The Rolandic operculum generates different semiologies in insulo-opercular and temporal lobe epilepsies.

PURPOSE: The role of the Rolandic operculum in in mesial temporal lobe epilepsy (MTLE) is to produce oroalimentary automatisms (OAAs). In insulo-opercular epilepsy (IOE), the Rolandic operculum may produce perioral muscle clonic or tonic movements or contractions. This paper aims to confirm the symptomatogenic zone of facial symptoms in IOE and to explain this phenomenon. METHODS: A total of 45 IOE patients and 15 MTLE patients were analyzed. The patients with IOE were divided into facial (+) and (-) groups according to the facial symptoms. The interictal positron emission tomography (PET) data were compared among groups. Furthermore, electroclinical correlation, functional connectivity and energy ratio (ER) were analyzed with stereo-electroencephalography (SEEG). RESULTS: Intergroup PET differences were observed mainly in the Rolandic operculum. Electroclinical correlation showed that the Rolandic operculum was the only brain area showing any correlations. Compared with the facial (-) group, the facial (+) group showed stronger functional connectivity and a higher ER in the alpha 1, alpha 2 and beta sub-bands. In the Rolandic operculum, compared with those of the MTLE group, the h2 and ER of the facial (+) group were higher in the high frequency sub-bands. Intergroup comparison of the ER in the seizure onset zones (SOZ) showed no significant difference. SIGNIFICANCE: The symptomatogenic zone of facial symptoms in IOE is the Rolandic operculum. Seizure propagation to the Rolandic operculum generates different semiologies because of the different synchronization frequencies and energies of the sub-bands depending on the site of seizure origin. This may be due to the complex spreading pathway from the SOZ to the symptomatogenic zone.

Neural networks underlying hyperkinetic seizures: A quantitative PET and SEEG study.

OBJECTIVE: Hyperkinetic seizures (HKS) are characterized by complex movements that commonly occur during seizures arising from diverse cortical structures. A common semiology network may exist and analyzing the anatomo-electrical mechanisms would facilitate presurgical evaluation. Here, quantitative positron emission tomography (PET) and stereoelectroencephalography (SEEG) analysis was used to explore the underlying mechanism of HKS. METHODS: We retrospectively collected patients with epilepsy with HKS between 2014 and 2019. The interictal PET data of patients with epilepsy with HKS were compared with those of 25 healthy subjects using statistical parametric mapping to identify regions with significant hypometabolism. Then, regions of interest (ROI) for SEEG analysis were identified based on the results of PET analysis. Patients in which the ROIs were covered by intracerebral electrodes were selected for further analysis. Stereoelectroencephalography -clinical correlations with latency measurements were analyzed, and we also performed coherence analysis among ROIs both before and during HKS. RESULTS: Based on the inclusion criteria, 27 patients were analyzed. In the PET analysis, significant hypometabolism was observed in the ipsilateral dorsoanterior insular lobe, bilateral mesial frontal lobes (supplementary motor area/middle cingulate cortex, SMA/MCC), and the bilateral heads of the caudate nuclei in patients with HKS compared with the control group (p < 0.001). We selected dorsoanterior insula and SMA/MCC as ROIs for SEEG analysis. Eight patients with 23 HKS events were selected for further analysis. There was a linear correlation between the ictal involvement of both the dorsoanterior insula and SMA/MCC with the onset of HKS. Stereoelectroencephalography analysis indicated alpha range activity seemed more often associated with dorsoanterior insula and SMA/MCC involvement during HKS. CONCLUSIONS: The dorsoanterior insular lobe, mesial frontal lobes (SMA/MCC), and the bilateral heads of the caudate nuclei were probably involved in the generation of HKS. The SEEG analysis further indicated that the occurrence of HKS might be partly associated with synchronized rhythmical alpha activity between dorsoanterior insula and SMA/MCC.

The electroclinical features and surgical outcomes of inferior perisylvian epilepsy.

OBJECTIVE: To summarize the clinical and electrophysiological observations of epilepsy originating from the inferior perisylvian cortex, and analyze the potential epileptic networks underlying the semiological manifestations. METHODS: We retrospectively analyzed patients with refractory inferior perisylvian epilepsy (IPE) who had undergone resective surgery, and then reviewed the demographic, clinical, neuroelectrophysiological, neuroimaging, surgical, histopathological, and follow-up data of the patients from the respective medical records. The selected patients were then categorized in accordance with the results of semiological analysis. Quantitative 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) analysis was performed to investigate the underlying neural network. RESULTS: Of the 18 IPE patients assessed in this study, ipsilateral frontotemporal epileptic discharges or its onsets were the dominant interictal or ictal scalp EEG observations. In addition, oroalimentary or manual automatism was the most frequently documented manifestation, followed by facial tonic or clonic movements. Moreover, the semiological analysis identified and classified the patients into 2 patterns, and the PET statistical analyses conducted on these 2 groups revealed differences in the neural network between them. CONCLUSION: Inferior perisylvian epilepsy possesses semiological manifestations similar to those of mesial temporal lobe epilepsy or rolandic opercular epilepsy, hence these conditions should be carefully differentiated. Performing lesionectomy or cortectomy, sparing the mesial temporal structures, was found to be an effective and safe treatment modality for IPE.

Stereoelectroencephalography-guided radiofrequency thermocoagulation for hypothalamic hamartoma: Electroclinical patterns and the relationship with surgical prognosis.

BACKGROUND: Radiofrequency thermocoagulation (RFTC) guided by stereo-electroencephalography (SEEG) has been proved to be a safe method to reduce seizure frequency in patients with drug-resistant epilepsy. However, there are few reports addressing the value and safety of this procedure in hypothalamic hamartoma (HH). OBJECTIVE: To present the results of our experience using SEEG-guided RFTC in HH patients with drug-resistant epilepsy, and identify outcome predictors. METHODS: We retrospectively reviewed the clinical and surgical characteristics of 27 HH-related patients with epilepsy in our center between 2015 and 2019. All patients underwent invasive recordings with SEEG before RFTC was performed. We reported surgical outcome predictors and postoperative follow-up concerning safety and efficacy (mean follow-up, 27.3 months; range, 12-63). Surgical strategy was also analyzed. RESULTS: Nineteen patients (70.4%) achieved Engel's class I outcome, while 4 patients (14.8%) did not show significant improvement. Of all observed seizures, two different onset patterns of intracranial electrophysiology recorded by SEEG were observed. Patients presented with focal low-voltage fast activity were more likely to obtain seizure freedom (p = 0.045), while classification (p = 0.478), volume (p = 0.546), history of resection (p = 0.713), seizure types (p = 0.859), or seizure duration (p = 0.415) showed no significant effect on the outcome. Weight gain was the most common long-term complication (18.5%). CONCLUSION: The SEEG can guide the ablation of HH and serve as an important factor to predict favorable seizure outcomes. Radiofrequency thermocoagulation guided by SEEG can offer a minimally invasive and low-risk surgical approach with excellent outcomes. Disconnecting the attachment of HH should be the appropriate strategy to obtain the best seizure outcome.

Intravenous methylprednisolone or immunoglobulin for anti-glutamic acid decarboxylase 65 antibody autoimmune encephalitis: which is better?

BACKGROUND: Patients positive for anti-glutamic acid decarboxylase 65 (GAD65) antibodies have attracted increasing attention. Their clinical manifestations are highly heterogeneous and can be comorbid with tumors. Currently, there is no consensus on the therapeutic regimen for anti-GAD65-associated neurological diseases due to the clinical complexity, rarity and sporadic distribution. We reported six anti-GAD65 autoimmune encephalitis (AE) patients who received intravenous methylprednisolone (IVMP) or immunoglobulin (IVIG) or both. Then, we evaluated the therapeutic effect of both by summarizing results in previous anti-GAD65 AE patients from 70 published references. RESULTS: Our six patients all achieved clinical improvements in the short term. Unfortunately, there was no significant difference between IVMP and IVIG in terms of therapeutic response according to the previous references, and the effectiveness of IVMP and IVIG was 45.56% and 36.71%, respectively. We further divided the patients into different subgroups according to their prominent clinical manifestations. The response rates of IVMP and IVIG were 42.65% and 32.69%, respectively, in epilepsy patients; 60.00% and 77.78%, respectively, in patients with stiff-person syndrome; and 28.57% and 55.56%, respectively, in cerebellar ataxia patients. Among 29 anti-GAD65 AE patients with tumors, the response rates of IVMP and IVIG were 29.41% and 42.11%, respectively. There was no significant difference in effectiveness between the two regimens among the different subgroups. CONCLUSION: Except for stiff-person syndrome, we found that this kind of AE generally has a poor response to IVMP or IVIG. Larger prospective studies enrolling large numbers of patients are required to identify the optimal therapeutic strategy in the future.

Acute symptomatic seizures secondary to autoimmune encephalitis and autoimmune-associated epilepsy: Conceptual definitions.

Seizures are a well-recognized and often prominent manifestation of autoimmune encephalitic syndromes. Progress in detection of pathogenic neural autoantibodies has led to increased awareness of autoimmune causes of seizures. Clinical studies of patients with these autoantibodies have improved our understanding of the seizure characteristics, treatments, and seizure prognosis in these disorders. The International League Against Epilepsy (ILAE) Autoimmunity and Inflammation Taskforce proposes conceptual definitions for two main diagnostic entities: (a) acute symptomatic seizures secondary to autoimmune encephalitis, and (b) autoimmune-associated epilepsy, the latter of which suggests an enduring predisposition to seizures. Such a distinction is relevant when discussing the pathophysiology, treatment, prognosis, and social consequences of these disorders. We discuss the role of biomarkers in the application of these conceptual definitions and illustrate their use in patients cared for by members of the task force.

Symptomatogenic zone and network of oroalimentary automatisms in mesial temporal lobe epilepsy.

OBJECTIVE: Oroalimentary automatisms (OAAs) are common clinical manifestations of medial temporal lobe epilepsy. Nevertheless, the location of the symptomatogenic zone of OAAs remains unclear. The generation mechanism of OAAs also has not been clarified. We attempt to explain these problems by analyzing interictal [18 F]-fluorodeoxyglucose positron emission tomography (18 FDG-PET) imaging and ictal stereo-electroencephalography (SEEG) recordings in patients with medial temporal lobe epilepsy. METHODS: Fifty-seven patients with mesial temporal lobe epilepsy were analyzed retrospectively. All underwent anterior temporal lobectomy (ATL) and were seizure-free. The patients were divided into OAA (+) and OAA (-) groups according to the occurrence of consistent stereotyped OAAs. The interictal PET data were compared with those of 18 healthy controls and were then compared between groups using statistical parametric mapping (SPM). Functional connectivity using linear regression analysis was performed between the target brain regions. To clarify the network of OAAs, ictal epileptogenicity index (EI) values, and the nonlinear correlation method h2 were performed with SEEG on patients. RESULTS: Compared to OAAs (-), the rolandic operculum was the only area with significant differences. Hippocampus and rolandic operculum showed significant correlations in the OAA (+) group (y = 0.758x+0.470, R2  = 0.456, P = 0.000). No correlation was found in the OAA (-) group (P = 0.486). The EI values of the OAA (+) group (median 0.20) were significantly higher (P < 0.0001) than those of the OAA (-) group (median 0). The h2 in the OAA (+) group (h2  = 0.23 ± 0.13) showed stronger functional connectivity (t = 6.166, P < 0.0001) than that of the OAA (-) group (h2  = 0.08 ± 0.05). SIGNIFICANCE: The rolandic operculum is most likely to be the symptomatogenic zone of OAAs. In medial temporal lobe epilepsy, unilateral functional connection from the hippocampus to the rolandic operculum during seizure onset is the basis for the generation of OAAs.

Seizure semiology in leucine-rich glioma-inactivated protein 1 antibody-associated limbic encephalitis.

OBJECTIVE: The objective of this study was to advance the characterization of seizure semiology in leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis (LE). METHODS: Eighteen patients diagnosed with LGI1 LE were identified. Seizure semiology, demographic features, MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), electroencephalograms, and outcomes following immunotherapy were evaluated. RESULTS: Patients were divided into the following groups based on seizure semiology: faciobrachial dystonic seizure only (FBDS-only, n=4), epileptic seizure without FBDS (Non-FBDS, n=6), and FBDS plus epileptic seizure (FBDS+, n=8). In the group with Non-FBDS, the majority of patients (5/6) manifested mesial temporal lobe epilepsy (MTLE) like semiology (i.e., fear, epigastric rising, staring, and automatisms) with a frequency of 7±5 times per day and a duration of 15.3±14.3s. In the group with FBDS+, the distinctive symptom was FBDS followed by epileptic events, especially automatisms (7/8), with a frequency of 16±12 times per day and a duration of 13.0±8.0s. In these cases, 67% and 50% of the patients showed abnormalities on MRI and FDG-PET, respectively, and the mesial temporal lobe structures were most often involved. Ictal discharges were observed in 0/4, 6/6, and 8/8 of the patients in the groups with FBDS only, Non-FBDS, and FBDS+, respectively. The temporal lobe was mainly affected. Immunotherapy had favorable therapeutic effects. SIGNIFICANCE: The LGI1 LE should be considered as one disease syndrome with a series of clinical manifestation. Identifying types of unique semiology features will facilitate the early diagnosis and the timely initiation of immunotherapy.

Circular RNA: a new star in neurological diseases.

Circular RNAs (circRNAs) are novel endogenous non-coding RNAs characterized by the presence of a covalent bond linking the 3' and 5' ends generated by backsplicing. In this review, we summarize a number of the latest theories regarding the biogenesis, properties and functions of circRNAs. Specifically, we focus on the advancing characteristics and functions of circRNAs in the brain and neurological diseases. CircRNAs exhibit the characteristics of species conservation, abundance and tissue/developmental-stage-specific expression in the brain. We also describe the relationship between circRNAs and several neurological diseases and highlight their functions in neurological diseases.

Temporal lobe epilepsy with amygdala enlargement: a subtype of temporal lobe epilepsy.

BACKGROUND: Some recent studies suggest that some imaging-negative temporal lobe epilepsy (TLE) had significant amygdala enlargement (AE). Contradictory data were also reported in previous studies regarding the association between AE and TLE. The present study was to investigate the clinical characters of a group of TLE with AE and compare the amygdala volume of the same patient before and after antiepileptic drugs treatment by a larger sample size. METHODS: This study recruited 33 mesial TLE patients with AE and 35 healthy volunteers. The clinical history, seizure semiology, electroencephalogram (EEG), fluorodeoxyglucose-positron emission tomography (FDG-PET) and amygdala volume were investigated. The amygdala volume were compared between ipsilateral and contralateral sides, TLE patients and 35 healthy controls, and patients at first and follow-up visit by 3.0 T MRI. RESULTS: Average seizure onset age was 42.0 years (SD 14.3). All patients had complex partial seizures, fourteen had occasional generalized tonic-clonic seizures which often happened during sleep. Ninety percent patients suffered from anxiety or depression. Thirty percent patients had memory decline. Interictal epileptiform discharges appeared predominantly in the anterior or inferior temporal area ipsilateral to AE. Interictal FDG-PET showed regional glucose hypometabolism in the ipsilateral temporal lobe. No hippocampal sclerosis (HS) was suspected in all patients. 22 patients demonstrated good seizure control and significantly reduced volume of the enlarged amygdala after treatment (P < 0.01). The other 11 patients showed initial response to treatment, followed by a gradual increase in seizure frequency over time, and no volume change of the enlarged amygdala after treatment. CONCLUSIONS: TLE with AE probably represents a distinct nosological and probably less homogeneous syndrome which is most likely a subtype of TLE without ipsilateral HS. The chronic and long lasting inflammatory processes or focal cortical dysplasia could lead to amygdala enlargement possibly.

Somatosensory reflex seizures in a patient with hypertrophic cranial pachymeningitis.

Somatosensory-evoked reflex epilepsy is characterized by seizures in response to specific stimuli. It is highly uncommon for somatosensory-evoked focal seizures to be caused by movement or a change in posture. Reflex epilepsy induced by both somatosensory and proprioceptive stimulations has not been previously reported. In this study, we present a case of reflex epilepsy evoked by somatosensory and proprioceptive stimulation in a patient with hypertrophic cranial pachymeningitis. After comparing our patient with other cases of previously reported somatosensory-evoked reflex epilepsy, we determined that our patient had an unusual cause of reflex epilepsy.

Long-term seizure outcome with the surgically remediable syndrome of frontal lobe epilepsy associated with superior frontal sulcus-related dysplasia.

Objective: To assess the long-term outcome of the surgically remediable syndrome of frontal lobe epilepsy (FLE) associated with superior frontal sulcus (SFS)-related dysplasia. Methods: We retrospectively reviewed the medical charts and surgical features of 31 patients with drug-resistant frontal lobe epilepsy in our centers between 2016 and 2018. All patients underwent surgical resection. According to the epileptogenic zone (EZ), localization and resection extent were classified as (1) pure SFS group (PS group), (2) associated SFS group (AS group), and (3) no SFS group (NS group). The general characteristics, neuroradiological findings, morbidity, pathology, and long-term seizure outcome after surgery were analyzed to extract the potential value of the surgery for SFS-related dysplasia. Results: Of 31 patients with FLE who underwent epilepsy surgery, 15 patients (nine men) were included PS group, five patients (five men) in the AS group, and 11 patients (eight men) in the NS group. Eleven patients detected abnormal focal signals in the presurgical MRI. Six patients in the PS group demonstrated the suspected focal cortical dysplasia (FCD) in the SFS detected with MRI. All patients demonstrated focal abnormal hypometabolism foci in the PET-MR co-registration. Twenty-five patients (80.6%) were seizure-free since surgery, including all 15 patients (100%) of the PS group, three in five patients (60%) of the AS group, and seven in 11 patients (63.6%) of the NS group. The difference in outcome between different groups was significant (p = 0.004, PS vs. AS group; p = 0.005, PS vs. NS group). As of the last follow-up (mean 66.2 ± 9.7months), 25 patients (80.6%) were seizure-free since surgery (Engel's class I). In addition, antiseizure medication was withdrawn in 19 patients (61.3%). Histologic examination of resected specimens revealed FCD in all 31 patients. The percentage of FCD II type was 100, 60, and 63.6% in the three different groups, respectively. Conclusion: SFS-related dysplasia is a neuropathologic entity with a favorable postoperative outcome. FCD II is the most common type of SFS-related dysplasia. FDG-PET co-registered with MRI should be performed in patients with suspected SFS-related dysplasia, since it may depict areas of hypometabolism suggestive of dysplasia in the absence of MRI abnormalities.

Associated factors with stimulation induced seizures and the relevance with surgical outcomes.

OBJECTIVE: To analyze the associated factors with stimulation-induced seizures (SIS) and the relevant factors in predicting surgical outcomes. METHODS: We analyzed 80 consecutive epilepsy patients explored by stereo-electroencephalography with routine electrical stimulation mapping (ESM). If seizures induced by ESM, patients were classified as SIS-positive (SIS-P); otherwise, SIS-negative (SIS-N). Patients received radical surgery were further classified as favorable (Engel I) and unfavorable (Engel II-IV) groups. RESULTS: Of the 80 patients included, we identified 44 (55.0%) and 36(45.0%) patients in the SIS-P and SIS-N groups, respectively. Multivariate analysis revealed that the seizure onset pattern (SOP) of preceding repetitive epileptiform discharges following LVFA (PRED→LVFA) (OR 3.319, 95% CI 1.200-9.183, P = 0.021) and pathology of focal cortical dysplasia (FCD) type II (OR 3.943, 95% CI 1.093-14.226, P = 0.036) were independent factors influencing whether the electrical stimulation can induce a seizure. Among the patients received radical surgery, there were 55 and 15 patients in the favorable and unfavorable groups separately. Multivariate analysis revealed that the SOP of PRED→LVFA induced seizures by stimulation (OR 11.409, 95% CI 1.182-110.161, P = 0.035) and bilateral implantation (OR 0.048, 95% CI 0.005-0.497, P = 0.011) were independent factors affecting surgical outcomes. The previous epilepsy surgery had a trend to be a negative factor with SIS (OR 0.156, 95% CI 0.028-0.880, P = 0.035) and surgical outcomes (OR 0.253, 95% CI 0.053-1.219, P = 0.087). CONCLUSION: ESM is a highly valuable method for localizing the seizure onset zone. The SOP of PRED→LVFA and FCD type II were associated with elicitation of SIS by ESM, whereas a previous epilepsy surgery showed a negative association. Furthermore, the SOP of PRED→LVFA together with SIS in the same patient predicted favorable surgical outcomes, whereas bilateral electrode implantation predicted unfavorable outcomes.

Neural network mapping of gelastic behavior in children with hypothalamus hamartoma.

BACKGROUND: Hypothalamus hamartomas (HHs) are rare, congenital, tumor-like, and nonprogressive malformations resulting in drug-resistant epilepsy, mainly affecting children. Gelastic seizures (GS) are an early hallmark of epilepsy with HH. The aim of this study was to explore the disease progression and the underlying physiopathological mechanisms of pathological laughter in HH. METHODS: We obtained clinical information and metabolic images of 56 HH patients and utilized ictal semiology evaluation to stratify the specimens into GS-only, GS-plus, and no-GS subgroups and then applied contrasted trajectories inference (cTI) to calculate the pseudotime value and evaluate GS progression. Ordinal logistic regression was performed to identify neuroimaging-clinical predictors of GS, and then voxelwise lesion network-symptom mapping (LNSM) was applied to explore GS-associated brain regions. RESULTS: cTI inferred the specific metabolism trajectories of GS progression and revealed increased complexity from GS to other seizure types. This was further validated via actual disease duration (Pearson R = 0.532, P = 0.028). Male sex [odds ratio (OR) = 2.611, P = 0.013], low age at seizure onset (OR = 0.361, P = 0.005), high normalized HH metabolism (OR = - 1.971, P = 0.037) and severe seizure burden (OR = - 0.006, P = 0.032) were significant neuroimaging clinical predictors. LNSM revealed that the dysfunctional cortico-subcortico-cerebellar network of GS and the somatosensory cortex (S1) represented a negative correlation. CONCLUSIONS: This study sheds light on the clinical characteristics and progression of GS in children with HH. We identified distinct subtypes of GS and demonstrated the involvement of specific brain regions at the cortical-subcortical-cerebellar level. These valuable results contribute to our understanding of the neural correlates of GS.

The localization and lateralization of fear aura and its surgical prognostic value in patients with focal epilepsy.

OBJECTIVE: Fear aura has traditionally been considered relevant to epileptic discharges from mesial temporal areas, and few studies have investigated its effect on surgical outcome in drug-resistant epilepsy. We aim to assess the localizing and lateralizing value as well as prognostic significance of fear aura in patients with focal epilepsy. METHODS: The occurrence of fear aura in relation to epileptogenic origin and its association with postoperative outcome were analyzed in 146 consecutive patients undergoing resective surgery for intractable epilepsy. RESULTS: Ninety-four (64.4%) patients reported auras, and 31 (21.2%) reported fear aura in their seizures. One hundred ten (75.3%) patients had an Engel class I outcome until last follow-up, of whom 24 experienced fear aura preoperatively. Fear aura appeared more frequently during temporal and frontal lobe seizures, but did not lateralize the seizure onset zone. There were no significant baseline differences between patients with and without fear aura. No correlation was found between postoperative outcome and the presence of auras. Occurrence of fear aura failed to show predictive value in surgical outcome whether in pooled or subgroup analysis. INTERPRETATION: This study advances our understanding of the origin of fear aura, and is helpful for presurgical evaluation and outcome prediction. Without lateralizing value, fear aura is more commonly seen with temporal or frontal origin. When taken as a whole, auras do not have a significant impact on seizure outcome in focal epilepsy. Patients with fear aura are no more likely to become seizure-free than those without fear aura.

Interictal pattern on scalp electroencephalogram predicts excellent surgical outcome of epilepsy caused by focal cortical dysplasia.

OBJECTIVE: Focal cortical dysplasia (FCD) represents an essential cause of drug-resistant epilepsy with surgery as an effective treatment option. This study aimed to identify the important predictors of favorable surgical outcomes and the impact of the interictal scalp electroencephalogram (EEG) patterns in predicting postsurgical seizure outcomes. METHODS: We retrospectively evaluated 210 consecutive patients between 2015 and 2019. They were diagnosed with FCD by pathology, underwent resection, and had at least one year of postsurgical follow-up. Predictors of seizure freedom were analyzed. RESULTS: Based on the information at the latest follow-up, seizure outcome was classified as Engel Class I (seizure-free) in 81.4% and Engel Class II-IV (non-seizure-free) in 18.6% of patients. There were 43, 105, and 62 cases of FCD type I, type II, and type III, respectively. The interictal EEG showed a repetitive discharge pattern (REDP) in 87 (41.4%) patients, polyspike discharge pattern (PDP) in 41 (19.5%), and the coexistence of REDP and PDP in the same location in 32 (15.2%) patients. The analyzed patterns in order of frequency were repetitive discharges lasting 5 seconds or more (32.4%); polyspikes (16.7%); RED type 1 (11.4%); continuous epileptiform discharges occupying >80% of the recording (11.4%); RED type 2 (6.2%); brushes (3.3%); focal, fast, continuous spikes (2.4%); focal fast rhythmic epileptiform discharges (1.43%); and frequent rhythmic bursting epileptiform activity (1.4%). The coexistence of REDP and PDP in the same location on scalp EEG and complete resection of the assumed epileptogenic zone (EZ) was independently associated with favorable postsurgical prognosis. SIGNIFICANCE: Resective epilepsy surgery for intractable epilepsy caused by FCD has favorable outcomes. Interictal scalp EEG patterns were revealed to be predictive of excellent surgical outcomes and may help clinical decision-making and enable better presurgical evaluation.

Voxel-Based Morphometric MRI Postprocessing-Assisted Laser Interstitial Thermal Therapy for Focal Cortical Dysplasia-Suspected Lesions: Technique and Outcomes.

BACKGROUND: MRI-guided laser interstitial thermal therapy (MRgLITT) is a novel treatment modality for focal cortical dysplasia (FCD). However, identifying the location and extent of subtle FCD by visual analysis during MRgLITT remains challenging. OBJECTIVE: To introduce voxel-based morphometric MRI postprocessing into the procedure of MRgLITT for FCD-suspected lesions and assess the complementary value of the MRI postprocessing technique for the trajectory design and thermal parameter setting of MRgLITT. METHODS: Junction and normalized fluid-attenuated inversion recovery signal intensity images were used to detect the gray-white matter junction blurring and cortical fluid-attenuated inversion recovery hyperintensity, respectively. According to the 2 postprocessing images, the region of interest (ROI) for ablation was drawn. The main principle of presurgical planning is that the trajectory of the laser fiber was designed as far as possible along the long axis of the ROI while the extent of planned ablation covered the entire ROI. The subsequent intraoperative procedure was performed under the guidance of the presurgical plan. RESULTS: Nine patients with epilepsy with FCD-suspected lesions underwent MRgLITT with the assistance of MRI postprocessing images. Among them, 4 patients were junction positive, 2 patients were normalized fluid-attenuated inversion recovery signal intensity positive, and the remaining 3 patients were positive for both. Postsurgical MRI demonstrated that the ROIs were ablated entirely in 7 patients. Engel Ia, Ib, and IV scores were obtained at 1-year follow-up for 6, 1, and 2 patients, respectively. CONCLUSION: MRI postprocessing provides complementary information for designing the laser fiber trajectory and subsequent ablation for FCDs.

A polymorphism in CALHM1 is associated with temporal lobe epilepsy.

A recent study suggests that the P86L polymorphism (rs2986017) in the calcium homeostasis modulator 1 (CALHM1) gene interferes with calcium homeostasis and increases amyloid ß (Aß) levels. Moreover, in vitro and in vivo data show that both calcium homeostasis and high levels of Aß play an important role in the induction and maintenance of epileptic seizures in hippocampus, indicating CALHM1 might play a potential role in pathophysiological pathways involved in temporal lobe epilepsy (TLE). The aim of this study was to investigate the genetic contribution of CALHM1 to TLE. Five single-nucleotide polymorphisms (SNPs) of CALHM1 were selected and genotyped using polymerase chain reaction restriction fragment length polymorphism in 560 patients with TLE and 401 healthy controls. We found a positive association between rs11191692 and TLE, but a negative result between rs2986017 and TLE. The rs11191692-A allele frequency was found in 32.4% of the patients and in 26.2% of control subjects (OR=1.35, 95% CI=1.10-1.65, uncorrected P=0.003, corrected P=0.015). Furthermore, the positive association between rs11191692 and TLE independent of apolipoprotein E ε4 was supported by five SNPs haplotype analysis. The results of this study provide the first evidence that the SNP rs11191692 in CALHM1 confers highly increased susceptibility to TLE.

Recognition of seizure semiology and semiquantitative FDG-PET analysis of anti-LGI1 encephalitis.

AIMS: Anti-leucine-rich glioma-inactivated 1 (LGI1) autoimmune encephalitis (AE) is characterized by complex manifestations of seizures. Here, we report a new seizure semiology, attempt to classify the disease by semiology type, and explore the metabolic pattern of each group. METHODS: Anti-LGI1 AE patients were retrospectively screened between May 2014 and September 2019 in our tertiary epilepsy center. All enrolled patients had seizures during long-range video electroencephalogram (EEG) recordings, and all patients (except one) underwent [18 F] fluoro-2-deoxyglucose (FDG) positron emission tomography (PET) scans. Voxel-based metabolic analysis and z-distribution analysis were carried out to determine the metabolic pattern. RESULTS: Thirty-three patients were enrolled. According to the patients' seizure semiology, we divided the patients into four groups: focal impaired awareness seizures (FIAS, n = 17), faciobrachial dystonic seizures (FBDS)-only (n = 6), FBDS-plus (n = 8), and focal aware motor seizures (FAMS) (n = 2). No significant differences were found in the clinical manifestations or accessory tests except for the onset age (FIAS < FBDS-plus) and seizure semiology. This was the first study to extensively describe the clinical manifestations and EEG of FAMS in anti-LGI1 AE patients. In addition, we found that the patients with different semiologies all showed a wide range of abnormal metabolism, which is not limited to the temporal regions and basal ganglia, and extends far beyond our previous interpretation of FDG-PET data. CONCLUSION: Our results showed that FAMS can serve as a rare indicative seizure semiology of anti-LGI1 AE and that individuals with this disease exhibited widespread functional network alterations.