Metabolic profiles and correlation with surgical outcomes in mesial versus neocortical temporal lobe epilepsy

Aims

Differentiating mesial temporal lobe epilepsy (MTLE) and neocortical temporal lobe epilepsy (NTLE) remains challenging. Our study characterized the metabolic profiles between MTLE and NTLE and their correlation with surgical prognosis using ¹⁸F-FDG-PET.

Methods

A total of 137 patients with intractable temporal lobe epilepsy (TLE) and 40 age-matched healthy controls were recruited. Patients were divided into the MTLE group (N = 91) and the NTLE group (N = 46). ¹⁸F-FDG-PET was used to measure the metabolism of regional cerebra, which was analyzed using statistical parametric mapping. The volume of abnormal metabolism in cerebral regions and their relationship with surgical prognosis were calculated for each surgical patient.

Results

The cerebral hypometabolism of MTLE was limited to the ipsilateral temporal and insular lobes (p < 0.001, uncorrected). The NTLE patients showed hypometabolism in the ipsilateral temporal, frontal, and parietal lobes (p < 0.001, uncorrected). The MTLE patients showed extensive hypermetabolism in cerebral regions (p < 0.001, uncorrected). Hypermetabolism in NTLE was limited to the contralateral temporal lobe and cerebellum, ipsilateral frontal lobe, occipital lobe, and bilateral thalamus (p < 0.001, uncorrected). Among patients who underwent resection of epileptic lesions, 51 (67.1%) patients in the MTLE group and 10 (43.5%) in the NTLE group achieved Engel class IA outcome (p = 0.041). The volumes of metabolic increase for the frontal lobe or thalamus in the MTLE group were larger in non-Engel class IA patients than Engel class IA patients (p < 0.05).

Conclusions

The spatial metabolic profile discriminated NTLE from MTLE. Hypermetabolism of the thalamus and frontal lobe in MTLE may facilitate preoperative counseling and surgical planning.     

Association of ipsilateral motor automatisms and contralateral dystonic posturing: a clinical feature differentiating medial from neocortical temporal lobe epilepsy.

BACKGROUND: Clinical features that may help to differentiate medial temporal lobe epilepsy (MTLE) from neocortical temporal lobe epilepsy (NTLE) are lacking. OBJECTIVE: To investigate the localizing and lateralizing value of the association of ipsilateral motor automatisms and contralateral dystonic posturing in patients with medically refractory temporal lobe epilepsy. PATIENTS AND METHODS: Videotapes of 60 patients with well-defined MTLE, NTLE, or both were reviewed to assess the presence and the localizing value of unilateral dystonic posturing associated with motor automatisms. RESULTS: Twenty-eight of the 60 patients exhibited unilateral dystonic posturing. This sign was observed in patients with MTLE and NTLE. It was mostly contralateral to the seizure focus in patients with MTLE and exclusively ipsilateral in patients with NTLE. Unilateral motor automatisms occurred in 26 of the 60 patients with MTLE or NTLE. It was predominantly ipsilateral to the seizure focus in patients with MTLE and exclusively contralateral in patients with NTLE. The association of ipsilateral motor automatisms and contralateral dystonic posturing was found in 14 patients with MTLE but in none of the patients with NTLE. Two patients who had medial and neocortical seizure onset also exhibited this clinical feature. This association was not significantly correlated with the postoperative outcome in patients with MTLE. CONCLUSIONS: The association of ipsilateral motor automatisms and contralateral dystonic posturing may help to differentiate MTLE from NTLE with a reliable lateralizing value. This clinical association may reflect a specific pattern in the spread of the ictal discharge.     

The association of magnetoencephalography high-frequency oscillations with epilepsy types and a ripple-based method with source-level connectivity for mapping epilepsy sources

Objective

To explore the association between high-frequency oscillations (HFOs) and epilepsy types and to improve the accuracy of source localization.

Methods

Magnetoencephalography (MEG) ripples of 63 drug-resistant epilepsy patients were detected. Ripple rates, distribution, spatial complexity, and the clustering coefficient of ripple channels were used for the preliminary classification of lateral temporal lobe epilepsy (LTLE), mesial temporal lobe epilepsy (MTLE), and nontemporal lobe epilepsy (NTLE), mainly frontal lobe epilepsy (FLE). Furthermore, the seizure site identification was improved using the Tucker LCMV method and source-level betweenness centrality.

Results

Ripple rates were significantly higher in MTLE than in LTLE and NTLE (p < 0.05). The LTLE and MTLE were mainly distributed in the temporal lobe, followed by the parietal lobe, occipital lobe, and frontal lobe, whereas MTLE ripples were mainly distributed in the frontal lobe, then parietal lobe and occipital lobe. Nevertheless, the NTLE ripples were primarily in the frontal lobe and partially in the occipital lobe (p < 0.05). Meanwhile, the spatial complexity of NTLE was significantly higher than that of LTLE and MTLE and was lowest in MTLE (p < 0.01). However, an opposite trend was observed for the standardized clustering coefficient compared with spatial complexity (p < 0.01). Finally, the tucker algorithm showed a higher percentage of ripples at the surgical site when the betweenness centrality was added (p < 0.01).

Conclusion

This study demonstrated that HFO rates, distribution, spatial complexity, and clustering coefficient of ripple channels varied considerably among the three epilepsy types. Additionally, tucker MEG estimation combined with ripple rates based on the source-level functional connectivity is a promising approach for presurgical epilepsy evaluation.     

Panayiotopoulos syndrome and symptomatic occipital lobe epilepsy of childhood: a clinical and EEG study

Aim. Panayiotopoulos syndrome (PS) is an age‐related seizure susceptibility syndrome that affects the central autonomic system. Although the majority of the few ictal recordings obtained so far suggest an occipital origin, semiological and interictal EEG data appear to favour more extensive involvement. In this study, the characteristics (including those based on semiology and EEG) of children with Panayiotopoulos syndrome (n=24) and those with lesion‐related, symptomatic occipital lobe epilepsy (SOLE) (n=23) were compared. Methods. Detailed semiological information and EEG parameters including the localisation, distribution, density (n/sec), reactivity, and morphological characteristics of spike‐wave foci and their relationship with different states of vigilance were compared between the two groups. Results. The age at seizure onset was significantly younger in patients with symptomatic occipital lobe epilepsy than in those with PS (mean age at onset: 3.4 versus 5.6 years, respectively; p=0.044). Autonomic seizures (p=0.001) and ictal syncope (p=0.055) were more frequent in PS than in symptomatic occipital lobe epilepsy (87.5% and 37.5% versus 43.5% and 13%, respectively). The interictal spike‐wave activity increased significantly during non‐rapid eye movement (non‐REM) sleep in both groups. The spike waves in non‐REM seen in PS tended to spread mainly to central and centro‐temporal regions. Conclusions. The results indicate that although common features do exist, Panayiotopoulos syndrome differs from symptomatic occipital lobe epilepsy and has a unique low epileptogenic threshold related to particular brain circuits.     

Interictal regional delta slowing is an EEG marker of epileptic network in temporal lobe epilepsy

Purpose: Several studies have suggested that interictal regional delta slowing (IRDS) carries a lateralizing and localizing value similar to interictal spikes and is associated with favorable surgical outcomes in patients with temporal lobe epilepsy (TLE). However, whether IRDS reflects structural dysfunction or underlying epileptic activity remains controversial. The objective of this study is to determine the cortical electroencephalography (EEG) correlates of scalp‐recorded IRDS, in so doing, to further understand its clinical and biologic significances. Methods: We examined the cortical EEG substrates of IRDS with electrocorticography (ECoG‐IRDS) and delineated the spatiotemporal relationship between ECoG‐IRDS and both interictal and ictal discharges by recording simultaneously scalp and intracranial EEG in 18 presurgical candidates with TLE. Key Findings: Our results demonstrated that ECoG‐IRDS is typically a mixture of delta/theta slowing and spike‐wave potentials. ECoG‐IRDS was predominantly recorded from basal and anterolateral temporal cortex, occasionally in mesial, posterior temporal, and extratemporal regions. Abundant IRDS was most commonly observed in patients with neocortical temporal lobe epilepsy (NTLE), whereas infrequent to moderate IRDS was usually observed in patients with mesial temporal lobe epilepsy (MTLE). The anatomic distribution of ECoG‐IRDS was highly correlated with the irritative and seizure‐onset zones in 10 patients with NTLE. However, it was poorly correlated with the irritative and seizure‐onset zones in the 8 patients with MTLE. Significance: These findings demonstrate that IRDS is an EEG marker of epileptic network in patients with TLE. Although IRDS and interictal/ictal discharges likely arise from the same neocortical generator in patients with NTLE, IRDS in patients with MTLE may reflect a network disease that involves temporal neocortex.     

Medial Temporal Objective Lobe Epilepsy: Videotape Analysis of Clinical Seizure Characteristics

Summary: Purpose: The syndrome of temporal lobe epilepsy has been described in great detail. Here we focus specifically on the clinical manifestations of seizures originating in the hippocampus and surrounding mesial temporal structures.
Methods: Seizure origin was confirmed in 67 cases by depth EEG recording and surgical cure after mesial temporal resection.
Results: Among nonlateralized manifestations, we commonly found oral automatisms, pupillary dilatation, impaired consciousness, and generalized rigidity. Appendicular automatisms were often ipsilateral to the seizure focus, whereas dystonia and postictal hemiparesis were usually contralateral. Head deviation, when it occurred early in the seizure, was an ipsilateral finding, but was contralateral to the seizure focus when it occurred late. Clear Ictal speech and quick recovery were found when seizures originated in the non-language-dominant hemisphere, but postictal aphasia and prolonged recovery time were characteristic of seizure origin in the language-dominant hemisphere.
Conclusions: These signs help to define the mesial temporal lobe epilepsy (MTLE) syndrome and often provide information as to the side of seizure origin.     

Clinical features of neocoritcal temporal lobe epilepsy

Few Studies have examined the clinical features of neocortical temporal lobe epilepsy (NTLE) in carefully selected patients. We reviewed records from 21 patients wtih NTLE, defined by intracranial electroencephal9ogram (EEG), who have been seizure free for 1 year or more following temporal lobectomy. The mean age of onset at the time of first seizure was 14 years (range, 1–41 years). Febrile seizures were reported in only 2 patients (9.5%). In contrast to prior mesial temporal lobe epilepsy (MTLE) studies, seizure-free intervals between the initial cerebral insult or first seizure and habitual seizures were uncommon. Possible or known risk factors for epilepsy were reported in 13 of 21 patients (62%). Fifteen (71%) patients reproted auras, with experiential phenomena being the most common type. Magnetic resonance imaging was normal or nospecific in 15 patients, revealed mild hippocampal atrophy in 2, tumors in 2, and resonance imaging was normal or nospecifc in 15 patients, revealed mild hippocampal atrophy in 2, tumors in 2, and heterotopic gray matter and hippocampal atrophy in 1, and cortical dysgensis in 1. Neuropsychological testing showed deficits consistent with the seizure focus in 13 patients (62%), and Wada test showed ipsilateral memory deficits in 10 (48%). The most common behavioral manifestatin was a motionless stare at ictal onset (48%). In contrast to prior studies of MTLE, only 1 NTLE patient had frequent independent, contralateral temporal lobe epileptiform spikes on scalp EEG.     

Complex partial seizures of frontal lobe onset statistical analysis of ictal semiology.

OBJECTIVE: To identify the ictal semiology of complex partial seizures originating from the frontal lobe (FLCPS) and mesial temporal lobe (MTLE) in patients who became seizure free after surgery. METHODS: We analysed 149 seizures from 42 patients, 28 with MTLE (75 seizures) and 14 with FLCPS (74 seizures) seizure free for at least 1 year after surgery. Fifty-eight symptoms and signs were looked for in every seizure and their time of onset and ending noted. Statistical analysis was then used to define the frequency, time of onset and cluster analysis of these symptoms/signs. RESULTS: Epigastric aura was more frequent in MTLE while an aura of a general body sensation or indescribable feeling occurred only in FLCPS. Alimentary automatisms were more common and occurred earlier in MTLE (P<0.001). Perseverative automatisms, retching and vomiting occurred exclusively in MTLE while bicycling movements occurred only in FLCPS. Abdominal, psychic or olfactory aura followed by behavioural arrest, alimentary automatisms, repetitive distal upper extremity movements, complete loss of consciousness, looking around and whole body movements were typical of MTLE. Repetitive coarse upper extremity movements, complete loss of consciousness, complex motor and hypermotor activity were typical of FLCPS. CONCLUSION: The earliest symptoms and signs as well as their order of appearance allow one to distinguish between complex partial seizures arising from the frontal lobe and mesial temporal lobe.     

Semiology of hyperkinetic seizures of frontal versus temporal lobe origin

Aims. Hyperkinetic seizures are usually associated with frontal lobe epilepsy. However, some patients have hyperkinetic seizures of temporal lobe origin. The semiological differences in hyperkinetic seizures between frontal and temporal lobe epilepsy have not been well studied. Here, we retrospectively assessed ictal semiology in order to distinguish between hyperkinetic seizures of frontal lobe origin and those of temporal lobe origin. Methods. We retrospectively reviewed data on patients who had undergone surgery for hyperkinetic seizures of temporal or frontal lobe origin and achieved favourable seizure outcomes (Engel Class I) with a minimum postoperative follow‐up of 24 months. We reviewed seizure histories, imaging reports, video‐EEG monitoring data, operative records, and pathological findings. We analysed and compared the hyperkinetic semiology of video‐recorded seizures of temporal lobe origin and those of frontal lobe origin. Results. Forty hyperkinetic seizures in eight patients (seven adult patients and one 12‐year‐old patient) with temporal lobe epilepsy and 45 hyperkinetic seizures in nine patients (eight adult patients and one 16‐year‐old patient) with frontal lobe epilepsy were analysed. Emotional facial expressions (such as fear, laughing, or anger), bilateral forceful elbow flexion, bilateral forceful grasping, facial flushing, and bilateral facial contraction were observed significantly more frequently in seizures of frontal lobe origin. Oroalimentary automatisms, seizures during wakefulness, salivation, and bilateral drop of the corners of the mouth were observed significantly more frequently in seizures of temporal lobe origin. Conclusions. Observation of a number of signs during hyperkinetic manifestations may help to predict whether a seizure originates from the frontal lobe or the temporal lobe.     

Neocortical temporal lobe epilepsy: intracranial EEG features and surgical outcome.

Patients with neocortical temporal lobe epilepsy (NTLE) may have less favorable outcome with anterior temporal lobectomy than those with mesial temporal foci. The authors analyzed ictal intracranial electroencephalograms (EEGs) in patients with NTLE to identify features that predict surgical outcome. The following intracranial ictal EEG features in 31 consecutive medically intractable NTLE patients were studied: Frequency (i.e., low-voltage fast [>20 Hz], recruiting ictal-onset spikes, ictal-onset rhythms less than 5 Hz, ictal-onset rhythms with repetitive sharp waves between 5 and 20 Hz); extent of ictal onset (focal, sublobar, and lobar); localization within the temporal lobe (anterior, posterior, or regional); and the time to seizure spread outside the temporal lobe (rapid, intermediate, and slow). The average follow-up period was 36.7 months (range, 18 to 60 months). Findings between two outcome groups were compared: class I group (seizure-free) and class II to IV group (persistent seizures). Twenty-one (66.7%) of 31 patients with NTLE were seizure-free. Intracranial EEG features which were significantly associated with seizure-free outcome were focal or sublobar onset, anterior temporal onset, and slow propagation time (P < 0.05). There was a trend for patients with ictal onset morphologies of slow ictal-onset rhythm and repetitive sharp waves to be seizure-free (P = 0.07). Intracranial EEG is helpful in predicting surgical outcome in NTLE patients.     

Monoamine neurotransmitters in resected hippocampal subparcellations from neocortical and mesial temporal lobe epilepsy patients: in situ microvoltammetric studies

It is known that epilepsy patients diagnosed with neocortical temporal lobe epilepsy (NTLE), differ from those diagnosed with mesial temporal lobe epilepsy (MTLE), e.g., in hippocampal (HPC) pathology. In the present studies, we tested the hypothesis that NTLE and MTLE subtypes of human epilepsy might differ in regards to their HPC monoamine neurochemistry. Monoamine neurotransmitters were studied in separate signals and within s with semiderivative microvoltammetry, used in combination with stearate indicator, Ag-AgCl reference and stainless steel auxiliary microelectrodes. Anterior HPC specimens from the patients' epileptogenic zone, defined by electrocorticography, were resected neurosurgically from 13 consecutive patients with intractable temporal lobe epilepsy. Four patients were diagnosed with NTLE and nine with MTLE. The criteria for the diagnosis of NTLE versus MTLE was absence versus presence of HPC sclerosis, respectively, based on MRI examination of resected tissue. In addition, NTLE patients demonstrated seizure onset in anterolateral temporal neocortex on electroencephalography (EEG). HPC subparcellations studied were: (a) Granular Cells of the Dentate Gyrus (DG), (b) Polymorphic Layer of DG and (c) Pyramidal Layer: subfields, CA1 and CA2. Dopamine (DA), serotonin (5-HT), norepinephrine (NE) and ascorbic acid (AA) (co-factor in DA to NE synthesis), exhibited separate and characteristic half-wave potentials in millivolts. Each half-wave potential, i.e., the potential at which maximum current was generated, was experimentally established in vitro. Concentrations of neurotransmitters found in HPC subparcellations were interpolated from calibration curves derived in vitro from electrochemical detection of monoamines and AA in saline phosphate buffer. Significant differences between subtypes in concentration of monoamines were analyzed by the Mann Whitney rank sum test and those differences in probability distribution of monoamines were analyzed by the Fisher Exact test; in each case, P<0.01 was the criteria selected for determining statistical significance. DA concentrations were higher in NTLE compared with MTLE in each HPC subparcellation [P=0.037, 0.024 and 0.007, respectively (P<0.01)] and DA occurred more frequently in NTLE in the Pyramidal Layer [P=0.077 (P<0.01)]. AA was present in one NTLE patient. NE concentrations were higher in MTLE vs. NTLE in each subparcellation [P=0.012, 0.067 and 0.07, respectively (P<0.01)] and NE occurred more frequently in MTLE in Granular Cells of DG and Pyramidal Layer [P=0.052 and 0.014, respectively (P<0.01)]. In MTLE, NE concentrations in the CA1 subfield of the Pyramidal Layer were decreased vs. the CA2 subfield [P=0.063 (P<0.01)]. Serotonin was found in every HPC subparcellation of each subtype but 5-HT concentrations were higher in NTLE vs. MTLE in the Granular Cells of DG and the Pyramidal Layer (CA1 subfield) [P=0.076 and 0.095, respectively (P<0.01)]. Thus, this preliminary study showed that marked differences in HPC monoamine neurochemistry occurred in NTLE patients as compared with MTLE patients.     

Bilateral mesial temporal lobe epilepsy: comparison of scalp EEG and hippocampal MRI-T2 relaxometry

OBJECTIVE: Bilateral hippocampal abnormality is frequent in mesial temporal lobe sclerosis and might affect outcome in epilepsy surgery. The objective of this study was to compare the lateralization of interictal and ictal scalp EEG with MRI T2 relaxometry. MATERIAL AND METHODS: Forty-nine consecutive patients with intractable mesial temporal lobe epilepsy (MTLE) were studied with scalp EEG/video monitoring and MRI T2 relaxometry. RESULTS: Bilateral prolongation of hippocampal T2 time was significantly associated with following bitemporal scalp EEG changes: (i) in ictal EEG left and right temporal EEG seizure onsets in different seizures, or, after regionalized EEG onset, evolution of an independent ictal EEG over the contralateral temporal lobe (left and right temporal asynchronous frequencies or lateralization switch; P = 0.002); (ii) in interictal EEG both left and right temporal interictal slowing (P = 0.007). Bitemporal T2 changes were not, however, associated with bitemporal interictal epileptiform discharges (IED). Lateralization of bilateral asymmetric or unilateral abnormal T2 findings were associated with initial regionalization of the ictal EEG in all but one patient (P < 0.005), with lateralization of IED in all patients (P < 0.005), and with scalp EEG slowing in 28 (82,4%) of 34 patients (P = 0.007). CONCLUSION: Our data suggest that EEG seizure propagation is more closely related to hippocampal T2 abnormalities than IED. Interictal and ictal scalp EEG, including the recognition of ictal propagation patterns, and MRI T2 relaxometry can help to identify patients with bitemporal damage in MTLE. Further studies are needed to estimate the impact of bilateral EEG and MRI abnormal findings on the surgical outcome.     

Distribution of regional gray matter abnormalities in a pediatric population with temporal lobe epilepsy and correlation with neuropsychological performance

ObjectiveThe goals of the work described here were to determine if hippocampal and extrahippocampal atrophy in children with temporal lobe epilepsy (TLE) follows a pattern similar to that in adult patients, and to assess the clinical and neuropsychological relevance of regional brain atrophy in pediatric TLE.MethodsChildren with symptomatic TLE (n = 14: 9 with mesial TLE due to hippocampal atrophy and 5 with TLE due to neocortical lesions), healthy children (n = 14), and 9 adults with mesial temporal lobe epilepsy (MTLE) were compared using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI). The children underwent a comprehensive neuropsychological battery.ResultsChildren with MTLE with unilateral hippocampal atrophy (n = 9) exhibited a significant reduction in gray matter in the hippocampus ipsilateral to the seizure origin and significant atrophy in the ipsilateral cingulate gyrus and contralateral middle frontal lobe. Children with TLE (n = 14) exhibited a significant reduction in the gray matter of the ipsilateral hippocampus and parahippocampal gyrus. There was a correlation between gray matter volume in children with TLE and scores on several neuropsychological tests. Atrophy in pediatric patients with MTLE was less extensive than that in adults, and involved the hippocampi and the frontal cortex.ConclusionsSimilar to adult MTLE, pediatric MTLE is associated with hippocampal and extrahippocampal cell loss. However, children display less intense quantifiable gray matter atrophy, which affects predominantly frontal lobe areas. There was a significant association between volume of gray matter in medial temporal and frontal regions and scores on neuropsychological tests. In childhood, TLE and the concomitant cognitive/behavior disturbances are the result of a damaged neural network.     

颞叶内侧癫痫的两种术式对发作控制的对比分析

目的观察前颞叶切除术和选择性海马、杏仁核切除术对颞叶内侧癫痫的发作控制效果是否有差别。方法 2009年1月至2010年12月在我科行前颞叶切除术的67例颞叶内侧患者为A组;2011年6月至2013年5月在我科行选择性海马、杏仁核切除术的46例颞叶内侧患者为B组;统计分析两组术后1年发作控制为Engel I-II级和Engel III-IV级的人数。结果 A组Engel I-II级56例(83.58%),Engel III-IV级11例(16.42%);B组Engel I-II级40例(86.95%),Engel III-IV级6例(13.05%)。经χ2检验两组术后对MTLE发作的控制率无统计学差异,χ2=0.243,P0.05。结论前颞叶切除术和选择性海马、杏仁核切除术对颞叶内侧癫痫发作都能获得良好的控制,两者疗效无明显差异。     

Outcome after cortico-amygdalo-hippocampectomy in patients with temporal lobe epilepsy and normal MRI

RationaleWe describe seizure and neuropsychological outcome obtained after CAH in patients with TLE and normal MRI evaluated in the modern imaging era.MethodsForty-five adult consecutive patients with TLE and normal MRI were studied. All patients had neuropsychological testing, interictal and ictal EEG recordings and MRI. They were divided into two groups: Group 1 (n = 18), included patients in whom non-invasive neurophysiological evaluation was lateralizing and Group 2 (n = 27) included patients with non-lateralizing neurophysiological data who were submitted to invasive recordings.ResultsSeventy-seven percent of the Group 1 patients were rated as Engel I; 11% were rated as Engel II and 11% as Engel III. In Group 2, there were 57% of patients seizure-free, 26% in Engel II and 14% in Engel III. Pre-operatively, mean general IQ was 82 and 78 in Groups1 and 2, respectively; post-operatively, mean general IQ was respectively 86 and 71. Some degree of verbal memory decline was noted in all patients submitted to dominant temporal lobe resection in both Groups 1 and 2. At last follow-up visit, 22% of Group 1 and 11% of Group 2 patients were receiving no antiepileptic drugs (AED).ConclusionsOur data showed that patients with TLE and normal MRI could get good surgical results after CAH although 60% of them would need invasive recordings and their results regarding seizure control and cognition were worse than those obtained in patients with MRI defined temporal lobe lesions. Caution should be taken in offering dominant temporal lobe resection to this subset of patients.     

Hyperkinetic seizures in patients with temporal lobe epilepsy: clinical features and outcome after temporal lobe resection

Purpose: Temporal lobe epilepsy (TLE) is usually associated with automatisms. Hyperkinetic seizures are supposed to be unusual. Because we witnessed several patients with TLE and ictal hyperkinetic symptoms, we retrospectively assessed the number, clinical findings, and seizure outcome in such patients who had undergone temporal lobe resection. Methods: We reviewed medical history, video–electroencephalography (EEG) recording and neuroimaging of adult patients who underwent epilepsy surgery for TLE at the Kork Epilepsy Center over the last 20 years with a minimum postoperative follow‐up of 12 months. Key Findings: Among 294 patients who were resected exclusively in the temporal region, we identified 17 (6%) who presented with hyperkinetic semiology such as violent vocalization, complex movements of the proximal segments of the limbs, rotation of the trunk, pelvic thrusting, or early tonic or dystonic posturing. Most of the patients had a preceding aura. Ictal EEG activity was located in the corresponding temporal region, usually with a wide distribution over temporal electrodes with fast spread to unilateral frontal electrodes and to the contralateral side. Neuroimaging revealed extended lesions in the temporal lobe involving mesial and neocortical structures. Most of the patients underwent classical anterior temporal lobe resection including amygdalo‐hippocampectomy. Fourteen patients (82%) became completely seizure‐free (Engel class Ia). Histopathology showed mainly focal cortical dysplasia plus hippocampal sclerosis. Significance: Hyperkinetic seizure semiology may occasionally occur in patients with TLE and is, therefore, no contradiction to the hypothesis of TLE if scalp EEG patterns and neuroimaging findings correspond. The postoperative seizure outcome is favorable in such patients and not different from outcome data in classical TLE.     

Mimetic automatisms expressing a negative affect in two patients with temporal lobe epilepsy

Ictal automatisms of fear or sadness, of which the patient is unaware and which are not preceded by a corresponding emotion, have not been well characterized. Of 557 patients admitted for video/EEG monitoring, 2 (0.36%) were identified who had automatisms of fear and sadness. One patient was found to have a sudden ictal expression of sadness of which he was not aware. The second patient showed a sudden fearful expression, followed by oral automatisms, staring, and amnesia for the event. Both patients had left mesial temporal lobe epilepsy. The patient with ictal fear underwent further invasive monitoring and became seizure free after a limited mesial temporal resection. The mesial temporal structures not only mediate emotional experiences, but can also activate stereotyped expressions of fear or sadness without the patient's awareness, arguing for an efferent pathway for expressing negative affects within the mesial temporal lobe.     

Seizure outcome and hippocampal atrophy in familial mesial temporal lobe epilepsy

OBJECTIVE: To describe the clinical, genetic and MR characteristics of patients with familial mesial temporal lobe epilepsy (MTLE). DESIGN/METHODS: The familial occurrence of MTLE was identified by a systematic search of family history of seizures in patients followed in the authors' epilepsy clinic. All probands and, whenever possible, other affected family members underwent EEG and MR investigations. RESULTS: Twenty-two unrelated families with at least two individuals with MTLE were identified by clinical and EEG findings. Ninety-eight individuals with history of seizures were evaluated. Sixty-eight patients fulfilled the diagnostic criteria for MTLE. MRI was performed in 84 patients, and showed hippocampal atrophy with increased T2 signal in 48 (57%). The distribution of hippocampal atrophy according to the seizure outcome groups was 6 of 13 patients (46%) with seizure remission, 16 of 31 (51%) with good seizure control under medication, and all 16 patients with refractory MTLE. Hippocampal atrophy was found also in patients that did not fulfill the criteria for MTLE: 3 of 10 (30%) patients with febrile seizure alone, 6 of 10 (60%) patients with recurrent generalized tonic-clonic seizures, and 1 of 4 (25%) patients with a single partial seizure. CONCLUSION: Familial MTLE is a clinically heterogeneous syndrome. Hippocampal atrophy was observed in 57% of patients, including those with benign course or seizure remission, indicating that the relationship between hippocampal atrophy and severity of epilepsy might be more complex than previously suspected. In addition, these findings indicate the presence of a strong genetic component determining the development of mesial temporal sclerosis in these families.     

Analysis of the initial ictal phenomenon in patients with temporal lobe epilepsy

We aimed to assess the localizing value of the initial semiological element in temporal lobe epilepsy (TLE). Video-EEG-documented seizures of 97 adult TLE patients were studied in relation to seizure origin (left versus right; mesial versus extra-mesial). Strikingly, seizures with mesial onset started with very few ictal phenomena, while seizures of extra-mesial origin began with a larger variety of ictal elements. Furthermore, following noticeable distributions were observed for the mesial group: (i) aura was the most common initial ictal phenomenon in the total patient collective, occurring significantly more frequently in mesial than in extra-mesial seizure onset. Aura appeared most often in seizures of left mesial origin. (ii) Vocalization presented a trend towards mesial left seizure origin. (iii) Oral automatisms showed a trend towards mesial seizure origin. Following noticeable distribution was observed for the extra-mesial group: In patients without aura, restlessness as initial ictal phenomenon appeared exclusively in seizures of extra-mesial right origin. Finally, behavioral arrest showed a trend towards left-sided seizure origin. In conclusion, the initial ictal element may add useful information concerning differentiation of seizure onset in TLE.