiological features pointed to the anatomical localization of a challenging, MRI‐negative epilepsy. [Published with video sequence]     

Type II focal cortical dysplasia: electroclinical phenotype and surgical outcome related to imaging

Purpose: Type II focal cortical dysplasia (TTFCD), a highly epileptogenic lesion with severe epilepsy curable by surgery, is missed by magnetic resonance imaging (MRI) in about one third of cases. Little is known about the electroclinical presentation in these MRI‐negative patients and a poor surgical outcome is frequently reported. We compared the clinical and neurophysiologic features in MRI‐negative and MRI‐positive cases in order to better identify candidates for surgery. Methods: Among 62 consecutive TTFCD patients (38 male, 24 female; 7–52 years old; 22 children) operated for intractable epilepsy, 25 (40%) presented negative MRI findings. We compared the history of epilepsy; the type, frequency, and distribution of seizures; neurologic examination cognitive and psychiatric impairment; interictal‐ictal electroencephalography (EEG) and stereo‐EEG (SEEG) data, fluorodeoxyglucose positron emission tomography (FDG‐PET) data, neuropathologic findings; and surgical outcome in the MRI‐negative and the MRI‐positive groups. Key Findings: Severe partial epilepsy beginning in childhood, high seizure frequency including status epilepticus, stereotyped seizures suggestive of precise brain localization, extratemporal location and functional area involvement were characteristic and similarly found in both groups. On EEG, pseudorhythmic activity was found in about 40% of patients in each group. SEEG recordings demonstrated the typical pattern characterizing TTFCD in both groups. FDG‐PET had a localization value in 84% of the MRI‐negative cases and helped to delineate the dysplastic cortex in 65% of the MRI‐positive cases. The combination of imaging and neurophysiologic data allowed us to perform safe and restricted resections, limited to a single gyrus in more than half of all cases. In addition, we were able to avoid invasive monitoring in most MRI‐positive cases and even in some selected MRI‐negative cases. The proportion of patients with a favorable surgical outcome was comparable in both groups (88% in MRI‐negative and 94% in MRI‐positive cases). The main difference between the groups was a significantly higher frequency of sleep‐related epilepsy in the MRI‐negative group (p = 0.028). This phenotypic characteristic provides a new argument for TTFCD in MRI‐negative extratemporal epilepsy. Significance: These results lead us to consider that children or adult patients in whom electroclinical data suggest TTFCD, are highly suitable for surgery, especially for cryptogenic sleep‐related epilepsy.     

Coregistration of multimodal imaging is associated with favourable two‐year seizure outcome after paediatric epilepsy surgery

Aims. Multimodal coregistration uses multiple image datasets coregistered to an anatomical reference (i.e. MRI), allowing multiple studies to be viewed together. Commonly used in intractable epilepsy evaluation and generally accepted to improve localization of the epileptogenic zone, data showing that coregistration improves outcome is lacking. We compared seizure freedom following epilepsy surgery in paediatric patients, evaluated before and after the use of coregistration protocols at our centre, to determine whether this correlated with a change in outcome. Methods. We included paediatric epilepsy surgery patients with at least one anatomical and one functional neuroimaging study as part of their presurgical evaluation. Preoperatively designated palliative procedures and repeat surgeries were excluded. Multiple pre‐, peri‐, and postoperative variables were compared between groups with the primary outcome of seizure freedom. Results. In total, 115 were included with an average age of 10.63 years (0.12–20.7). All evaluations included video‐EEG (VEEG) and MRI. Seven (6%) had subtraction single‐photon emission CT (SPECT), 46 (40%) had positron emission tomography (PET), and 62 (54%) had both as part of their evaluation. Sixty (52%) had extratemporal epilepsy and 25 (22%) were MRI‐negative. Sixty‐eight (59%) had coregistration. Coregistered patients were less likely to undergo invasive EEG monitoring (p=0.045) and were more likely to have seizure freedom at one (p=0.034) and two years (p<0.001) post‐operatively. A logistic regression accounting for multiple covariates supported an association between the use of coregistration and favourable post‐surgical outcome. Conclusions. Coregistered imaging contributes to favourable postoperative seizure reduction compared to visual analysis of individual modalities. Imaging coregistration is associated with improved outcome, independent of other variables after surgery. Coregistered imaging may reduce the need for invasive EEG monitoring, likely due to improved confidence in presurgical localization. These findings support the use of multimodal coregistered imaging as part of the presurgical assessment in patients evaluated for surgical treatment of intractable epilepsy.     

Ictal kissing in a patient with right frontal lobe epilepsy

When performing pre‐surgical evaluation of patients with refractory epilepsy, the analysis of seizure semiology is one of the key elements used to generate a hypothesis about the location of the epileptogenic zone. Ictal kissing is a very rarely observed ictal automatism described in patients with temporal lobe epilepsy. We present a 62‐year‐old man who was referred to our epilepsy centre for comprehensive evaluation. During prolonged video‐EEG monitoring, six focal‐onset hyperkinetic seizures were registered. In five seizures, the patient repeatedly produced sonorous kisses “into the air”. Initial ictal EEG pattern consisted of rhythmic theta or alpha activity at the right fronto‐polar and fronto‐medial electrodes. MRI depicted focal cortical dysplasia located in the right prefrontal medial cortex. This case suggests that ictal kissing can also occur in the setting of right frontal lobe epilepsy; we therefore believe that this observation expands the anatomo‐clinical correlation for this rare ictal automatism. [Published with video sequences].     

Identification of Frontal Lobe Epileptic Foci in Children Using Positron Emission Tomography

Summary: Purpose: Presurgical evaluation for intractable frontal lobe epilepsy (FLE) is difficult and invasive, partly because anatomic neuroimaging studies with computed tomography (CT) and magnetic resonance imaging (MRI) typically do not show a discrete lesion. In adult patients with FLE, functional neuroimaging of glucose metabolism with positron emission tomography (PET) is less sensitive in detecting focal metabolic abnormalities than in temporal lobe epilepsy (TLE). Comparable data on children with FLE are not available. Methods: We used high-resolution PET scanning of glucose metabolism to evaluate 13 children (age 17 months to 17 years; mean age 9.5 years) with intractable FLE being considered for surgical treatment. Only children with normal CT and MRI scans were included. Results: Hypometabolism including the frontal lobe was evident in 12 of the 13 children, was unilateral in 11 of 13, and was restricted to the frontal lobe in 8 of 13. One child showed bilateral frontal cortex hypometabolism and another had anictal PET scan demonstrating unilateral frontal cortex hyper-metabolism surrounded by hypometabolism. Additional hypo–metabolic areas outside the frontal cortex were observed in 5 children in parietal and/or temporal cortex. Localization of seizure onset on scalp EEG was available in 10 children and corresponded to the location of frontal lobe PET abnormality in 8. However, in 4 of the 10 children, the extent of hypometabolism exceeded the epileptogenic region indicated by ictal EEG. In 2 of the 13 children, the abnormality evident on EEG was more extensive than that evident on PET. In the remaining 3 children for whom only interictal EEG data were available, the PET foci did not correspond in location to the interictal EEG abnormalities. In 11 of the 13 children, the presumed region of seizure onset in the frontal lobe, as based on analysis of seizure semiology, corresponded to the locations of frontal lobe glucose metabolism abnormalities. Conclusions: Although high-resolution PET appears to be very sensitive in localizing frontal lobe glucose metabolic abnormalities in children with intractable FLE and normal CT/ MRI scans, the significance of extrafrontal metabolic disturbances requires further study; these may represent additional epileptogenic areas, effects of diaschisis, seizure propagation sites, or secondary epileptogenic foci.     

Epilepsy surgery in patients with normal or nonfocal MRI scans: integrative strategies offer long-term seizure relief

PURPOSE: Excisional surgery achieves seizure freedom in a large proportion of children with intractable lesional epilepsy, but the outcome for children without a focal lesion on MRI is less clear. We report the outcome of a cohort predominantly of children with nonlesional intractable partial epilepsy undergoing resective surgery. METHODS: We studied 102 patients with nonlesional intractable partial epilepsy who underwent excisional surgery. The epileptogenic region was identified by integrating clinical exam and video-EEG data complemented by ictal SPECT (n = 40), PET (n = 10), extraoperative subdural monitoring (n = 80), and electrocorticography (n = 22). All patients had follow-up greater than 2 years, 76 patients had 5-year follow-up, and 43 patients had 10-year follow-up. RESULTS: A total of 66 resections were unilobar; 36 were multilobar. One patient died of causes unrelated to seizures or surgery. At 2-year follow-up, 44 of 101 patients were seizure-free, 15 experienced >90% reduction, 17 had >50% reduction, and 25 were unchanged. At 5-year follow-up, 35 of 76 patients were seizure-free, 12 experienced >90% reduction, 12 had >50% reduction, and 17 were unchanged. At 10-year follow-up, 16 of 43 patients were seizure-free, 13 experienced >90% reduction, 7 had >50% reduction, and 7 were unchanged. Outcomes correlated with the presence of convergent focal interictal spikes (p < 0.005) on the scalp EEG and completeness of resection (p < 0.0005). CONCLUSIONS: Our findings demonstrate that excisional surgery is successful in the majority of children with nonlesional partial epilepsy. A multimodal integrative approach can minimize the size of resection and alleviate the need for invasive EEG monitoring. Focal interictal spikes and completeness of resection predict good outcome. The benefits of surgery are long-lasting.     

Paediatric epilepsy surgery in the posterior cortex: a study of 62 cases

Past surgical series have emphasized the diagnostic complexity of posterior cortex epilepsy. Available data are sparse, especially in children, and most published series report a high number of surgical failures and post‐operative neurological deficits. In this article, we present a paediatric cohort of 62 children who underwent surgery for drug resistant posterior cortex epilepsy before the age of 16 years with a mean post‐operative follow‐up of 6.94 years (range: 2–16). Mean age at epilepsy onset was 3.2 years and 28 children (45%) had onset before 1 year of age. The mean age at surgery was 7.9 years (range: 1–16). Daily seizures were present in 63% of children. MRI was positive in 58 cases (93.5%) and invasive stereo‐EEG was judged mandatory in 24/62 (39%) of patients. Surgery was confined to the parietal lobe in 11 children, the occipital lobe in 8, the occipito‐parietal region in four, the occipito‐temporal region in 18, and involved both the temporal and parietal lobes in the remaining 21. Following surgery, 53 subjects (85.5%) remained seizure‐free and among those who underwent a SEEG procedure, 75% achieved seizure freedom. Focal cortical dysplasia was the most frequent histopathological diagnosis (50%), followed by tumoural (24%) and gliotic lesions (14.5%). An older age at epilepsy onset, the presence of a rather restricted epileptogenic area, and a complete resection of the epileptogenic zone were predictive of a favourable surgical outcome. These results demonstrate that a good surgical outcome is possible in children with drug resistant posterior cortex epilepsy. Accurate analysis of the chronology of ictal semiology and electrophysiological features, viewed in the context of the complete electroclinical pattern, provides a topographical orientation for posterior cortex epilepsy and, together with the presence of a lesion detectable on imaging, may improve the rate of surgical success of posterior cortex epilepsy at paediatric age.     

无影像学病灶顽固性癫癎病人的手术治疗

目的探讨脑MRI与CT为阴性的顽固性癎癫病人的手术治疗及术前评估时应注意的问题。方法回顾性分析MRI及CT为阴性表现的22例癫癎手术病人的资料,按照Engel疗效分级标准,将其效果分为满意组(10例)与非满意组(12例),对两组的术前检查情况进行对比。结果手术后整体满意率为45%。发作间期头皮EEG癫癎波局限在单一脑叶者,满意组中多于非满意组;颅内电极发作期脑电图局限性起源者,满意组中多于非满意组;单光子发射断层显像(SPECT)报告结果与手术部位关系各指标比较,两组无显著性差异;单脑叶切除术者,满意组中多于非满意组。结论MRI为阴性的癫癎手术效果不如病灶性癫癎。术前头皮视频脑电图(VEEG)发作间期性波局限在单一脑叶者治疗效果优于非局限者。手术前评估除重视各项非侵袭性检查外,颅内电极检查往往是不可缺少的。发作间期SPECT目前还不能做为一项决定性的定位手段。多脑叶切除手术效果不优于单脑叶切除,手术的关键是致灶的彻底切除。     

MRI阴性难治性儿童额叶癫的脑电图特点及外科治疗(附25例分析)

目的总结分析MRI阴性难治性儿童额叶癫的脑电图特点及手术治疗方法和预后。方法回顾性分析25例MRI阴性难治性儿童额叶癫病例的治疗经验。根据病儿发作的症状及头皮脑电图特点,在可疑的癫灶起源区植入颅内电极,行皮质脑电图(ECoG)监测,根据其间期、发作期特点制定癫灶切除计划。病灶位于功能区时术中行皮质电刺激,进行癫灶及功能区定位。结果本组行单纯前额叶切除7例,前额叶切除加局限性皮质切除4例,局限性皮质切除加皮质热灼6例,前额叶外侧切除加局限性皮质切除3例,前额叶内侧切除加局限性皮质切除5例(其中3例加胼胝体切开);对其中6例灶位于功能区病人于局限性皮质切除后加行皮质热灼。无手术死亡及严重并发症发生,随访12～24个月,手术后疗效按Wilson标准评判,癫发作完全消失7例,发作次数显著减少8例,发作程度减轻6例,无明显改善4例;优良率为84%。结论分析头皮脑电图初步定位癫灶后,再应用颅内电极进行精确致灶及功能区定位,制定个体化治疗计划,选择前额叶切除、局限性皮质切除、皮质热灼、胼胝体切开或根据需要联合多种术式,是治疗MRI阴性难治性儿童额叶癫的有效方法。     

MEG predicts outcome following surgery for intractable epilepsy in children with normal or nonfocal MRI findings

PURPOSE: To identify the predictors of postsurgical seizure freedom in children with refractory epilepsy and normal or nonfocal MRI findings. METHODS: We analyzed 22 children with normal or subtle and nonfocal MRI findings, who underwent surgery for intractable epilepsy following extraoperative intracranial EEG. We compared clinical profiles, neurophysiological data (scalp EEG, magnetoencephalography (MEG) and intracranial EEG), completeness of surgical resection and pathology to postoperative seizure outcomes. RESULTS: Seventeen children (77%) had a good postsurgical outcome (defined as Engel class IIIA or better), which included eight (36%) seizure-free children. All children with postsurgical seizure freedom had an MEG cluster in the final resection area. Postsurgical seizure freedom was obtained in none of the children who had bilateral MEG dipole clusters (3) or only scattered dipoles (1). All five children in whom ictal onset zones were confined to < or = 5 adjacent intracranial electrodes achieved seizure freedom compared to three of 17 children with ictal onset zones that extended over >5 electrodes (p = 0.002). None of six children with more than one type of seizure became seizure-free, compared to eight of 16 children with a single seizure type (p = 0.04). Complete resection of the preoperatively localized epileptogenic zone resulted in seizure remission in 63% (5/8) and incomplete resections, in 21% (3/14) (p = 0.06). Age of onset, duration of epilepsy, number of lobes involved in resection, and pathology failed to correlate with seizure freedom. CONCLUSIONS: Surgery for intractable epilepsy in children with normal MRI findings provided good postsurgical outcomes in the majority of our patients. As well, restricted ictal onset zone predicted postoperative seizure freedom. Postoperative seizure freedom was less likely to occur in children with bilateral MEG dipole clusters or only scattered dipoles, multiple seizure types and incomplete resection of the proposed epileptogenic zone. Seizure freedom was most likely to occur when there was concordance between EEG and MEG localization and least likely to occur when these results were divergent.     

Ictal spitting in non‐dominant temporal lobe epilepsy: an anatomo‐electrophysiological correlation

We report a patient presenting drug‐resistant, non‐dominant temporal lobe epilepsy with ictal spitting and prosopometamorphopsia, both extremely rare semiologies. Second‐phase pre‐surgical monitoring was performed using SEEG due to lesion‐negative imaging and the rare semiology. The seizure onset zone was delimited to the right anterior hippocampus and the temporobasal cortex, with the propagation zone within the entorhinal cortex. Interestingly, direct electrical stimulation to the entorhinal cortex, which was reproduced in a number of trials, evoked spitting without leading to seizures or post‐discharges. After the resection of the epileptogenic zone, the patient remained seizure‐free without AEDs for a follow‐up period of five years (Engel Class 1a). The neuropathology revealed a focal cortical dysplasia type FCD‐Ia. Spectral analysis of intracranial ictal EEG (iEEG) data suggested a possible role of the basal temporal and entorhinal cortex as a necessary node in ictal spitting. [Published with video sequences on www.epilepticdisorders.com ].     

Additional clinical value of voxel‐based morphometric MRI post‐processing for MRI‐negative epilepsies: a prospective study

Aims: Magnetic resonance imaging is of paramount importance in the presurgical evaluation of drug resistant epilepsy. Detection of a potentially epileptogenic lesion significantly improves seizure outcome after surgery. To optimize the detection of subtle lesions, MRI post‐processing techniques may be of essential help. Methods: In this study, we aimed to evaluate the detection rate of the voxel‐based morphometric analysis program (MAP) in a prospective trial. We aimed to study the MAP+ findings in terms of their clinical value in the decision‐making process of the presurgical evaluation. Results: We included, prospectively, 21 patients who had negative MRI by visual analysis. In a first step, results of the conventional non‐invasive presurgical evaluation were discussed, blinded to the MAP results, in multidisciplinary patient management conferences to determine the possible seizure onset zone and to set surgical or invasive evaluation plans. Thereafter, MAP results were presented, and the change of initial clinical plan was recorded. All MAP detections were reaffirmed by a neuroradiologist with epilepsy expertise. For the 21 patients included, mean age at the time of patient management conference was 26 years (SD 15 +/‐ years, range: 5–54 years). In total, 4/21 had temporal lobe epilepsy and 17/21 had extra‐temporal lobe epilepsy. MAP was positive in 10/21 (47%) patients and in 6/10 (60%) a diagnosis of focal cortical dysplasia was confirmed after neuroradiologist review, corresponding to a 28% detection rate. MAP+ findings had a clear impact on the initial management in 7/10 patients (7/21, 33% of all patients), which included an adaptation of the intracranial EEG plan (6/7 patients), or the decision to proceed directly to surgery (1/7 patients). Conclusion: MRI post‐processing using the MAP method yielded an increased detection rate of 28% for subtle dysplastic lesions in a prospective cohort of MRI‐negative patients, indicating its potential value in epilepsy presurgical evaluation.     

Neuroimaging and presurgical evaluation of symptomatic epilepsies

The goal of presurgical evaluation of intractable epilepsy is to identify epileptogenic regions in the brain. From our experience of 38 cases of resective epilepsy surgery from the last 3 years, ictal SPECT was considered the most sensitive at detecting focal changes relating to seizures compared to other neuroimaging modalities, such as MRI, FDG-PET, SPECT and MEG. At interictal state, on the other hand, FDG-PET was most sensitive, especially in cases with focal cortical dysplasia, which is often MRI-invisible. In dysplastic tumors, MRI showed the highest concordance rate to clinically verified epileptogenic regions. Activation studies using functional neuroimaging such as PET and fMRI is useful to evaluate brain functions at epileptogenic regions presurgically. The role of functional brain imaging in epilepsy surgery is considered to be: (i). case selection for resective surgery, (ii). case selection for invasive EEG monitoring, and (iii). navigation of electrode placement and cortical resection.     

Surgical treatment of medically refractory epilepsy in childhood

Twenty five percent of children with epilepsy continue to seize despite best medical management and may be defined as medically refractory. Many children with medically refractory localization-related epilepsy, i.e. seizures which originate in a particular area of brain and secondarily spread to involve other brain regions, may benefit from a variety of surgical treatments including hemispherectomy, corpus callosotomy, focal cortical resection of the temporal lobe, focal cortical resection of extratemporal regions of brain, and multiple subpial resections. A successful outcome from epilepsy surgery is generally defined as a seizure-free state with no imposition of neurologic deficit. In order to achieve these twin goals two criteria must be fulfilled. First, precise localization of the epileptogenic zone in the brain is necessary. The epileptogenic zone may be defined as the region of epileptogenic cerebral cortex whose removal will result in a seizure-free state. Second, one must determine the anatomic localization of eloquent cortex in brain in order to spare these areas during any planned cortical excision of epileptogenic cortex. Several diagnostic measures may be used to achieve a successful surgical outcome. A clinical history to ascertain the earliest symptom in the clinical progression of the seizure (semiology) is imperative as is ictal and interictal scalp EEG, neuropsychological testing, magnetic resonance imaging (MRI), positron emission tomography (PET), single photon emission computerized tomography (SPECT), interictal magnetoencephalography (MEG). In the typical child undergoing evaluation for epilepsy surgery, if the clinical, neuropsychological, EEG, and radiological data are all concordant and point to the same area of epileptogenicity in brain, cortical excision of the suspected epileptogenic zone is undertaken. However, if the data are discordant, and/or the epileptogenic zone resides wholly or in part within eloquent cortex, invasive intracranial monitoring from depth and/or subdural electrodes during a seizure is required to map out the areas of epileptogenicity in brain. The assessment of potential risks and benefits for this type of epilepsy surgery in children involve complex age-related issues, including the possible impact of uncontrolled seizures, medication, or surgery, on learning and development.