Characterizing Frontal Lobe Seizure Semiology in Children.

OBJECTIVE: The objective was to analyze seizure semiology in pediatric frontal lobe epilepsy patients, considering age, to localize the seizure onset zone for surgical resection in focal epilepsy. METHODS: Fifty patients were identified retrospectively, who achieved seizure freedom after frontal lobe resective surgery at Great Ormond Street Hospital. Video-electroencephalography recordings of preoperative ictal seizure semiology were analyzed, stratifying the data based on resection region (mesial or lateral frontal lobe) and age at surgery (≤4 vs >4). RESULTS: Pediatric frontal lobe epilepsy is characterized by frequent, short, complex seizures, similar to adult cohorts. Children with mesial onset had higher occurrence of head deviation (either direction: 55.6% vs 17.4%; p = 0.02) and contralateral head deviation (22.2% vs 0.0%; p = 0.03), ictal body-turning (55.6% vs 13.0%; p = 0.006; ipsilateral: 55.6% vs 4.3%; p = 0.0003), and complex motor signs (88.9% vs 56.5%; p = 0.037). Both age groups (≤4 and >4 years) showed hyperkinetic features (21.1% vs 32.1%), contrary to previous reports. The very young group showed more myoclonic (36.8% vs 3.6%; p = 0.005) and hypomotor features (31.6% vs 0.0%; p = 0.003), and fewer behavioral features (36.8% vs 71.4%; p = 0.03) and reduced responsiveness (31.6% vs 78.6%; p = 0.002). INTERPRETATION: This study presents the most extensive semiological analysis of children with confirmed frontal lobe epilepsy. It identifies semiological features that aid in differentiating between mesial and lateral onset. Despite age-dependent differences, typical frontal lobe features, including hyperkinetic seizures, are observed even in very young children. A better understanding of pediatric seizure semiology may enhance the accuracy of onset identification, and enable earlier presurgical evaluation, improving postsurgical outcomes. ANN NEUROL 2024;95:1138-1148.

Strange episodes during sleep - epilepsy or parasomnia? / Underlige episoder under søvn ­ epilepsi eller parasomni?

In some forms of epilepsy, the seizures occur almost exclusively during sleep. This is particularly the case with hypermotor frontal lobe seizures. Clinically it can be difficult to distinguish such seizures from parasomnias and psychogenic non-epileptic seizures. This clinical review article aims to highlight the importance of making the correct diagnosis, as these conditions require completely different treatment.

[The relationship between immunological and psychopathological characteristics in patients with focal epilepsy depending on the profile of interhemispheric asymmetry]. / Vzaimosvyaz' immunologicheskikh i psikhopatologicheskikh kharakteristik u bol'nykh fokal'noi epilepsiei v zavisimosti ot profilya mezhpolusharnoi asimmetrii.

OBJECTIVE: To identify the possible influence of cellular immunity parameters and neurobiological variables (frequency of seizures of various semiotics and their severity) on comorbid psychopathological symptoms depending on the profile of interhemispheric asymmetry in patients with focal forms of epilepsy. MATERIAL AND METHODS: The study included 92 patients with epilepsy (38 men, 54 women, mean age 38.7+8.45 years). Focal temporal lobe epilepsy was diagnosed in 36 patients, focal frontal lobe epilepsy in 16 patients, and temporal-frontal lobe epilepsy in 40 patients. For each type of seizure, severity was assessed according to the National Seizure Severity Scale (NHS3). The mental status of patients was assessed using the SCL-90 self-report questionnaire. The Annette scale was used to assess the profile of interhemispheric asymmetry. The number of different clusters of lymphocytes was studied, including the number of T-lymphocytes (CD3+), T-helpers (CD3+CD4+), T-cytotoxic (CD3+CD8+), T-NK (natural killers CD3+CD16+CD56+), B-lymphocytes (CD3-CD19+), as well as immunoregulatory index (CD4/CD8 ratio). In order to identify any possible relationships between neurobiological and immune variables, on the one hand, and the SCL-90 constructs, on the other hand, a separate correlation analysis of Spearman ranks within the left-handed group and the right-handed group was carried out. RESULTS: We revealed the differences between groups of patients with epilepsy with right and left profiles of hemispheric asymmetry regarding the relationship between the frequency of seizures, their severity and accompanying psychopathological variables, on the one hand, and between immunity indices and psychopathological constructs, on the other hand. It has been established that neurobiological and immune variables in left-handers can determine the psychopathological structure of the comorbid mental disorder. CONCLUSION: Prediction of concomitant psychopathological syndromes in patients with epilepsy on the basis of clinical data and data on immunity is quite possible, but only in left-handed patients.

Optimizing Surgical Planning for Epilepsy Patients With Multimodal Neuroimaging and Neurophysiology Integration: A Case Study.

SUMMARY: Current preoperative evaluation of epilepsy can be challenging because of the lack of a comprehensive view of the network's dysfunctions. To demonstrate the utility of our multimodal neurophysiology and neuroimaging integration approach in the presurgical evaluation, we present a proof-of-concept for using this approach in a patient with nonlesional frontal lobe epilepsy who underwent two resective surgeries to achieve seizure control. We conducted a post-hoc investigation using four neuroimaging and neurophysiology modalities: diffusion tensor imaging, resting-state functional MRI, and stereoelectroencephalography at rest and during seizures. We computed region-of-interest-based connectivity for each modality and applied betweenness centrality to identify key network hubs across modalities. Our results revealed that despite seizure semiology and stereoelectroencephalography indicating dysfunction in the right orbitofrontal region, the maximum overlap on the hubs across modalities extended to right temporal areas. Notably, the right middle temporal lobe region served as an overlap hub across diffusion tensor imaging, resting-state functional MRI, and rest stereoelectroencephalography networks and was only included in the resected area in the second surgery, which led to long-term seizure control of this patient. Our findings demonstrated that transmodal hubs could help identify key areas related to epileptogenic network. Therefore, this case presents a promising perspective of using a multimodal approach to improve the presurgical evaluation of patients with epilepsy.

Pearls & Oy-sters: Mesial Temporal Seizures in the Absence of the Mesial Temporal Lobe. Seizure Onset vs Seizure Network

Seizure semiology represents the clinical expression of the activation of the several brain regions comprising an epileptic network. In mesial temporal lobe epilepsy (MTLE), this network includes the insular-opercular-neocortical temporal-hippocampal (IONTH) regions. In this study, we present the case of a patient with pharmacoresistant seizures characterized by nausea, lip-smacking, semipurposeful hand movements, and speechlessness, suggesting dominant hemisphere MTLE, with scalp video-EEG findings and left hippocampal sclerosis on brain MRI confirming the diagnosis. She underwent anterior temporal lobectomy with amygdalohippocampectomy and was seizure-free for 14 years before relapsing. Recurrent seizure semiology was similar to preoperative seizures, that is, consistent with left MTLE, despite the medial temporal lobe missing. Seizures were therefore assumed to arise from remnant portions of the IONTH network-the insula, operculum, and posterolateral temporal neocortex. Reinvestigation including MEG localization of spikes and acute MRI changes following a seizure cluster suggested a left opercular region epilepsy. Our patient thus demonstrated the principle that seizures with mesial temporal characteristics may arise from outside the mesial temporal lobe (MTL). MTLE semiology arises from the activation of a set of structures (the seizure network) associated with the MTL, which can be triggered by foci both within and outside the MTL itself, and indeed even in its absence. However, it is not necessary to resect the entire extended network to bring about extended periods of seizure freedom in patients with refractory MTLE.

Transcutaneous auricular vagus nerve stimulation therapy in patients with cognitively preserved structural focal epilepsy: A case series report.

OBJECTIVE: Transcutaneous auricular vagus nerve stimulation (taVNS) was performed in two patients suffering structural focal epilepsy with preserved intellectual ability to show the feasibility of taVNS for specific patient groups. CASE PRESENTATIONS: Patient 1 was a 24-year-old woman with frontal lobe epilepsy who had weekly hyperkinetic seizures despite multiple anti-seizure medications. Patient 2 was a 27-year-old woman with parietal lobe epilepsy and focal cortical dysplasia in the vicinity of the lipoma in the corpus callosum. She experienced weekly focal-impaired awareness seizures even with anti-seizure medication. taVNS was applied to the left earlobe of both patients at 1.5 mA, 25 Hz, 250 µs pulse width, and 30 s stimulation with 30 s rest for 4 h per day. Over an 8-week baseline and 20 weeks of stimulation, the rate of reduction in seizure frequency was evaluated, along with quality-of-life using the Short-Form 36-Item Health survey. RESULTS: At baseline, we measured up to 11 and 12 focal seizures per week in Patient 1 and 2, respectively, with both patients achieving seizure freedom after 4 and 20 weeks taVNS, respectively. Patient 1 and 2 were observed for 18 and 14 months, respectively, including the clinical trial and follow-up observation period. Quality-of-life ratings increased in both patients, and no significant adverse events occurred during the study period. During the maintenance period after 20 weeks, seizures remained absent in Patient 1, and seizures remained reduced in Patient 2. CONCLUSION: Our results demonstrate that taVNS may be a promising tool for structural focal epilepsy with preserved cognitive function. A multicenter double-blind clinical trial is needed to confirm the role of taVNS as an anti-seizure tool.

MRI evidence for material-specific encoding deficits and mesial-temporal alterations in presurgical frontal lobe epilepsy patients.

OBJECTIVE: Neuroimaging studies reveal frontal lobe (FL) contributions to memory encoding. Accordingly, memory impairments are documented in frontal lobe epilepsy (FLE). Still, little is known about the structural or functional correlates of such impairments. Particularly, material specificity of functional changes in cerebral activity during memory encoding in FLE is unclear. METHODS: We compared 24 FLE patients (15 right-sided) undergoing presurgical evaluation with 30 healthy controls on a memory fMRI-paradigm of learning scenes, faces, and words followed by an out-of-scanner recognition task as well as regarding their mesial temporal lobe (mTL) volumes. We also addressed effects of FLE lateralization and performance level (normal vs. low). RESULTS: FLE patients had poorer memory performance and larger left hippocampal volumes than controls. Volume increase seemed, however, irrelevant or even dysfunctional for memory performance. Further, functional changes in FLE patients were right-sided for scenes and faces and bilateral for words. In detail, during face encoding, FLE patients had, regardless of their performance level, decreased mTL activation, while during scene and word encoding only low performing FLE patients had decreased mTL along with decreased FL activation. Intact verbal memory performance was associated with higher right frontal activation in FLE patients but not in controls. SIGNIFICANCE: Pharmacoresistant FLE has a distinct functional and structural impact on the mTL. Effects vary with the encoded material and patients' performance levels. Thus, in addition to the direct effect of the FL, memory impairment in FLE is presumably to a large part due to functional mTL changes triggered by disrupted FL networks. PLAIN LANGUAGE SUMMARY: Frontal lobe epilepsy (FLE) patients may suffer from memory impairment. Therefore, we asked patients to perform a memory task while their brain was scanned by MRI in order to investigate possible changes in brain activation during learning. FLE patients showed changes in brain activation during learning and also structural changes in the mesial temporal lobe, which is a brain region especially relevant for learning but not the origin of the seizures in FLE. We conclude that FLE leads to widespread changes that contribute to FLE patients' memory impairment.

Cathodal HD-tDCS and attention: A study on patients with intractable left lateral frontal lobe epilepsy.

OBJECTIVE: Defects in the attentional network in patients with epilepsy are influenced by factors such as the location of epileptic foci. Examining the impact of cathodal high-definition transcranial direct current stimulation (HD-tDCS) on attention components could provide insights into potential attention-related side effects of tDCS. This study aimed to investigate the effect of cathodal HD-tDCS on interictal epileptiform discharges (IEDs), auditory/visual (A/V) attention components, and reaction time (RT) in patients with intractable focal left lateral frontal lobe epilepsy (LFLE). METHODS: To control for variations in individual epilepsy syndrome, 12 adult participants diagnosed with drug-resistant left LFLE with focal cortical IEDs on C3 underwent repeated measurements at pretest, posttest, and follow-up steps. 4 × 1 ring electrodes (cathode on C3 and four anodes on F3, P3, T3, and Cz) delivered 2 mA DC for 20 min per session for 10 consecutive days. The integrated visual and auditory continuous performance test (IVA+) assessed the A/V attention components and RT. One-way repeated-measure ANOVA was used. RESULTS: The findings suggest a significant effect in reducing IEDs. The IVA+ results showed a significant improvement in auditory divided attention and visual selective and focused attention (p < 0.05). In the follow-up, these changes demonstrated lasting efficacy. A/V speed scales increased (p < 0.05), showing a significant decrease in reaction time. CONCLUSIONS: Cathodal HD-tDCS significantly reduced IEDs and improved the components of auditory divided attention, visual focused attention, and visual selective attention, with a reduction in patient reaction time. A significant lasting, side-effect-free positive effect was observed for up to one month after the intervention.

Application of the International Classification of Cognitive Disorders in Epilepsy (IC-CoDE) to frontal lobe epilepsy using multicenter data.

RATIONALE: The International Classification of Cognitive Disorders in Epilepsy (IC-CoDE) was recently introduced as a consensus-based, empirically-driven taxonomy of cognitive disorders in epilepsy and has been effectively applied to patients with temporal lobe epilepsy (TLE). The purpose of this study was to apply the IC-CoDE to patients with frontal lobe epilepsy (FLE) using national multicenter data. METHODS: Neuropsychological data of 455 patients with FLE aged 16 years or older were available across four US-based sites. First, we examined test-specific impairment rates across sites using two impairment thresholds (1.0 and 1.5 standard deviations below the normative mean). Following the proposed IC-CoDE guidelines, patterns of domain impairment were determined based on commonly used tests within five cognitive domains (language, memory, executive functioning, attention/processing speed, and visuospatial ability) to construct phenotypes. Impairment rates and distributions across phenotypes were then compared with those found in patients with TLE for which the IC-CoDE classification was initially validated. RESULTS: The highest rates of impairment were found among tests of naming, verbal fluency, speeded sequencing and set-shifting, and complex figure copy. The following IC-CoDE phenotype distributions were observed using the two different threshold cutoffs: 23-40% cognitively intact, 24-29% single domain impairment, 13-20% bi-domain impairment, and 18-33% generalized impairment. Language was the most common single domain impairment (68% for both thresholds) followed by attention and processing speed (15-18%). Overall, patients with FLE reported higher rates of cognitive impairment compared with patients with TLE. CONCLUSIONS: These results demonstrate the applicability of the IC-CoDE to epilepsy syndromes outside of TLE. Findings indicated generally stable and reproducible phenotypes across multiple epilepsy centers in the U.S. with diverse sample characteristics and varied neuropsychological test batteries. Findings also highlight opportunities for further refinement of the IC-CoDE guidelines as the application expands.

Rescue of Normal Excitability in LGI1-Deficient Epileptic Neurons.

Leucine-rich glioma inactivated 1 (LGI1) is a glycoprotein secreted by neurons, the deletion of which leads to autosomal dominant lateral temporal lobe epilepsy. We previously showed that LGI1 deficiency in a mouse model (i.e., knock-out for LGI1 or KO-Lgi1) decreased Kv1.1 channel density at the axon initial segment (AIS) and at presynaptic terminals, thus enhancing both intrinsic excitability and glutamate release. However, it is not known whether normal excitability can be restored in epileptic neurons. Here, we show that the selective expression of LGI1 in KO-Lgi1 neurons from mice of both sexes, using single-cell electroporation, reduces intrinsic excitability and restores both the Kv1.1-mediated D-type current and Kv1.1 channels at the AIS. In addition, we show that the homeostatic-like shortening of the AIS length observed in KO-Lgi1 neurons is prevented in neurons electroporated with the Lgi1 gene. Furthermore, we reveal a spatial gradient of intrinsic excitability that is centered on the electroporated neuron. We conclude that expression of LGI1 restores normal excitability through functional Kv1 channels at the AIS.SIGNIFICANCE STATEMENT The lack of leucine-rich glioma inactivated 1 (LGI1) protein induces severe epileptic seizures that leads to death. Enhanced intrinsic and synaptic excitation in KO-Lgi1 mice is because of the decrease in Kv1.1 channels in CA3 neurons. However, the conditions to restore normal excitability profile in epileptic neurons remain to be defined. We show here that the expression of LGI1 in KO-Lgi1 neurons in single neurons reduces intrinsic excitability, and restores both the Kv1.1-mediated D-type current and Kv1.1 channels at the axon initial segment (AIS). Furthermore, the homeostatic shortening of the AIS length observed in KO-Lgi1 neurons is prevented in neurons in which the Lgi1 gene has been rescued. We conclude that LGI1 constitutes a critical factor to restore normal excitability in epileptic neurons.

Arousal deregulation in the co-shaping of neuropsychological dysfunction in frontal and mesial temporal lobe epilepsy.

OBJECTIVE: Our work aims to investigate the role of physiological arousal in the expression of neuropsychological deficits in frontal lobe epilepsy (FLE) and mesial temporal lobe epilepsy (mTLE), by drawing on the Lurian theory of brain function. METHODS: For this study a total of 43 patients with focal onset epilepsy has been taken; twenty-four patients with FLE, 19 patients with mTLE and 26 healthy controls, all matched for age and education. Participants underwent a comprehensive neuropsychological assessment including various cognitive domains, such as attention, episodic memory, speed of information processing, response inhibition and mental flexibility, working memory, verbal fluency (phonological & semantic). RESULTS: There were no significant differences between FLE and mTLE patients in terms of neuropsychological performance. However, both FLE and mTLE patients showed significantly worse performance in several cognitive domains than HCs. The results seem to support our hypothesis that aberrant physiological arousal, as reflected in patients' worse performance in vigilance and attention, response inhibition, and processing speed, along with other disease-specific variables, may co-determine neuropsychological dysfunction and/or impairment in both FLE and mTLE. CONCLUSION: Identifying a differential arousal-related neuropsychological affection in FLE and mTLE, among the known deleterious effects of the functional deficit zone and other disease-related variables, may further our understanding of the underlying cognitive-pathophysiological mechanisms in focal epilepsy syndromes.

Frontal lobe epilepsy and mild malformation with oligodendroglial hyperplasia: Further observations on electroclinical and imaging phenotypes, and surgical perspectives.

OBJECTIVE: Mild malformation with oligodendroglial hyperplasia (MOGHE) is a recently described clinicopathologic entity, associated with drug-resistant epilepsy and extensive epileptogenic networks. Knowledge is accumulating about particular electroclinical phenotypes, correlations with imaging, and potential prognostic significance for surgical outcomes. The study adds relevant information by documenting the presence of a hyperkinetic frontal lobe seizure phenotype in adolescents and an epileptic encephalopathy phenotype in young children. METHODS: Five cases were subjected to a structured presurgical evaluation protocol, including EEG-FMRI, chronic and acute invasive EEG, subjected to frontal lobe surgery with postoperative follow-up between 15 months and 7 years. RESULTS: In the two adult cases, surface EEG demonstrated lateralized widespread frontal lobe epileptogenicity and hyperkinetic semiological features. MRI demonstrated cortical white matter blurring and deeper white matter abnormalities. EEG-FMRI suggested concordant frontal lobe involvement. iEEG demonstrated a widespread frontal lobe epilepsy network. The three young children demonstrated a diffuse epileptic encephalopathy phenotype, with nonlocalizing, nonlateralizing surface EEG, and "spasms" as the main seizure type. MRI demonstrated extensive frontal lobe subcortical gray and white matter abnormalities, consistent with MOGHE literature for this age, while EEG-FMRI, in 2/3, demonstrated concordant frontal lobe involvement. They did not undergo chronic iEEG, and the resection was assisted by acute intraoperative ECoG. All cases were subjected to extensive frontal lobectomies with Engel class IA (2/5), IB (1/5), and IIB (2/5) outcomes. SIGNIFICANCE: The study confirms the presence of frontal lobe epilepsy and epileptic encephalopathy phenotypes, in accordance with epilepsy phenotypes already described in MOGHE literature. Presurgical evaluation studies, including EEG-FMRI, can provide strong lateralizing and localizing evidence of the epileptogenic networks involved. All responded favorably to extensive frontal lobe resections, despite widespread epileptic activity recorded by surface and intracranial EEG pre- and postoperatively; an epileptic encephalopathy phenotype, in the first years of life, should not discourage such a resection.

Decision-making under ambiguity after frontal lobe resection for epilepsy.

PURPOSE: Decision-making is crucial to daily life and can impact our society as well as economic conditions. Although the frontal lobes have been identified as important for decision-making, this capacity has only been studied to a limited extent in frontal lobe epilepsy and not at all after frontal lobe resection (FLR) for epilepsy. This study aimed to explore decision-making under ambiguity after FLR for epilepsy. METHODS: Fourteen patients having undergone FLR for epilepsy completed the Iowa Gambling Task (IGT) which is a widely used tool to measure decision-making under ambiguity. Iowa Gambling Task scores included in the analysis were: total net score, separate scores from five blocks across the test, and a change score (last block of IGT minus first block). A group of healthy controls (n = 30) was used as a comparison. Associations between IGT and standardized neuropsychological methods for assessment of executive functions, self-rating questionnaires of mental health, fatigue, and behavior linked to frontal lobe dysfunction were also investigated. RESULTS: The patient group performed inferior to controls at the final block of the IGT (p =.001).A group difference in IGT change scores was found (p =.005), reflectingthe absence of a positive change in performance over time for the FLR group compared to the control group. Correlations with tests of executive functions as well as self-rating scales were mainly statistically nonsignificant. CONCLUSIONS: This study shows that patients having undergone FLR for epilepsy have difficulties with decision-making under ambiguity. The performance illustrated a failure to learn throughout the task. Executive as well as emotional deficits may impact decision-making processes in this patient group and need to be considered in further studies. Prospective studies with larger cohorts are needed.

Executive functioning in children with posterior cortex epilepsy compared to temporal and frontal lobe epilepsies.

OBJECTIVE: People with epilepsy are at an increased risk of experiencing executive dysfunction, particularly those with frontal lobe epilepsy (FLE). The literature has also demonstrated alterations in executive functioning (EF) in patients with temporal lobe epilepsy (TLE). However, few studies have examined the neuropsychological profile of posterior cortex epilepsy (PCE), and little attention has been given to cognitive impairments in the pediatric population with PCE. This study aims to investigate EF performance in children with drug-resistant PCE compared to patients with FLE and TLE. METHODS: We analyzed neuropsychological data from 217 patients aged 6-18 years who underwent preoperative evaluation for epilepsy surgery. The EF of patients with PCE was compared to patients with FLE and TLE. RESULTS: There was no significant difference in Full-Scale Intelligence Quotient (FSIQ) means between groups. However, we found a significant effect of brain region on the Coding task, in which patients with PCE and FLE performed worse than those with TLE (p = 0.034). We also observed performance differences between groups on the Stroop test (p = 0.005), with patients with PCE and FLE performing worse than the TLE group. SIGNIFICANCE: These findings suggest that children with PCE have alterations in their EF that are similar to the deficits found in FLE compared to patients with TLE. This emphasizes the importance of understanding the neuroanatomy of executive functions and the model of neural networks extending beyond the prefrontal cortex.

Thalamostriatal disconnection underpins long-term seizure freedom in frontal lobe epilepsy surgery.

Around 50% of patients undergoing frontal lobe surgery for focal drug-resistant epilepsy become seizure free post-operatively; however, only about 30% of patients remain seizure free in the long-term. Early seizure recurrence is likely to be caused by partial resection of the epileptogenic lesion, whilst delayed seizure recurrence can occur even if the epileptogenic lesion has been completely excised. This suggests a coexistent epileptogenic network facilitating ictogenesis in close or distant dormant epileptic foci. As thalamic and striatal dysregulation can support epileptogenesis and disconnection of cortico-thalamostriatal pathways through hemispherotomy or neuromodulation can improve seizure outcome regardless of focality, we hypothesize that projections from the striatum and the thalamus to the cortex may contribute to this common epileptogenic network. To this end, we retrospectively reviewed a series of 47 consecutive individuals who underwent surgery for drug-resistant frontal lobe epilepsy. We performed voxel-based and tractography disconnectome analyses to investigate shared patterns of disconnection associated with long-term seizure freedom. Seizure freedom after 3 and 5 years was independently associated with disconnection of the anterior thalamic radiation and anterior cortico-striatal projections. This was also confirmed in a subgroup of 29 patients with complete resections, suggesting these pathways may play a critical role in supporting the development of novel epileptic networks. Our study indicates that network dysfunction in frontal lobe epilepsy may extend beyond the resection and putative epileptogenic zone. This may be critical in the pathogenesis of delayed seizure recurrence as thalamic and striatal networks may promote epileptogenesis and disconnection may underpin long-term seizure freedom.

Posterior Cingulate Epilepsy: A Systematic Review.

SUMMARY: In this review, authors discuss epilepsy originating from posterior cingulate regions, a challenging entity to diagnose and most likely underrecognized. A systematic review of posterior middle and posterior cingulate epilepsy cases was conducted to present a summary of current knowledge about this localization-based type of epilepsy. The literature search identified 32 articles, for a total of 69 patients (34 with posterior middle cingulate epilepsy [pMCE] and 35 with posterior cingulate epilepsy [PCE]). Most patients were children and young adults with drug-resistant lesional epilepsy with high seizure burden. In both groups, most patients reported auras, mainly sensory, but various types were reported, including autonomic, behavioral, and cognitive manifestations. Most pMCE and PCE showed motor manifestations (mainly respectively asymmetric tonic posturing and automotor features). Impaired awareness during seizures was more frequently reported in PCE than in pMCE. As for ictal scalp EEG, epileptogenic abnormalities were poorly lateralized and did not localize the seizure onset zone. An ictal temporal involvement was only observed in PCE. Interictal EEG findings were nonspecific. As for other presurgical noninvasive investigations, data are limited, and no studies have adequately assessed their value. Partly explained by our inclusion criteria, most patients underwent a surgical procedure (either lesionectomy or resection of epileptogenic zone as defined by intracranial EEG study results), which overall yielded good outcomes.

Anterior Cingulate Epilepsy: A Review.

SUMMARY: In this review, the semiology, and characteristics of noninvasive investigations suggestive of anterior cingulate and anterior midcingulate epilepsy are detailed by the authors. The clinical presentation is representative of a recently recognized rostrocaudal gradient of functional connectivity with seizures of the anterior cingulate cortex manifesting emotional and interoceptive aura followed by a hyperkinetic or complex motor seizures. The few reports of anterior midcingulate epilepsy show a trend toward a higher proportion of sensory auras and premotor semiology. Ictal pouting, vocalizations, and, in particular, laughter are strong indicators of epilepsy arising or spreading to this region. Although scalp EEG was traditionally thought to provide little information, the data provided in this review demonstrate that most patients will have abnormalities over the frontal or frontotemporal regions. Frontotemporal abnormalities at least interictally provide valuable information regarding lateralization. The etiology of epilepsy arising from the anterior cingulate region seems to be most frequently secondary to focal cortical dysplasia (FCD), followed by neoplasms and vascular lesions, particularly cavernomas, although one cannot rule out a publication bias. Findings of nuclear medicine imaging is seldomly reported but both positron emission tomography and ictal single-photon computed tomography can identify the generator or the network often showing abnormalities extending to the frontal regions. The few available magnetoencephalography (MEG) studies reveal mixed results, sometimes providing false lateralization of the focus. Anterior cingulate epilepsy is difficult to recognize, but the features summarized in this review should prompt suspicion in clinical practice.