Retrospective evaluation of low long-term efficacy of antiepileptic drugs and ketogenic diet in 39 patients with CDKL5-related epilepsy.

OBJECTIVE: Mutations in the CDKL5 gene cause an early-onset epileptic encephalopathy. To date, little is known about effective antiepileptic treatment in this disorder. METHOD: Accordingly, the aim of this retrospective study was to explore the role of different antiepileptic drugs (AEDs) and the ketogenic diet (KD) in the treatment of this rare genetic disorder. We evaluated the efficacy in 39 patients with CDKL5 mutations at 3, 6 and 12 months after the introduction of each treatment. One patient was lost to follow-up after 6 and 12 months. RESULTS: The responder rate (>50% reduction in seizure frequency) to at least one AED or KD was 69% (27/39) after 3 months, 45% (17/38) after 6 months and 24% (9/38) after 12 months. The highest rate of seizure reduction after 3 months was reported for FBM (3/3), VGB (8/25), CLB (4/17), VPA (7/34), steroids (5/26), LTG (5/23) and ZNS (2/11). Twelve patients (31%) experienced a seizure aggravation to at least one AED. Most patients showed some but only initial response to various AEDs with different modes of actions. SIGNIFICANCE: Considering both age-related and spontaneous fluctuation in seizure frequency and the unknown impact of many AEDs or KD on cognition, our data may help defining realistic treatment goals and avoiding overtreatment in patients with CDKL5 mutations. There is a strong need to develop new treatment strategies for patients with this rare mutation.

Valproate treatment normalizes EEG functional connectivity in successfully treated idiopathic generalized epilepsy patients.

AIM: To investigate the effect of chronic VPA treatment of EEG functional connectivity in successfully treated idiopathic generalized epilepsy (IGE) patients. PATIENTS AND METHODS: 19-channel waking, resting-state EEG records of 26 IGE patients were analyzed before treatment (IGE) and after the 90th day of treatment (VPA), in seizure-free condition. Three minutes of artifact-free EEG background activity (without epileptiform potentials) was analyzed for each patient in both conditions. A group of 26 age-matched healthy normative control persons (NC) was analyzed in the same way. All the EEG samples were processed to LORETA (Low Resolution Electromagnetic Tomography) to localize multiple distributed sources of EEG activity. Current source density time series were generated for 33 regions of interest (ROI) in each hemisphere for four frequency bands. Pearson correlation coefficients (R) were computed between all ROIs in each hemisphere, for four bands across the investigated samples. R values corresponded to intrahemispheric, cortico-cortical functional EEG connectivity (EEGfC). Group and condition differences were analyzed by statistical parametric network method. MAIN RESULTS: p<0.05, corrected for multiple comparisons: (1) The untreated IGE group showed increased EEGfC in the delta and theta bands, and decreased EEGfC in the alpha band (as compared to the NC group); (2) VPA treatment normalized EEGfC in the delta, theta and alpha bands; and (3) degree of normalization depended on frequency band and cortical region. CONCLUSIONS: VPA treatment normalizes EEGfC in IGE patients.

Neurophysiology of juvenile myoclonic epilepsy: EEG-based network and graph analysis of the interictal and immediate preictal states.

INTRODUCTION: The neuronal mechanisms of enduring seizure propensity and seizure precipitation in juvenile myoclonic epilepsy (JME) are not known. We investigated these issues, within the framework of the "network concept" of epilepsy. METHODS: Design1: 19, unmedicated JME patients were compared with nineteen, age-, and sex-matched normal control persons (NC). A total of 120s, artifact-free, paroxysm-free, eyes-closed, resting state EEG background activity was analyzed for each person. Design2: interictal and immediate preictal periods of the JME patients were compared in order to explore interictal-preictal network differences. For both comparison designs, statistically significant differences of EEG functional connectivity (EEGfC), nodal and global graph parameters were evaluated. MAIN RESULTS: Design1: maximum abnormalities were: increased delta, theta, alpha1 EEGfC and decreased alpha2 and beta EEGfC in the JME group as compared to the NC group, mainly among cortical areas that are involved in sensory-motor integration. Nodal degree and efficiency of three, medial, basal frontal nodes were greater in JME than in NC, in the alpha1 band. Design2: preictal delta EEGfC showed further increase in the above-mentioned areas, as compared to the interictal state. DISCUSSION: Increased EEGfC indicates a hypercoupled state among the specified cortical areas. This interictal abnormality further increases in the preictal state. Nodal graph statistics indicates abnormal neuronal dynamics in the cortical area that is the ictal onset zone in JME. SIGNIFICANCE: Interictal and preictal neuronal dysfunction has been described in terms of network dynamics and topography in JME patients. Forthcoming investigations of seizure precipitation and therapeutic drug effects are encouraged on this basis.

Clinical and genetic aspects of PCDH19-related epilepsy syndromes and the possible role of PCDH19 mutations in males with autism spectrum disorders.

Epilepsy and mental retardation limited to females (EFMR), caused by PCDH19 mutations, has a variable clinical expression that needs further exploration. Onset of epilepsy may be provoked by fever and can resemble Dravet syndrome. Furthermore, transmitting males have no seizures, but are reported to have rigid personalities suggesting possible autism spectrum disorders (ASD). Therefore, this study aimed to determine the phenotypic spectrum associated with PCDH19 mutations in Dravet-like and EFMR female patients and in males with ASD. We screened 120 females suffering from Dravet-like epilepsy, 136 females with EFMR features and 20 males with ASD. Phenotypes and genotypes of the PCDH19 mutation carriers were compared with those of 125 females with EFMR reported in the literature. We report 15 additional patients with a PCDH19 mutation. Review of clinical data of all reported patients showed that the clinical picture of EFMR is heterogeneous, but epilepsy onset in infancy, fever sensitivity and occurrence of seizures in clusters are key features. Seizures remit in the majority of patients during teenage years. Intellectual disability and behavioural disturbances are common. Fifty percent of all mutations are missense mutations, located in the extracellular domains only. Truncating mutations have been identified in all protein domains. One ASD proband carried one missense mutation predicted to have a deleterious effect, suggesting that ASD in males can be associated with PCDH19 mutations.

EEG-LORETA endophenotypes of the common idiopathic generalized epilepsy syndromes.

OBJECTIVE: We tested the hypothesis that the cortical areas with abnormal local EEG synchronization are dissimilar in the three common idiopathic generalized epilepsy (IGE) phenotypes: IGE patients with absence seizures (ABS), juvenile myoclonic epilepsy (JME) and epilepsy with generalized tonic-clonic seizures exclusively (EGTCS). PATIENTS AND METHODS: Groups of unmedicated ABS, JME and EGTCS patients were investigated. Waking EEG background activity (without any epileptiform potentials) was analyzed by a source localization method, LORETA (Low Resolution Electromagnetic Tomography). Each patient group was compared to a separate, age-matched group of healthy control persons. Voxel-based, normalized broad-band (delta, theta, alpha, and beta) and very narrow band (VNB, 1Hz bandwidth, from 1 to 25Hz) LORETA activity (=current source density, A/m(2)) were computed for each person. Group comparison included subtraction (average patient data minus average control data) and group statistics (multiple t-tests, where Bonferroni-corrected p<0.05 values were accepted as statistically significant). RESULTS: Statistically not significant main findings were: overall increased delta and theta broad band activity in the ABS and JME groups; decrease of alpha and beta activity in the EGTCS group. Statistically significant main findings were as follows. JME group: bilaterally increased theta activity in posterior (temporal, parietal, and occipital) cortical areas; bilaterally increased activity in the medial and basal prefrontal area in the 8Hz VNB; bilaterally decreased activity in the precuneus, posterior cingulate and superior parietal lobule in the 11Hz and 21-22Hz VNBs. ABS group: bilaterally increased theta activity emerged in the basal prefrontal and medial temporal limbic areas. Decreased activity was found at 19-21Hz in the right postcentral gyrus and parts of the right superior and medial temporal gyri. EGTCS group: decreased activity was found in the frontal cortex and the postcentral gyrus at 10-11Hz, increased activity in the right parahippocampal gyrus at 16-18Hz. DISCUSSION: Increased theta activity in the posterior parts of the cortex is the endophenotype for JME. Increased theta activity in the fronto-temporal limbic areas is the endophenotype for ABS. Statistically not significant findings might indicate diffuse biochemical abnormality of the cortex in JME and ABS. SIGNIFICANCE: EEG-LORETA endophenotypes may correspond to the selective propensity to generate absence and myoclonic seizures in the ABS and JME syndromes.

EEG background activity is abnormal in the temporal and inferior parietal cortex in benign rolandic epilepsy of childhood: a LORETA study.

INTRODUCTION: Benign rolandic epilepsy of childhood (BERS) is an epilepsy syndrome with presumably genetic-developmental etiology. The pathological basis of this syndrome is completely unknown. We postulated that a developmental abnormality presumably results in abnormal EEG background activity findings. PATIENTS AND METHODS: 20 children with typical BERS and an age- and sex-matched group of healthy control children underwent EEG recording and analysis. 60×2 s epochs of waking EEG background activity (without epileptiform potentials and artifacts) were analyzed in the 1-25 Hz frequency range, in very narrow bands (VNB, 1 Hz bandwidth). LORETA (Low Resolution Electromagnetic Tomography) localized multiple distributed sources of EEG background activity in the Talairach space. LORETA activity (current source density) was computed for 2394 voxels and 25 VNBs. Normalized LORETA data were processed to voxel-wise comparison between the BERS and control groups. Bonferroni-corrected p<0.05 Student's t-values were accepted as statistically significant. RESULTS: Increased LORETA activity was found in the BERS group (as compared to the controls) in the left and right temporal lobes (fusiform gyri, posterior parts of the superior, middle and inferior temporal gyri) and in the angular gyri in the parietal lobes, in the 4-6 Hz VNBs, mainly at 5 Hz. DISCUSSION: (1) Areas of abnormal LORETA activity exactly correspond to the temporal and parietal cortical areas that are major components of the Mirsky attention model and also the perisylvian speech network. Thus the LORETA findings may correspond to impaired attention and speech in BERS patients. (2) The LORETA findings may contribute to delineating the epileptic network in BERS. SIGNIFICANCE: The novel findings may contribute to investigating neuropsychological disturbances and organization of the epileptic network in BERS.

EEG functional connectivity of the intrahemispheric cortico-cortical network of idiopathic generalized epilepsy.

AIMS: Intrahemispheric, cortico-cortical EEG functional connectivity (fC) was investigated in untreated patients with idiopathic generalized epilepsy (IGE) in this explorative study. PATIENTS AND METHODS: Group comparison was carried out between 19, drug-naive IGE patients and 19, matched healthy persons. 90×2s of 19 channels waking, interictal background EEG signal (without epileptiform potentials) were processed to the LORETA (low resolution electromagnetic tomography) software to compute current source density for 2394 voxels representing parcels of the cerebral cortex for 25 very narrow bands of 1Hz bandwidth (VNBs) from 1 to 25Hz. EEG fC was investigated among the already localized sources. Pearson correlation coefficients (R) were computed among the 33 regions of interest (ROI) within the left and within the right hemisphere, separately. Group differences were computed by means of t-statistics. Corrected p<0.05 differences were accepted as statistically significant. MAIN RESULTS: (1) The anatomical patterns of the fC differences showed great frequency-dependency. (2) Hemispheric asymmetry was prominent within most VNBs. (3) Decreased fC in the IGE group was found across all VNBs in the 1-6Hz frequency range as compared to mixed patterns comprising both increased and decreased fC at >6Hz frequencies. (4) In the 5-25Hz range, decreased fC dominated in the anterior, increased fC in the posterior parts of the cortex. (5) The results delineated an anterior and a posterior network. DISCUSSION: (1) Decreased fC in the 1-6Hz band might indicate some relationship to yet hidden structure network abnormalities. (2) The anatomical patterns of fC indicate frequency-dependent, pathological coupling and decoupling processes in the interictal state. (3) The two networks might help to understand seizure liability and seizure precipitation in IGE. SIGNIFICANCE: This is the first study to explore EEG fC in the interictal condition of IGE patients. The importance of EEG frequencies in evaluating fC in IGE was demonstrated and starting points for further research were given.

Periictal heart rate variability analysis suggests long-term postictal autonomic disturbance in epilepsy.

BACKGROUND: One of the possible pathomechanisms of sudden death in epilepsy (SUDEP) is a postictal dysregulation of autonomic nervous system. We performed a heart rate variability (HRV) analysis of the periictal state to analyze whether a cardiac autonomic disturbance exists after an epileptic seizure. METHODS: We included 31 periictal video-EEG-ECG recordings of 31 patients with epilepsy who had consecutively undergone pre-surgical evaluation. Nine generalized tonic-clonic (GTCS), 15 complex partial, and seven simple motor seizures were included. HRV was evaluated by analyzing 5-min-long ECG epochs, sampling from baseline, direct preictal, early-postictal (<15 min after the seizure), and late-postictal (5-6 h after the seizure) periods. RESULTS: The heart rate was elevated immediately after the seizures, but 5-6 h postictally returned to the baseline level. Time-domain components of HRV decreased after the seizure and this decrease lasted for 5-6 h. Low-frequency power decreased in the early-postictal phase and high-frequency power of HRV dropped in the late-postictal phase. GTCS had an impact on short-term but not on long-term postictal HRV decrease. CONCLUSIONS: We found decreased HRV immediately after the seizures, which lasted at least 5-6 h postictally, indicating a long-term postictal disturbance of the autonomous nervous system. GTCS were accompanied by a more decreased HRV than other seizures. Our results may have relevance in explaining pathomechanism of SUDEP.

Peri-ictal vegetative symptoms in temporal lobe epilepsy.

We investigated peri-ictal vegetative symptoms (PIVS) in 141 patients with adult temporal lobe epilepsy (TLE) and assessed frequency, gender effect, and lateralizing value of peri-ictal autonomic signs. We recorded abdominal auras in 62%, goosebumps in 3%, hypersalivation in 12%, spitting in 1%, cold shivering in 3%, urinary urge in 3%, water drinking in 7%, postictal nose wiping (PNW) in 44%, and postictal coughing in 16%. At least one vegetative sign appeared in 86% of the patients. The presence of PIVS did not have a significant lateralizing value. PNW occurred in 52% of women and in 33% of men, whereas any PIVS was present in 93% of women and 77% of men. In summary, contradictory to previous studies, the presence of PIVS has no lateralizing value, which may be linked to a low frequency of occurrence of PIVS. PIVS, especially PNW, occurred more frequently in women, supporting the gender differences in epilepsy.

Patients' ability to react before complex partial seizures.

OBJECTIVE: The purpose of the study described here was to investigate the pathophysiology of patients' ability to react during the conscious (aura) phase of complex partial seizures (CPS) originating from the temporal lobe. METHODS: We reviewed video recordings of CPS experienced by 130 adult patients who had undergone epilepsy surgery for intractable medial temporal lobe epilepsy. All patients were instructed to push the alarm button when they felt an aura. We defined the preictal reactivity as the ability to push the alarm button before the complex partial (unconscious) phase of seizures. RESULTS: Seventy-seven patients (59%) pushed the alarm button before seizures. Patients with preictal reactivity were significantly younger, more often had lateralized EEG seizure patterns, and had a better postoperative outcome. Patients who did not push the alarm button had secondarily generalized seizures more often. CONCLUSIONS: Ability to react before CPS is associated with a circumscribed region involved at seizure onset and spread, and with a seizure-free postoperative outcome.

Unilateral hand automatisms in temporal lobe epilepsy.

OBJECTIVES: To analyse the lateralising value of unilateral manual automatism (UMA), its relation to contralateral dystonia and the hand by which the UMA was performed. METHODS: In this retrospective study, we reviewed video recordings of 141 patients (mean age 34.1+/-10) who had consecutively undergone presurgical evaluations with ictal video-EEG recordings and high-resolution MRI, had had epilepsy surgery due to intractable medial temporal lobe epilepsy with complex partial seizures due to unilateral medial temporal lobe lesions. The video recordings were prospectively reviewed by one of the authors blinded to patient's clinical data except the diagnosis of medial temporal lobe epilepsy. Altogether 310 archived seizures were analysed. RESULTS: Hand automatisms occurred in 86.5% of patients. UMA occurred in 53% of patients. If UMA was accompanied by contralateral hand dystonia, it had a high lateralising value to the ipsilateral epileptic focus (EF), it was ipsilateral in 85% of patients. Conversely, if UMA occurred without contralateral dystonia, it had only a limited lateralising value because it was ipsilateral to the EF in only 63% of patients. However, we found that left-sided UMA without dystonia had a high lateralising value to the left hemisphere (ipsilateral to the EF in 82%), while right-sided UMA without dystonia has practically no lateralising value. CONCLUSIONS: UMA with contralateral dystonia has a high lateralising value to the ipsilateral hemisphere. Left-sided UMA without contralateral dystonia has a lateralising value to the left hemisphere. Right-sided UMA without contralateral dystonia has no lateralising value.

Electrode manipulation automatism during temporal lobe seizures.

OBJECTIVE: To describe clinical characteristics and lateralizing value of peri-ictal electrode manipulation automatism (EMA) in patients with temporal lobe epilepsy (TLE) and compare our data with ictal manual automatisms described in the literature. METHODS: Two-hundred and five videotaped seizures of 55 consecutive patients with refractory TLE and postoperatively seizure-free outcome were analyzed and EMA (tugging, scratching or adjusting the electrodes and cables) were monitored. RESULTS: Twenty-eight (51%) patients showed EMA during 47 (23%) seizures. Ictal start was noted in 22 seizures and in 19/22 cases EMA finished before the end of seizure. Ictal EMAs were always associated with automotor seizure components. During 25 seizures, exclusively postictal EMAs were observed. Electrode manipulation was presented during 24/112 left-sided and 23/93 right-sided seizures (p = 0.742). Peri-ictal EMA was unilateral (completed by one hand) in 24/47 seizures (10 ictal, 14 postictal); it was done by the hand ipsilateral to the seizure onset zone in 17/24 and by contralateral hand in 7/24 cases (p = 0.064). We observed concomitant contralateral dystonic posturing during 3/10 seizures with unilateral ictal EMA. Unilateral hand automatism, temporally independent from the EMA appeared in 30 (64%) of the 47 seizures. CONCLUSION: Peri-ictal EMA is a frequent phenomenon but shows no lateralizing value in TLE. The mechanism of EMA is in many ways dissimilar from that of earlier described manual automatisms.

Lateralizing value of postictal automatisms in temporal lobe epilepsy.

PURPOSE: To describe clinical characteristics and lateralizing value of postictal automatisms in patients with temporal lobe epilepsy (TLE). METHODS: One hundred and ninety-three videotaped seizures of 55 consecutive patients with refractory TLE and postoperatively seizure-free outcome were analyzed. Ictal as well as postictal (manual, oral and speech) automatisms were monitored. RESULTS: Thirty-four (62%) of the 55 patients showed PA at least once during their seizures. Postictal automatism was observed in 70 (36%) attacks as manual (21%), oral (13%) or speech (9%) automatisms. Fifteen seizures contained a combination of two different postictal automatisms. The presence of postictal oral automatisms did not lateralize the seizure onset zone (p=0.834). Speech automatisms (repetitive verbal behavior) occurred more frequently after left-sided seizures (p=0.002). Postictal unilateral manual automatism showed no lateralizing value occurring by the ipsilateral hand in 10 and the contralateral upper limb in 6 seizures (p=0.454). CONCLUSION: : Postictal automatism is a relatively frequent phenomenon in TLE. Postictal speech automatism lateralizes the seizure onset zone to the left hemisphere. Our observation can help the presurgical evaluation of TLE because verbal perseveration frequently occurs spontaneously, even in seizures without appropriate postictal language testing.

Prognostic factors for surgery of neocortical temporal lobe epilepsy.

OBJECTIVES: In the current classification of epilepsies two forms of temporal lobe epilepsy (TLE) were included: mesial and lateral (neocortical) TLE. We aimed at identifying prognostic factors for the surgical outcome of lesional neocortical TLE. METHODS: We included consecutive patients who had undergone presurgical evaluation including ictal video-EEG and high-resolution MRI, who had TLE due to neocortical lateral epileptogenic lesions, who had a lesionectomy and who had >2-year follow-up. RESULTS: There were 29 patients who met the inclusion criteria. Twenty of them became postoperatively seizure-free. Patients' mean age was 34.8+/-9 years (range 18-52). The age at epilepsy onset was 20.1+/-8 years. We found that left-sided surgery (p=0.048) and focal cortical dysplasia (FCD) on MRI (p=0.005) were associated with non-seizure-free outcome, while lateralized/localized EEG seizure pattern (p=0.032), tumors on the MRI (p=0.013), and a favorable seizure situation at the 6-month postoperative evaluation were associated with 2-year postoperative seizure-freedom (p<0.001). Multivariate analysis indicated that the side of surgery was not an independent predictor. CONCLUSION: More than two-thirds of the patients with neocortical TLE became seizure-free postoperatively. Lateralized/localized EEG seizure pattern and tumors on the MRI were associated with postoperative seizure-freedom, while FCD were associated with a poor outcome. The 6-month postoperative outcome is a reliable predictor for the long-term outcome.

Bilateral epilepsy surgery in a 4-year-old child.

OBJECTIVE: Reports on bilateral epilepsy surgical interventions are anecdotal because of the possible neurological deficits caused by them. METHODS: We report on a four-year-old amaurotic child with catastrophic epilepsy due to bilateral occipital cortical dysplasia. After video-EEG monitoring and intraoperative electrocorticography he underwent a two-step bilateral occipital lobectomy. RESULTS: The first resection resulted in only temporary seizure cessation; however, he became seizure-free after the second operation (follow-up: 20 months). CONCLUSION: Patients with catastrophic epilepsy due to bilateral epileptogenic lesions but without a high risk of additional postsurgical deficit may be good candidates for epilepsy surgery.

Clinical differences in patients with unilateral hippocampal sclerosis and unitemporal or bitemporal epileptiform discharges.

PURPOSE: To investigate factors determining the presence of bilateral interictal epileptiform discharges (IEDs) in temporal lobe epilepsy (TLE) with unilateral hippocampal sclerosis (HS). METHODS: We analysed data of 243 TLE patients with unilateral HS who had long-term video-EEG. Eighty-one patients (33%) had bitemporal IEDs. RESULTS: We categorised patients into a unilateral group (UG), a bilateral group (BG) according to presence of bitemporal IEDs. We found no difference between UG and BG regarding epilepsy duration, secondarily generalised seizures, and history of febrile seizures. Mean seizure frequency was significantly higher in the BG (UG: 7.7+/-14.7 seizures/month; BG: 13+/-35 seizures/month, P=0.01). We found a significant correlation between late epilepsy onset and the presence of bitemporal IEDs. The mean age at epilepsy onset in UG was 10.1+/-7.9 years, while in BG it was 13+/-9.2 years (P=0.02). CONCLUSIONS: The traditional concept of the evolution of mirror focus cannot apply for humans because the duration of epilepsy does not influence the evolution of bitemporal IEDs. Other factors, i.e. age at onset and seizure frequency may play a role in this process. The association between the higher seizure frequency and mirror foci indicates that the development of mirror focus depends on seizures and not on a progressive 'interictal' epileptogenesis.

Spatiotemporal relationship between seizure activity and interictal spikes in temporal lobe epilepsy.

PURPOSE: To analyze the spatio-temporal relationship between seizure propagation and interictal epileptiform discharges (IEDs) in patients with bitemporal epilepsy. METHODS: We investigated 18 adult patients with intractable temporal lobe epilepsy (TLE) who had undergone continuous video-EEG monitoring during presurgical evaluation. Only those patients were selected who had independent IEDs over both temporal lobes. Two authors evaluated the ictal and interictal EEG data independently. RESULTS: We analyzed 52 lateralized seizures of 18 patients. Thirty-one seizures showed ipsilateral seizure spread exclusively, whereas in 21 seizures the contralateral hemisphere was also involved. In lateralized seizures without contralateral propagation, we found that spikes ipsilateral to the seizure onset occurred postictally in a greater ratio than preictally (P<0.001). In lateralized seizures with contralateral propagation, we found no significant changes in the postictal spike distribution. CONCLUSIONS: Our findings showed that the lateralization of IEDs may depend on the brain areas involved by the preceding seizures, suggesting that spikes can be influenced by the seizure activity, and are not independent signs of epileptogenicity.

[Surgically treatable epilepsy--a review]. / Mútéttel gyógyítható epilepszia--összefoglaló tanulmány.

20-25% of epileptic patients do not become seizure free on adequate drug therapy. In 25-50% of patients with intractable epilepsy, the brain area responsible for seizures is well localizable and does not involve eloquent regions. In these patients, the surgical excision of the epileptic focus may lead to relief from seizures. In Hungary, there may be 5-6000 patients who needs an epilepsy surgery, but till now only 200 patients with chronic epilepsy underwent a surgical procedure. In the surgically remediable epilepsies, the operation is not a "ultima ratio". Concerning these syndromes, if 2-3 adequate antiepileptic drugs do not lead to seizure freedom within 1-3 years after the epilepsy onset, then a presurgical evaluation is necessary. The most common surgically remediable epilepsy is the temporal lobe epilepsy in which 60-90% of drug-resistant patients could be surgically cured. In lesional neocortical epilepsies 50-80% of patients become postoperatively seizure free. In childhood hemispheric epilepsies, the surgery could lead to seizure freedom in 70-80% of patients. The basic tools of the presurgical evaluation are the detailed history, the high resolution-MRI, the video-EEG monitoring, and the neuropsychological assessment. These investigation methods are usually enough to evaluate the necessity of the surgery and the postoperative outcome as well as to plan the localization and the extension of the resection. In some cases, ictal SPECT, PET, or video-EEG monitoring with intracranial electrodes could also be necessary in order to localize the epileptic focus.

A detailed analysis of frontal lobe seizure semiology in children younger than 7 years.

PURPOSE: We sought to analyze semiology of seizure onset and evolution in young children with frontal lobe epilepsy (FLE), compare this with adult reports, and assess age-related differences. METHODS: We analyzed 111 videotaped seizures from 14 patients with FLE based on focal cortical dysplasia aged 3-81 months (mean, 30 months). Ictal events were categorized into behavioral, consciousness, autonomic, and sensory features, as well as motor patterns, which included tonic, clonic, epileptic spasm, and myoclonic seizure components. We developed a time-scaled datasheet to record each epileptic event as onset, very early, early, or late manifestation. RESULTS: Patients had a high seizure frequency with up to 40 attacks/day; half of them showed a cluster tendency. Forty-seven percent of the seizures started in sleep. Mean duration of seizures was short (29 s). Most common seizure components were motor manifestations, mostly tonic-clonic seizures, and epileptic spasms. Behavioral change was frequent, and hypermotor seizures were not seen. In five patients, the motor features were contralateral to the epileptic focus, including two children with asymmetric epileptic spasms. Secondarily generalized tonic-clonic seizures (SGTCSs) were not recorded, but had been reported in the history of two patients. Complex motor automatisms were not seen, whereas oral automatism appeared in three children. CONCLUSIONS: Motor features are common in young children with FLE, as reported in adults. The characteristics, however, differ. Epileptic spasms and subtle behavioral change were frequent. Hypermotor seizures and complex motor automatisms were not seen, and SGTCSs were unusual. Our results suggest that FLE in young children shows age-related features differing from those of adults.

Lateralizing value of unilateral motor and somatosensory manifestations in frontal lobe seizures.

PURPOSE: To evaluate the lateralizing value of unilateral somatosensory aura, unilateral tonic posturing, head version, non-forced head turning, ictal cloni, dystonic posturing, and postictal nose wiping in seizures originating in the frontal lobe. METHODS: We included patients who had consecutively undergone presurgical evaluation with ictal video-EEG monitoring at our institution, had had resective epilepsy surgery involving the frontal lobe, and had remained seizure-free >1 year after operation. Twenty-seven patients aged 1-42 years (mean 18) met the inclusion criteria. Fifteen patients had right-sided, 12 patients had left-sided epileptogenic regions. Seizures recorded during EEG-video monitoring were re-evaluated by two investigators in order to identify lateralization signs in frontal lobe seizures. One of the investigators was blind to patients' clinical data. RESULTS: We analyzed 153 seizures of 27 patients. The most common unilateral phenomenon was the unilateral tonic posturing occurring in 48% of all the patients and in 25% of all seizures. Somatosensory aura and head version appeared exclusively contralateral whereas clonus occurred in 92% and unilateral tonic posturing in 89% of seizures contralateral to the epileptogenic region. Ictal non-forced head turning and postictal nose wiping showed no lateralizing significance. Dystonic posturing did not occur. CONCLUSIONS: Somatosensory aura, head version, ictal cloni, and tonic posturing are reliable lateralizing signs in frontal seizures. These signs may help in identifying the epileptogenic region during presurgical evaluation of patients suffering from frontal lobe epilepsy.