Strategies for surgical treatment of epilepsies in developing countries

Epilepsy surgery has been proved to be efficacious, safe and cost-effective in developing countries. However, the success of epilepsy surgery depends on selecting suitable candidates based on the available resources and technologies. Some of the challenges to provide appropriate surgical treatment for epilepsy patients in developing countries include providing human and technological resources and developing realistic presurgical protocols. Detection of ideal candidates for epilepsy surgery is possible for well-trained epileptologists with the help of basic investigative technologies, for example, magnetic resonance imaging (MRI) and electroencephalography (EEG). Patients with potentially epileptogenic, well-circumscribed lesions on MRI and patients with mesial temporal lobe epilepsy (MTLE) are reasonable candidates for surgery. Palliative epilepsy surgeries include corpus callosotomy and other disconnections. These operations are feasible in developing countries with a knowledgeable team consisting of an epileptologist, neurosurgeon and technicians and with using MRI and EEG as basic investigative technologies.     

Neuroimaging and connectomics of drug‐resistant epilepsy at multiple scales: From focal lesions to macroscale networks

Epilepsy is among the most common chronic neurologic disorders, with 30%‐40% of patients having seizures despite antiepileptic drug treatment. The advent of brain imaging and network analyses has greatly improved the understanding of this condition. In particular, developments in magnetic resonance imaging (MRI) have provided measures for the noninvasive characterization and detection of lesions causing epilepsy. MRI techniques can probe structural and functional connectivity, and network analyses have shaped our understanding of whole‐brain anomalies associated with focal epilepsies. This review considers the progress made by neuroimaging and connectomics in the study of drug‐resistant epilepsies due to focal substrates, particularly temporal lobe epilepsy related to mesiotemporal sclerosis and extratemporal lobe epilepsies associated with malformations of cortical development. In these disorders, there is evidence of widespread disturbances of structural and functional connectivity that may contribute to the clinical and cognitive prognosis of individual patients. It is hoped that studying the interplay between macroscale network anomalies and lesional profiles will improve our understanding of focal epilepsies and assist treatment choices.     

Hyperventilation revisited: physiological effects and efficacy on focal seizure activation in the era of video-EEG monitoring

PURPOSE: Hyperventilation is an activation method that provokes physiological slowing of brain rhythms, interictal discharges, and seizures, especially in generalized idiopathic epilepsies. In this study we assessed its effectiveness in inducing focal seizures during video-EEG monitoring. METHODS: We analyzed the effects of hyperventilation (HV) during video-EEG monitoring (video-EEG) of patients with medically intractable focal epilepsies. We excluded children younger than 10 years, mentally retarded patients, and individuals with frequent seizures. RESULTS: We analyzed 97 patients; 24 had positive seizure activation (PSA), and 73 had negative seizure activation (NSA). No differences were found between groups regarding sex, age, age at epilepsy onset, duration of epilepsy, frequency of seizures, and etiology. Temporal lobe epilepsies were significantly more activated than frontal lobe epilepsies. Spontaneous and activated seizures did not differ in terms of their clinical characteristics, and the activation did not affect the performance of ictal single-photon emission computed tomography (SPECT). CONCLUSIONS: HV is a safe and effective method of seizure activation during monitoring. It does not modify any of the characteristics of the seizures and allows the obtaining of valuable ictal SPECTs. This observation is clinically relevant and suggests the effectiveness and the potential of HV in shortening the presurgical evaluation, especially of temporal lobe epilepsy patients, consequently reducing its costs and increasing the number of candidates for epilepsy surgery.     

Prognostic factors in neocortical epilepsy surgery: multivariate analysis

PURPOSE: Defining prognostic factors for neocortical epilepsy surgery is important for the identification of ideal candidates and for predicting the prognosis of individual patients. We use multivariate analysis to identify favorable prognostic factors for neocortical epilepsy surgery. METHODS: One hundred ninety-three neocortical epilepsy patients, including 91 without focal lesions on MRI, were included. Sixty-one had frontal lobe epilepsy (FLE), 80 had neocortical temporal lobe epilepsy (nTLE), 21 had parietal lobe epilepsy (PLE), and 22 had occipital lobe epilepsy (OLE). The primary outcome variable was patient status >or=2 years after surgery (i.e., seizure free or not). Clinical characteristics and the recent presurgical diagnostic modalities were considered as probable prognostic factors. Univariate and standard multiple logistic regression analyses were used to identify favorable prognostic factors. RESULTS: The seizure-free rate was 57.5%. By univariate analysis, a focal lesion on MRI, localized ictal onset on surface EEG, epilepsies other than FLE, localized hypometabolism on fluorodeoxyglucose-positron emission tomography (FDG-PET), and pathologies other than cortical dysplasia were significantly associated with a seizure-free outcome (p<0.05). Multivariate analysis revealed that a focal lesion on MRI (p=0.003), correct localization by FDG-PET (p=0.007), and localized ictal onset on EEG (p=0.01) were independent predictors of a good outcome. CONCLUSIONS: The presence of a focal lesion on MRI, correct localized hypometabolism on FDG-PET, or localized ictal rhythms on EEG were identified as predictors of a seizure-free outcome. Our results suggest that these findings allow the selection of better candidates for neocortical epilepsy surgery.     

MR-Spektroskopie und T2-Relaxometrie bei kryptogener Temporallappenepilepsie und postoperativer Prognose

The preoperative localization of pharmaco-resistant focal epilepsies before surgery and the prognosis concerning seizure outcome are both of importance. In addition to conventional MRI diagnostics for the detection of small epileptogenic lesions, proton magnetic resonance spectroscopic imaging (HMR spectroscopy) can be useful for assessing the bilaterality of pathological changes in cryptogenic temporal lobe epilepsies. The technique and findings of HMR spectroscopy are reported in patients with cryptogenic temporal lobe epilepsies. The findings indicate that chemical shift imaging (CSI) investigations of the ipsilateral and contralateral hemispheres provide important information for the prediction of seizure outcome after epilepsy surgery.     

An audit of the presurgical evaluation and patient selection for extratemporal resective epilepsy surgery in a resource-poor country

PurposeThe selection of ideal candidates for extratemporal resective epilepsy surgery is a challenge in resource-poor countries because of the limited presurgical diagnostic facilities and their affordability. To audit the presurgical evaluation strategy and selection for extratemporal resective epilepsy surgery in a resource-poor region.MethodsFrom the prospective database maintained at an epilepsy surgery center in southern India, we reviewed the data of consecutive patients who underwent presurgical evaluation from January 2005 through December 2008 for antiepileptic drug-resistant focal epilepsies emanating from the frontal, parietal and occipital lobes. Out of 285 patients, only 71 (24.9%) underwent resective surgery; the remaining 214 (75.1%) patients could not be selected for surgery. We inquired the reasons for their exclusion from surgery.ResultsThe difference in the rates of seizure-free outcome between surgical and non-surgical groups was highly significant (73.2% vs. 7.7%, P < 0.0005). The major reasons for exclusion from surgery were normal MRI in 107 (50%), inability to afford invasive EEG monitoring in 40 (18.7%) and lesion location adjacent to eloquent cortical areas in 27 (12.6%) patients. While clustering of seizures and presence of preoperative neurological deficits favored surgical selection, the presence of secondary generalized seizures and discordant interictal epileptiform abnormalities were associated with exclusion from surgery.ConclusionsWe conclude that, in a resource-poor country, ideal candidates for extratemporal resective epilepsy surgery are those with well-circumscribed lesions not adjoining eloquent cortical areas. In such patients, concordant EEG findings and absence of preoperative secondary generalized seizures reinforce selection for surgery.     

Symptomatic occipital lobe epilepsy following neonatal hypoglycemia

This study reports on the clinical, electrophysiologic, and neuroradiologic aspects of patients with epilepsy secondary to neonatal hypoglycemia. Fifteen patients with epilepsy and/or posterior cerebral lesions, and neonatal hypoglycemia were studied in the epilepsy clinic between February 1990 and March 2003. The mean age was 12 years. The different types of neonatal hypoglycemia were as follows: four patients had transitional-adaptive, seven classic transient, two secondary-associated, and two severe recurrent hypoglycemia. As to epilepsy, we recognized a larger group of 12 patients characterized by focal seizures and posterior abnormalities on the electroencephalogram, the majority of whom had a good outcome, and a second group of two patients presenting electroclinical features of encephalopathy with refractory seizures. All patients except two manifested parieto-occipital lesions on neuroradiologic images. Neurologic examination was normal in one patient. Six patients had microcephaly; eight manifested visual disturbances. Fourteen patients were mentally retarded. One had a pervasive developmental disorder. This study indicates neonatal hypoglycemia may cause posterior cerebral lesions, abnormal findings at neurologic examination, and symptomatic epilepsy, most frequently occipital lobe epilepsy, usually with a good prognosis, and occasionally epileptic encephalopathy with refractory seizures. MRI studies are essential to define the characteristics of cerebral lesions after neonatal hypoglycemia.     

Long-term epilepsy surgery outcomes in patients with MRI-negative temporal lobe epilepsy

Purpose: The outcome of surgery in patients with temporal lobe epilepsy (TLE) and normal high‐resolution magnetic resonance imaging (MRI) has been significantly worse than in patients with unilateral hippocampal damage upon MRI. The purpose of this study was to determine the long‐term outcomes of consecutive true MRI‐negative TLE patients who all underwent standardized preoperative evaluation with intracranial electroencephalography (EEG) electrodes. Methods: In this study we present all adult MRI‐negative TLE surgery candidates evaluated between January 1990 and December 2006 at Kuopio Epilepsy Center in Kuopio University Hospital, which provides a national center for epilepsy surgery in Finland. During this period altogether 146 TLE surgery candidates were evaluated with intracranial electrodes, of whom 64 patients with normal high‐resolution MRI were included in this study. Results: Among the 38 patients who finally underwent surgery, at the latest follow‐up (mean 5.8 years), 15 (40%) were free of disabling seizures (Engel class I) and 6 (16%) were seizure‐free (Engel class IA). Twenty‐one (55%) of 38 patients had poor outcomes (Engel class III–IV). Outcomes did not change compared to 12‐month follow‐up. Histopathologic examination failed to reveal any focal pathology in 68% of our MR‐negative cases. Only patients with noncongruent positron emission tomography (PET) results had worse outcomes (p = 0.044). Discussion: Our results suggest that epilepsy surgery outcomes in MRI‐negative TLE patients are comparable with extratemporal epilepsy surgery in general. Seizure outcomes in the long‐term also remain stable. Modern imaging techniques could further improve the postsurgical seizure‐free rate. However, these patients usually require chronic intracranial EEG evaluation to define epileptogenic areas.     

A child with ictal vocalizations and generalized epilepsy

Ictal vocalizations in the form of both articulate speech and non‐speech vocalizations have been described in focal epilepsies, with seizures originating mainly from the frontal and temporal lobe, however, this phenomenon has not been described in generalized epilepsies. We report the case of an adolescent boy with juvenile‐onset generalized epilepsy who presented with ictal “ovine vocalizations” (resembling the bleating of sheep). The ictal EEG revealed a clear correlate of vocalizations with time‐locked generalized spikes and polyspike discharges. The 3T cerebral MRI ruled out any focal lesion. The boy is currently seizure‐free under valproic acid, after twelve months of follow‐up. We conclude that ictal non‐speech vocalizations may be observed not only in focal or structural epilepsies, but also in generalized epilepsies; the exact underlying mechanism of this phenomenon needs to be further delineated. [Published with video sequence]     

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.     

Localizing and lateralizing features of auras and seizures

The symptomatology of auras and seizures is a reflection of activation of specific parts of the brain by the ictal discharge, the location and extent of which represent the symptomatogenic zone. The symptomatogenic zone is presumably, though not necessarily, in close proximity to the epileptogenic zone, the area responsible for seizure generation, the complete removal or disconnection of which is necessary for seizure freedom. Knowledge about the symptomatogenic zone in focal epilepsy is acquired through careful video/EEG monitoring and behavioral correlation of seizures and electrical stimulation studies. Ictal symptomatogy provides important lateralizing and/or localizing information in the presurgical assessment of epilepsy surgery candidates. As the initial symptoms of epileptic seizures, many types of auras have highly significant localizing or lateralizing value. Similarly, motor signs during focal and secondary generalized seizures, language manifestations, and autonomic features offer reliable clues to the delineation of the epileptogenic zone. Some focal epilepsies (e.g., neocortical temporal lobe epilepsy, insular lobe epilepsy, temporal-plus epilepsies, and parieto-occipital lobe epilepsy) generate seizure manifestations that mimic temporal lobe epilepsy, potentially contributing to surgical failure. To optimize surgical outcome, careful interpretation of ictal symptomatology in conjunction with other components of the presurgical evaluation is required.     

Role of SOX2 mutations in human hippocampal malformations and epilepsy

PURPOSE: Seizures are noted in a significant proportion of cases of de novo, heterozygous, loss-of-function mutations in SOX2, ascertained because of severe bilateral eye malformations. We wished to determine the underlying cerebral phenotype in SOX2 mutation and to test the candidacy of SOX2 as a gene contributing to human epilepsies. METHODS: We examined high-resolution MRI scans in four patients with SOX2 mutations, two of whom had seizures. We determined the Sox2 expression pattern in developing murine brain. We searched for SOX2 mutation in 24 patients with typical hippocampal sclerosis and for common variations in SOX2 in 655 patients without eye disease but with epilepsy, including 91 patients with febrile seizures, 93 with hippocampal sclerosis, and 258 with temporal lobe epilepsy. RESULTS: Striking hippocampal and parahippocampal malformations were seen in all cases, with a history of febrile seizures or epilepsy in two of four cases. The Sox2 expression pattern in developing mouse brain supports the pattern of malformations observed. Mutation screening in patients with epilepsy did not reveal any abnormalities in SOX2. No associations were found between any clinical epilepsy phenotype and common variation in SOX2. CONCLUSIONS: SOX2 haploinsufficiency causes mesial temporal malformation in humans, making SOX2 dysfunction a candidate mechanism for mesial temporal abnormalities associated with chronic epilepsy. However, although mutation of SOX2 in humans causes hippocampal malformation, SOX2 mutation or variation is unlikely to contribute commonly to mesial temporal lobe epilepsy or its structural (hippocampal sclerosis) or historic (febrile seizures) associations in humans.     

Parahippocampal epilepsy with subtle dysplasia: A cause of “imaging negative” partial epilepsy

Purpose: Lesion‐negative refractory partial epilepsy is a major challenge in the assessment of patients for potential surgery. Finding a potential epileptogenic lesion simplifies assessment and is associated with good outcome. Here we describe imaging features of subtle parahippocampal dysplasia in five cases that were initially assessed as having imaging‐negative frontal or temporal lobe epilepsy. Methods: We analyzed the clinical and imaging features of five patients with seizures from the parahippocampal region. Results: Five patients had subtle but distinctive magnetic resonance imaging (MRI) abnormalities in the parahippocampal gyrus. This was a unilateral signal abnormality in the parahippocampal white matter extending into gray matter on heavily T₁‐ and T₂‐weighted images with relative preservation of the gray–white matter boundary on T₁‐weighted volume sequences. Only one of these patients had typical electroclinical unilateral temporal lobe epilepsy (TLE); one mimicked frontal lobe epilepsy, two showed bitemporal seizures, and one had unlocalized partial seizures. All have had surgery; four are seizure‐free (one has occasional auras only, follow‐up 6 months to 10 years), and one has a >50% seizure reduction. Histopathologic evaluation suggested dysplastic features in the surgical specimens in all. Discussion: In patients with lesion‐negative partial epilepsy with frontal or temporal semiology, or in cases with apparent bitemporal seizures, subtle parahippocampal abnormalities should be carefully excluded. Recognizing the MRI findings of an abnormal parahippocampal gyrus can lead to successful surgery without invasive monitoring, despite apparently incongruent electroclinical features.     

The mismatch negativity (MMN) potential as a tool for the functional mapping of temporal lobe epilepsies

Temporal lobe epilepsies are associated with cognitive dysfunctions in memory which are important clues currently used clinically for the lateralization of the epileptic focus in evaluations for epilepsy surgery. Because these lobes also contain the primary auditory cortex, the study of auditory evoked potentials (AEPs) is a candidate, not yet established, complementary method to characterize epilepsy-induced dysfunction. We aimed to establish the clinical usefulness of auditory evoked potentials for the study of pediatric symptomatic temporal lobe epilepsies. A group of 17 patients (ages 4–16) with symptomatic epilepsies undergoing evaluation for epilepsy surgery epilepsy was submitted to auditory evoked potentials using 35-channel scalp EEG recordings. A control group of 10 healthy volunteers was studied with the same protocol. The P100 and mismatch negativity (MMN) potential latencies and normalized amplitudes were studied. We also performed a voxel-based lesion-symptom mapping (VLSM) to determine the anatomical areas associated with changes in the AEPs. Eleven patients had temporal lobe epilepsy, three had frontal lobe epilepsy, and three had occipital lobe epilepsy. Latencies for the P100 were normal in 15/17 and in 11/17 for the MMN, with no consistent correlation with the epilepsy type. The MMN amplitude was abnormal in 7/17 patients, all with temporal lobe epilepsies (sensitivity of 64%). Of these patients, four had a decreased MMN associated with a Heschl's gyrus lesion in the VLSM, and three had an increased MMN associated with hippocampal lesion. No extratemporal epilepsy showed MMN amplitude abnormalities (specificity of 100%). The P100 amplitude was abnormal in 3/17, two with temporal and one with frontal lobe epilepsies. The auditory MMN has a high specificity but a low sensitivity for temporal lobe epilepsy in symptomatic pediatric epilepsies. Amplitude decreases of the MMN are associated with homolateral Heschl's gyrus lesions, and MMN increases with hippocampal lesions.     

Seizure outcome after anterior temporal lobectomy and its predictors in patients with apparent temporal lobe epilepsy and normal MRI

PURPOSE: Very little reliable information is available regarding the role of anterior temporal lobectomy (ATL), optimal presurgical evaluation strategy, post-ATL seizure outcome, and the factors that predict the outcome in patients with medically refractory temporal lobe epilepsy (TLE) and normal high-resolution magnetic resonance imaging (MRI). To be cost-effective, epilepsy surgery centers in developing countries will have to select candidates for epilepsy surgery by using the locally available technology and expertise. METHODS: We reviewed the electroclinical and pathological characteristics and seizure outcome of 17 patients who underwent ATL for medically refractory TLE after being selected for ATL based on a noninvasive selection protocol without the aid of positron emission tomography (PET) or single-photon emission computed tomography (SPECT), despite a normal preoperative high-resolution MRI. RESULTS: Seven (41%) patients achieved an excellent seizure outcome; five of them were totally seizure free. An additional five (29%) patients had >75% reduction in seizure frequency. The following pre-ATL factors predicted an excellent outcome: antecedent history of febrile seizures, strictly unilateral anterior temporal interictal epileptiform discharges (IEDs), and concordant type 1 ictal EEG pattern. All the five patients with pathologically verified hippocampal formation neuronal loss were seizure free. The presence of posterior temporal, bilateral temporal, and generalized IEDs portended unfavorable post-ATL seizure outcome. CONCLUSIONS: A subgroup of patients destined to have an excellent post-ATL outcome can be selected from MRI-negative TLE patients by using history and scalp-recorded interictal and ictal EEG data. The attributes of these patients are antecedent history of febrile seizures, strictly unilateral anterior IEDs, and concordant type 1 ictal EEG pattern.     

EEG predicts surgical outcome in lesional frontal lobe epilepsy

BACKGROUND: Because of the relatively poor results of frontal lobe epilepsy (FLE) surgery, identification of prognostic factors for surgical outcome is of great importance. METHODS: To identify predictive factors for FLE surgery, we analyzed the data of 61 patients (mean age at surgery 19.2) who had undergone presurgical evaluation and resective surgery in the frontal lobe. Postoperative follow-up ranged from 0.5 to 5 years (mean 1.78). Fifty-nine patients had MRI-detectable lesions. Histopathologic examination showed dysplasia (57.4%), tumor (16.4%), or other lesions (26.2%). Thirty postoperatively seizure-free patients were compared with 31 non-seizure-free patients with respect to clinical history, seizure semiology, EEG and neuroimaging data, resected area, and postoperative data including histopathology. RESULTS: Three preoperative and two postoperative variables were related to poor outcome: generalized epileptiform discharges, generalized slowing, use of intracranial electrodes, incomplete resection detected by MRI, and postoperative epileptiform discharges. The only preoperative factor associated with seizure-free outcome was the absence of generalized EEG signs. Multivariate analysis showed that only the absence of generalized EEG signs predicts the outcome independently. Moreover, the occurrence of a somatosensory aura, secondarily generalized seizures, and negative MRI was identified as additional independent risk factors for poor surgical results. CONCLUSIONS: The absence of generalized EEG signs is the most predictive variable for a seizure-free outcome in FLE surgery. Furthermore, nonlesional MRI, somatosensory aura, and secondarily generalized seizures are risk factors for poor surgical results.     

Advances in MRI for 'cryptogenic' epilepsies

Nearly one-third of patients with focal epilepsy experience disabling seizures that are refractory to pharmacotherapy. Drug-resistant focal epilepsy is, however, potentially curable by surgery. Although lesions associated with the epileptic focus can often be accurately detected by MRI, in many patients conventional imaging based on visual evaluation is unable to pinpoint the surgical target. Patients with so-called cryptogenic epilepsy represent one of the greatest clinical challenges in many tertiary epilepsy centers. In recent years, it has become increasingly clear that epilepsies that are considered cryptogenic are not necessarily nonlesional, the primary histopathological substrate being subtle cortical dysplasia. This Review considers the application of new advances in brain imaging, such as MRI morphometry, computational modeling and diffusion tensor imaging. By revealing dysplastic lesions that previously eluded visual assessments, quantitative structural MRI methods such as these have clearly demonstrated an increased diagnostic yield of epileptic lesions, and have provided successful surgical options to an increasing number of patients with 'cryptogenic' epilepsy.     

Adult‐onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy

To evaluate the clinical associations of adult‐onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically‐induced seizures, while 15 had exclusively, or almost exclusively, reflex photically‐induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically‐induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first‐seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.     

Hypermotor seizures in patients with temporal pole lesions

Hypermotor seizures are considered to be characteristic of frontal lobe epilepsy, with only rare occurrence in temporal lobe epilepsy. After noting hypermotor seizures in several patients with lesions involving the pole of the temporal lobe, we investigated whether temporal pole lesions were associated with hypermotor seizures. We systematically reviewed medical records, MRI images and pathological findings in consecutive patients who underwent epilepsy surgery over the preceding 10 years in our institution and identified eight patients with temporal pole lesions and intractable complex partial seizures. We analyzed all recorded seizures for semiology, classifying seizures as hypermotor or typical "psychomotor." Four patients exhibited hypermotor seizure semiology and four patients manifested typical psychomotor seizure characteristics. In patients with hypermotor seizures, scalp EEG tended to demonstrate lateral anterior temporal ictal onset, with lesser involvement of the sphenoidal electrode, while the patients with psychomotor seizures had initial inferomesial temporal rhythmic theta activity. Two patients with hypermotor seizures had implanted frontal and temporal subdural grids demonstrating orbitofrontal spread before hypermotor behavior. Patients underwent either anterior temporal lobectomy or lesionectomy. All improved considerably, with six patients seizure-free since surgery. We conclude that hypermotor seizures occur frequently in patients with temporal pole lesions. A search for temporal pole pathology is recommended for patients with hypermotor seizures and temporal epileptiform discharges. Modification of the surgical approach to include this region should be considered in patients who exhibit hypermotor seizures.     

Epilepsy surgery outcome in children with focal epilepsy due to tuberous sclerosis complex

Tuberous sclerosis complex (TSC) is frequently associated with focal epilepsy due to cerebral tubers. Seizures are the first symptoms in most patients with brain involvement. These epilepsies are frequently severe, drug-resistant and may have a negative impact on the child's global development. Although most epilepsies are multicentric, these patients may be candidates for epilepsy surgery, if it is possible to determine a leading epileptogenic tuber. Nine patients with TSC were examined with long-term video-EEG monitoring, different neuroimaging techniques and neuropsychological tests. A main epileptogenic tuber could be identified in all of our patients. We found good correlations between neuroimaging and EEG. Surgery was performed in eight patients. Seizure outcome was good in all. Two patients became seizure-free, one patient had a single prolonged seizure five days postoperatively, four patients had a significant seizure reduction of more than 75 % and one patient had a seizure reduction of more than 50 % after surgery without additional neurologic deficits. In conclusion, patients with TSC and drug-resistant epilepsy may benefit from epilepsy surgery with reduction in frequency and severity of seizures as well as improved mental and behavioural development leading to a better quality of life. In view of recent developments in functional and metabolic imaging, primary epileptogenic lesions will be more easily detectable in patients with diffuse brain involvement in TSC and surgical treatment may be more specifically applied at an earlier age to a selected subgroup of patients with this disorder.