Fully automated detection of focal cortical dysplasia: Comparison of MPRAGE and MP2RAGE sequences.

OBJECTIVE: The detection of focal cortical dysplasia (FCD) in magnetic resonance imaging is challenging. Voxel-based morphometric analysis and automated FCD detection using an artificial neural network (ANN) integrated into the Morphometric Analysis Program (MAP18) have been shown to facilitate FCD detection. This study aimed to evaluate whether the detection of FCD can be further improved by feeding this approach with magnetization prepared two rapid acquisition gradient echoes (MP2RAGE) instead of magnetization-prepared rapid acquisition gradient echo (MPRAGE) datasets. METHODS: MPRAGE and MP2RAGE datasets were acquired in a consecutive sample of 32 patients with FCD and postprocessed using MAP18. Visual analysis and, if available, histopathology served as the gold standard for assessing the sensitivity and specificity of FCD detection. Out-of-sample specificity was evaluated in a cohort of 32 healthy controls. RESULTS: The sensitivity and specificity of FCD detection were 82.4% and 62.5% for the MPRAGE and 97.1% and 34.4% for the MP2RAGE sequences, respectively. Median volumes of true-positive voxel clusters were .16 ml for the MPRAGE and .52 ml for the MP2RAGE sequences compared to .08- and .04-ml volumes of false-positive clusters. With regard to cluster volumes, FCD detection was substantially improved for the MP2RAGE data when the estimated optimal threshold of .23 ml was applied (sensitivity = 72.9%, specificity = 83.0%). In contrast, the estimated optimal threshold of .37 ml for the MPRAGE data did not improve FCD lesion detection (sensitivity = 42.9%, specificity = 79.5%). SIGNIFICANCE: In this study, the sensitivity of FCD detection by morphometric analysis and an ANN integrated into MAP18 was higher for MP2RAGE than for MPRAGE sequences. Additional usage of cluster volume information helped to discriminate between true- and false-positive MP2RAGE results.

"Within a minute" detection of focal cortical dysplasia.

PURPOSE: To evaluate a MRI postprocessing tool for the enhanced and rapid detection of focal cortical dysplasia (FCD). METHODS: MP2RAGE sequences of 40 consecutive, so far MRI-negative patients and of 32 healthy controls were morphometrically analyzed to highlight typical FCD features. The resulting morphometric maps served as input for an artificial neural network generating a FCD probability map. The FCD probability map was inversely normalized, co-registered to the MPRAGE2 sequence, and re-transferred into the PACS system. Co-registered images were scrolled through "within a minute" to determine whether a FCD was present or not. RESULTS: Fifteen FCD, three subcortical band heterotopias (SBH), and one periventricular nodular heterotopia were identified. Of those, four FCD and one SBH were only detected by MRI postprocessing while one FCD and one focal polymicrogryia were missed, respectively. False-positive results occurred in 21 patients and 22 healthy controls. However, true positive cluster volumes were significantly larger than volumes of false-positive clusters (p < 0.001). The area under the curve of the receiver operating curve was 0.851 with a cut-off volume of 0.05 ml best indicating a FCD. CONCLUSION: Automated MRI postprocessing and presentation of co-registered output maps in the PACS allowed for rapid (i.e., "within a minute") identification of FCDs in our clinical setting. The presence of false-positive findings currently requires a careful comparison of postprocessing results with conventional MR images but may be reduced in the future using a neural network better adapted to MP2RAGE images.

Do all patients in the epilepsy monitoring unit experience the same level of comfort? A quantitative exploratory secondary analysis.

AIMS: To find out which variables may be associated with comfort of patients in an epilepsy monitoring unit. DESIGN: Exploratory, quantitative study design. METHODS: Data were collected from October 2018 to November 2019 in Austria and Southern Germany. A total of 267 patients of 10 epilepsy centres completed the Epilepsy Monitoring Unit Comfort Questionnaire which is based on Kolcaba's General Comfort Questionnaire. Secondary data analysis were conducted by using descriptive statistics and an exploratory model building approach, including different linear regression models and several sensitivity analyses. RESULTS: Total comfort scores ranged from 83 to 235 points. Gender, occupation and centre turned out to be possible influential variables. On average, women had a total comfort score 4.69 points higher than men, and retired persons 28.2 points higher than high school students ≥18 years. Comfort scores of younger patients were lower than those of older patients. However, age did not show a statistically significant effect. The same could be observed in marital status and educational levels. CONCLUSION: When implementing comfort measures, nurses must be aware of variables which could influence the intervention negatively. Especially, high school students ≥18 years should be supported by epilepsy specialist nurses, in order to reduce uncertainty, anxiety and discomfort. But, since the identified variables account only for a small proportion of the inter-individual variability in comfort scores, further studies are needed to find out additional relevant aspects and to examine centre-specific effects more closely. IMPACT: Nurses ensure patient comfort during a hospital stay. However, there are variables that may impair the effectiveness of the nursing measures. Our study showed that the experience of comfort was highly individual and could be explained by sociodemographic variables only to a limited extent. Nurses must be aware that additional factors, such as the situation in the individual setting, may be relevant.

[SUDEP in brief - knowledge and practice recommendations on sudden unexpected death in epilepsy]. / SUDEP kompakt ­ praxisrelevante Erkenntnisse und Empfehlungen zum plötzlichen, unerwarteten Tod bei Epilepsie.

Sudden unexpected death in epilepsy (SUDEP) is the sudden and unexpected death of an epilepsy patient, which occurs under benign circumstances without evidence of typical causes of death. SUDEP concerns all epilepsy patients. The individual risk depends on the characteristics of the epilepsy and seizures as well as on living conditions. Focal to bilateral and generalized tonic-clonic seizures (TCS), nocturnal seizures and lack of nocturnal supervision increase the risk. Most SUDEP cases are due to a fatal cascade of apnea, hypoxemia and asystole in the aftermath of a TCS. Two thirds of SUDEP cases in unsupervised epilepsy patients with TCS could probably be prevented. Wearables can detect TCS and alert caregivers. SUDEP information is desired by most patients and relatives, has a favorable impact on treatment adherence and behavior and has no negative effects on mood and quality of life.Recommendations of the committee on patient safety of the German Society of Epileptology: the ultimate treatment goal is seizure freedom. If this cannot be achieved, control of TCS should be sought. All epilepsy patients and their relatives should be informed about SUDEP and risk factors. Patients and relatives should be informed about measures to counteract the elevated risk and imminent SUDEP. The counselling should be performed during a face-to-face discussion, at the time of first diagnosis or during follow-up visits. The counselling should be documented. Wearables for TCS detection can be recommended. If TCS persist, therapeutic efforts should be continued. The bereaved should be contacted after a SUDEP.

High-frequency electrical stimulation of the anterior thalamic nuclei increases vigilance in epilepsy patients during relaxed and drowsy wakefulness.

OBJECTIVE: High-frequency deep brain stimulation (DBS) of anterior thalamic nuclei (ANT) reduces the frequency and intensity of focal and focal to bilateral tonic-clonic epileptic seizures. We investigated the impact of high-frequency ANT-DBS on vigilance in epilepsy patients during relaxed and drowsy wakefulness, to better understand the effects and the mechanisms of action of this intervention in humans. METHODS: Four patients with different structural epileptic pathologies were included in this retrospective case-cohort study. Short- and long-term electroencephalography (EEG) was used to determine states of relaxed or drowsy wakefulness and the vigilance changes during stimulation-on and stimulation-off intervals. RESULTS: In relaxed, wakeful patients with eyes closed, the eyelid artifact rate increased acutely and reproducibly during stimulation-on intervals, suggesting an enhanced vigilance. This effect was accompanied by a slight acceleration of the alpha rhythm. In drowsy patients with eyes closed, stimulation generated acutely and reproducibly alpha rhythms, similar to the paradoxical alpha activation during eyes opening. The occurrence of the alpha rhythms reflected an increase in the vigilance of the drowsy subjects during ANT-DBS. SIGNIFICANCE: This is the first demonstration that ANT-DBS increases the vigilance of wakeful epilepsy patients. Our results deliver circumstantial evidence that high-frequency ANT-DBS activates thalamocortical connections that promote wakefulness.

Visual illustration supporting patient-physician communication in epilepsy: A validation and reliability study of seizure images.

Seizure manifestations may be difficult to describe in words alone. Thus, initially, 24 seizure images were developed to support communication and gain assistance during obtaining the patient's history. Before being used in clinical practice, these seizure images must be investigated for validity and reliability. We tested the images with untrained participants including patients with epilepsy, persons who had witnessed seizures, and participants who had neither had nor witnessed epileptic seizures. The participants filled in a questionnaire evaluating the images twice within 3 days. The participants were asked to choose one of the 2 written descriptions that best matched each seizure image. The validity was assessed using one-proportion z-test. The reliability was assessed by Gwet's AC1. The first analysis showed that the proportion of correctly identified seizure images was higher than 70%, except for 2 images representing dystonia and myoclonus. The dystonia image was modified, and the myoclonus image was removed. In the final evaluation, the seizure images were identified with an overall correctness ratio of 96%. The final AC1 of the seizure images was classified as very high. The final 23 seizure images are proved to be valid and have a high agreement that can be used in clinical practice.

A European questionnaire survey on epilepsy monitoring units' current practice for postoperative psychogenic nonepileptic seizures' detection.

BACKGROUND: In cases undergoing epilepsy surgery, postoperative psychogenic nonepileptic seizures (PNES) may be underdiagnosed complicating the assessment of postsurgical seizures' outcome and the clinical management. We conducted a survey to investigate the current practices in the European epilepsy monitoring units (EMUs) and the data that EMUs could provide to retrospectively detect cases with postoperative PNES and to assess the feasibility of a subsequent postoperative PNES research project for cases with postoperative PNES. METHODS: We developed and distributed a questionnaire survey to 57 EMUs. Questions addressed the number of patients undergoing epilepsy surgery, the performance of systematic preoperative and postoperative psychiatric evaluation, the recording of sexual or other abuse, the follow-up period of patients undergoing epilepsy surgery, the performance of video-electroencephalogram (EEG) and postoperative psychiatric assessment in suspected postoperative cases with PNES, the existence of electronic databases to allow extraction of cases with postoperative PNES, the data that these bases could provide, and EMUs' interest to participate in a retrospective postoperative PNES project. RESULTS: Twenty EMUs completed the questionnaire sheet. The number of patients operated every year/per center is 26.7 ( ±â€¯19.1), and systematic preoperative and postoperative psychiatric evaluation is performed in 75% and 50% of the EMUs accordingly. Sexual or other abuse is systematically recorded in one-third of the centers, and the mean follow-up period after epilepsy surgery is 10.5 ±â€¯7.5 years. In suspected postoperative PNES, video-EEG is performed in 85% and psychiatric assessment in 95% of the centers. An electronic database to allow extraction of patients with PNES after epilepsy surgery is used in 75% of the EMUs, and all EMUs that sent the sheet completed expressed their interest to participate in a retrospective postoperative PNES project. CONCLUSION: Postoperative PNES is an underestimated and not well-studied entity. This is a European survey to assess the type of data that the EMUs surgical cohorts could provide to retrospectively detect postoperative PNES. In cases with suspected PNES, most EMUs perform video-EEG and psychiatric assessment, and most EMUs use an electronic database to allow extraction of patients developing PNES.

Is there a place for surgical treatment of nonpharmacoresistant epilepsy?

Epilepsy surgery has been shown to be the best possible treatment in well-defined and difficult-to-treat epilepsy syndromes, such as mesial temporal lobe epilepsy with unilateral hippocampal sclerosis, even early in the course of the disease if pharmacoresistance is proven. This review addresses the question if epilepsy surgery may be justified today even in nonpharmacoresistant cases. There are two possible groups of patients: first, there are epilepsy syndromes with a benign spontaneous course or with a potentially good treatment prognosis under appropriate antiepileptic drug (AED) treatment. Second, there are epilepsies with potentially worse AED treatment prognosis in which appropriate AED treatment has not yet been applied because of the short course of the disease, tolerability problems that prevented usually effective dosing, or adherence issues. In group one, the good spontaneous prognosis or the usually satisfying course under AED treatment in line with the commonly generalized underlying epileptogenesis does not suggest that epilepsy surgery is a realistic alternative, not even in cases with distinct focal clinical and/or electroencephalography (EEG) patterns like in Rolandic epilepsy with centrotemporal spikes. In the second group, the recent International League Against Epilepsy (ILAE) definition should allow assessment of individual pharmacoresistance early after the onset of the disease in order to avoid any delay. Concerns about a potential disease-specific or drug-specific cognitive decline that could be avoided in early surgery are speculative, a matter of controversial discussion, and certainly not relevant, if pharmacoresistance is consequently addressed in time according to the ILAE recommendations. One should also not forget that even in typically pharmacoresistant epilepsy syndromes that are suitable for surgical procedures, satisfying courses do exist that would not require early or any epilepsy surgery. Therefore, in almost any instance, epilepsy surgery as initial treatment or immediately after a first AED is still not recommended although, especially in cases with nonadherence to AEDs, it may be occasionally considered in order to outweigh the risks of ongoing seizures and epilepsy if surgery is not performed.

Hypothalamic hamartoma: Epileptogenesis beyond the lesion?

The discovery of intrinsic epileptogenicity of the hypothalamic hamartoma (HH) marked a new area in understanding the associated clinical syndrome, often manifesting as progressive epileptic encephalopathy. However, therapeutic procedures targeting the HH proved to be inefficient to cure seizures in up to 50% of cases, whereas in cases with partial improvement, the electroclinical patterns of persisting seizures suggest an involvement of distant cortical regions. The concept of kindling-like secondary epileptogenesis has been suggested as a possible underlying mechanism. Yet the role of the hypothalamic lesion in the pathophysiology of the syndrome remains debatable. In the Strasbourg-Kork series, the best outcomes were obtained when the duration of epilepsy before endoscopic HH surgery did not exceed 10 years. In two patients with HH ablation followed at a later time by a temporal lobectomy, only this second surgical step allowed complete seizure freedom. These findings suggest the existence of an independent, third stage of secondary epileptogenesis in human. In the Grenoble series, stereotactic intracerebral recordings (stereo electroencephalography [SEEG]) of five HH cases demonstrated that gelastic/dacrystic seizures were correlated with discharges within the HH, whereas other seizure types were related to discharges affecting cortical regions, which sometimes seemed to be triggered by HH. In the Marseille series, two cases explored by SEEG provided evidence of extended epileptogenicity outside the limits of the HH, forming complex epileptogenic networks, with HH still triggering clusters of neocortical seizures in the first, but not obligatory involved in spontaneous seizures in the second case. Taken together, our data argue for the existence of dynamic ictal network organization, with possible "kindling-like" relationships between the HH and the neocortex or widespread epileptogenesis. Despite the existence of secondary epileptogenesis, the epileptogenic zone could still be limited to the hamartoma, for which early surgical treatment should be pragmatically considered as a first surgical step.

Randomized controlled antiepileptic drug trials miss almost all patients with ongoing seizures.

In spite of the marketing of numerous new antiepileptic drugs (AEDs), their real-life effectiveness has often been disappointing. We therefore retrospectively investigated how many adult patients with drug-resistant epilepsy would have been potential candidates for the last five phase II and III trials that have been performed at our center. Out of a group of 216 consecutively collected patients, only 18 (8.3%) would have been acceptable for recruitment. Treatment with enzyme-inducing AEDs or concomitant medications (47.2%), too few seizures during a baseline period (41.7%), and EEGs showing a pattern not consistent with a diagnosis of focal epilepsy (e.g. generalized spike-wave) (31.5%) were the leading exclusion criteria. If only one criterion prevented recruitment, too few seizures during the baseline period and treatment with enzyme-inducing medications were the most frequent limitations for potential recruitment. Due to the limiting inclusion and exclusion factors of clinical AED trials, only a small fraction of patients with drug-resistant epilepsy is suitable. When new AEDs have passed such trials and are introduced, we have no information about the potential efficacy and tolerability in >90% of our patients with AED-resistant epilepsies. This may be one reason for the disappointing efficacy of many new AEDs after launch.

Current use of imaging and electromagnetic source localization procedures in epilepsy surgery centers across Europe.

OBJECTIVE: In 2014 the European Union-funded E-PILEPSY project was launched to improve awareness of, and accessibility to, epilepsy surgery across Europe. We aimed to investigate the current use of neuroimaging, electromagnetic source localization, and imaging postprocessing procedures in participating centers. METHODS: A survey on the clinical use of imaging, electromagnetic source localization, and postprocessing methods in epilepsy surgery candidates was distributed among the 25 centers of the consortium. A descriptive analysis was performed, and results were compared to existing guidelines and recommendations. RESULTS: Response rate was 96%. Standard epilepsy magnetic resonance imaging (MRI) protocols are acquired at 3 Tesla by 15 centers and at 1.5 Tesla by 9 centers. Three centers perform 3T MRI only if indicated. Twenty-six different MRI sequences were reported. Six centers follow all guideline-recommended MRI sequences with the proposed slice orientation and slice thickness or voxel size. Additional sequences are used by 22 centers. MRI postprocessing methods are used in 16 centers. Interictal positron emission tomography (PET) is available in 22 centers; all using 18F-fluorodeoxyglucose (FDG). Seventeen centers perform PET postprocessing. Single-photon emission computed tomography (SPECT) is used by 19 centers, of which 15 perform postprocessing. Four centers perform neither PET nor SPECT in children. Seven centers apply magnetoencephalography (MEG) source localization, and nine apply electroencephalography (EEG) source localization. Fourteen combinations of inverse methods and volume conduction models are used. SIGNIFICANCE: We report a large variation in the presurgical diagnostic workup among epilepsy surgery centers across Europe. This diversity underscores the need for high-quality systematic reviews, evidence-based recommendations, and harmonization of available diagnostic presurgical methods.

Long-term seizure outcome in 211 patients with focal cortical dysplasia.

OBJECTIVE: Focal cortical dysplasia (FCD) is currently recognized as the most common cause of neocortical pharmacoresistant epilepsy. Epilepsy surgery has become an increasingly successful treatment option. Herein, the largest patient cohort reported to date is analyzed regarding long-term outcome and factors relevant for long-term seizure control. METHODS: Two hundred eleven children and adults undergoing epilepsy surgery for histologically proven FCD and a follow-up period of 2-12 years were analyzed regarding the longitudinal course of seizure control, effects of FCD type, localization, magnetic resonance imaging (MRI), timing of surgery, and postoperative antiepileptic treatment. RESULTS: After 1 year, Engel class I outcome was achieved in 65% of patients and the percentage of seizure-free patients remained stable over the following (up to 12) years. Complete resection of the assumed epileptogenic area, lower age at surgery, and unilobar localization were positive prognostic indicators of long-term seizure freedom. Seizure recurrence was 12% after the first year, whereas 8% achieved late seizure freedom either following additional introduction of antiepileptic drugs (AEDs) (4%), a reoperation (2%), or a running down phenomenon (2%). Thirty-nine percent of patients had a reduction of AED from polytherapy to monotherapy or a complete cessation of AED treatment. Late seizure relapse was seen in nine patients during reduction of AEDs (i.e., in 12% of all patients with AED tapering); in four of them seizures persisted after reestablishment of antiepileptic medication. SIGNIFICANCE: Postoperative long-term seizure outcome was favorable in patients with FCD and remained stable in 80% of patients after the first postoperative year. Several preoperative factors revealed to be predictive for the postoperative outcome and may help in the preoperative counseling of patients with FCD and in the selection of ideal candidates for epilepsy surgery.

First description of pharmacoresistant epilepsy due to independent bilateral hypothalamic hamartomas.

Hypothalamic hamartomas (HHs) are rare developmental malformations consisting of mixed neurons and glial cells, usually unilaterally attached to the tuber cinereum or mammillary bodies. We report on two patients, both suffering from pharmacoresistant epilepsy, behavioural and cognitive disturbances. Ictal and interictal electroencephalographic (EEG) abnormalities appeared bilaterally and multiregionally with right-sided preponderance. Magnetic-resonance imaging (MRI) revealed independent bilateral hypothalamic hamartomas, more prominently on the right side. Endoscopic surgery of the right HH was performed in each patient, resulting in a significant seizure reduction in both cases. To the best of our knowledge, there are no other reports of independent bilateral HHs in the literature.

First clinical experiences with perampanel--the Kork experience in 74 patients.

Perampanel (PER) has been approved for adjunctive treatment of partial-onset seizures in patients age 12 years and older. In Germany, PER was licensed and marketed in September of 2012. At our tertiary referral epilepsy center, a couple of difficult-to-treat patients were awaiting this introduction of PER; therefore, we were able to initiate treatment in many patients within a short period of time. For this report we collected and analyzed the data of the first patients who had been started on add-on PER between September and December of 2012, so that we were able to evaluate at least 6 months of treatment when we made this analysis. At cutoff in June of 2013, 74 patients could be analyzed. Mean age was 38.4 years (range 15-71 years). PER doses ranged from 4 to 14 mg (mean 8.8 mg). All patients took PER once daily at bedtime. Seventy-one patients had focal epileptic seizures; the remaining four patients had Lennox-Gastaut syndrome. Considering the last 3 months of observation compared with baseline, 34 patients (46%) were responders with a reduction of seizure frequency of at least 50%. Ten patients of these (14% of all) were seizure-free. Adverse events were reported in 40 patients (54%). Leading side effects were somnolence (n = 31, 42%) and dizziness (n = 13, 18%), followed by ataxia, irritability, falls, cognitive slowing, and depression in single cases. Six-month retention rate was 70%. Our first clinical experiences with add-on PER in a highly selected group of difficult-to-treat epilepsies are promising.

Psychiatric assessment prior to and after switch from levetiracetam to brivaracetam.

PURPOSE: Brivaracetam is often used as an alternative to levetiracetam in patients with epilepsy (PWE) encountering efficacy issues or adverse events with levetiracetam. This study evaluated the psychological status of PWE who were switched from levetiracetam to brivaracetam due to psychiatric tolerability concerns in comparison to those who remained on levetiracetam. METHODS: We used various psychological assessments including the Symptom Checklist SCL-90-R, the Beck Depression Inventory-II, and the adverse event profile. Eligible participants completed the questionnaires at baseline and again 8 days later. Psychological changes were assessed using standard statistical methods to show differences between a group that immediately switched from levetiracetam to brivaracetam and another group with unchanged levetiracetam. RESULTS: Between May 2020 and May 2021, 63 patients participated in the study, of whom 34 switched from levetiracetam to brivaracetam. At baseline, participants who switched to brivaracetam had fewer antiseizure medications but experienced more monthly seizures. Baseline scores for anxiety (p = 0.020) and psychoticism (p = 0.046) on SCL-90-R in PWE switched to brivaracetam were higher than in the remaining group. In the subsequent assessment, all psychological scores were reduced and were no longer significantly different between both groups. Using multiple regression, initial treatment with a single antiseizure medication and male gender emerged as predictors of psychological improvement. CONCLUSION: Our study found no increased risk of adverse events or psychiatric symptoms after switching from levetiracetam to brivaracetam. Though statistically non-significant, a trend towards improved psychiatric outcomes in the switch group warrants further investigation in future trials with stronger designs for enhanced statistical power.

Is routine electroencephalography (EEG) a useful biomarker for pharmacoresistant epilepsy?

People with seizure disorders who have been treated at the Kork Epilepsy Center over a prolonged time period and who thus provide data concerning the chronic course of epilepsy were investigated in order to address the potential role of electroencephalography (EEG) as a biomarker for pharmacoresistant epilepsy. Clinical course and the corresponding findings from their first recorded EEG, their first EEG following appropriate treatment, and their last EEG were compared. Furthermore, we investigated if interictal epileptiform discharges (IEDs) differ in amplitude and morphology if recorded in long-term seizure-free patients. The early cessation of IEDs was a relatively good marker for a good prognosis, especially in idiopathic generalized epilepsies. However, persistent IEDs had no major impact on the long-term prognosis. We found no differences between IEDs in seizure-free patients or patients with ongoing seizures. Therefore, in our hands, routine EEG was not an appropriate biomarker for the prediction of pharmacoresistant epilepsy. Additional factors such as etiology and pathophysiology also need to be considered.

Hypothalamic hamartoma: is the epileptogenic zone always hypothalamic? Arguments for independent (third stage) secondary epileptogenesis.

Gelastic seizures associated with hypothalamic hamartomas (HHs) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography (EEG) abnormalities, precocious puberty, behavioral disturbances, and progressive cognitive deterioration. Surgery in adults provides seizure freedom in only one third of patients. The poor results of epilepsy surgery could be explained by an extrahypothalamic epileptogenic zone. The existence of an independent, secondary epileptogenic area with persistent seizures after resection of the presumably primary lesion supports the concept of a "hypothalamic plus" epilepsy. "Hypothalamic plus" epilepsy could be related to either an extrahypothalamic structural lesion (visible on magnetic resonance imaging [MRI] or on neuropathology) or if the former is absent, to a functional alteration with enhanced epileptogenic properties due to a process termed secondary epileptogenesis. We report two patients with gelastic seizures with HH (gelastic seizures isolated or associated with dyscognitive seizures of temporal origin). Both patients underwent two-step surgery: first an endoscopic resection of the HH, followed at a later time by temporal lobectomy. Both patients became seizure-free only after the temporal lobectomy. In both cases, neuropathology failed to demonstrate a significant structural lesion in the temporal lobe. To our knowledge, for the first time, these two cases suggest the existence of independent secondary epileptogenesis in humans.

Is there evidence for clinical differences related to the new classification of temporal lobe cortical dysplasia?

PURPOSE: The new International League Against Epilepsy (ILAE) classification for focal cortical dysplasia (FCD) differentiates between patients with isolated FCD (type 1) and FCD with an associated hippocampal sclerosis (HS) (type 3a). In contrast to the former FCD classification by Palmini, which considered only histologic features, the novel ILAE classification also relies on magnetic resonance imaging (MRI) findings and presumed pathogenesis. We investigated in a cohort of 100 patients with exclusively temporal FCD if the new subdivision of FCD is reflected in clinical characteristics. METHODS: Thirty-one patients with FCD type 1 and 50 patients with FCD type 3a in the temporal lobe were included. In all patients MRI and histology of the FCD were available. Both patient groups were compared to 19 patients with temporal FCD type 2 with clearly different histologic appearance. KEY FINDINGS: Patients with FCD type 1 and type 3a presented with similar clinical features in many respects. In univariate analyses, no statistically significant differences were found as to age at epilepsy onset (p = 0.07) and epilepsy surgery (p = 0.14), a normal appearing neocortical temporal lobe (p = 0.08) or diagnosis of FCD by visual inspection of MRI (p = 0.08), preoperative seizure frequency (p = 0.06), and the predominance of an epigastric aura (p = 0.08). The postoperative outcome was nearly identical 1 year (p = 0.8) and 2 (p = 0.8), 3 (p = 0.8), 5 (p = 0.7), and 8 (p = 1.0) years postoperatively. Only febrile seizures (p = 0.025) and an aura (p = 0.03) were significantly more frequently reported in patients with FCD type 3a. Similar results were obtained from a multivariate logistic regression analysis. Patients with FCD type 2 were more different: Compared to FCD type 3a, age at epilepsy surgery was significantly lower (p = 0.004) and auras (p = 0.005) were significantly less frequently reported. Epigastric auras (p = 0.04) and febrile seizures (p = 0.025) occurred significantly less frequently in patients with FCD type 2 without HS compared to FCD type 3a. The diagnosis of an FCD was significantly more frequently made (p = 0.03) by visual inspection of the MRI compared to FCD type 1. SIGNIFICANCE: Clinical features did not allow to clear separation of temporal FCD types 1 and 3a. Statistically significant differences were seen in a history of febrile seizures and the occurrence of auras more common in FCD type 3a. However, FCD type 2 in the same localization but with different histology presented with further differences such as more frequent FCD diagnosis by visual inspection of MRI, earlier operation, and less frequent epigastric auras.

Diagnostic Value of Intermittent Photic Stimulation Among Adult Patients in a Tertiary Referral Epilepsy Center: A Retrospective Study.

PURPOSE: Photosensitivity is a phenomenon that may be elicited by standardized intermittent photic stimulation during EEG recording and is detected more frequently in children and adolescents. Nevertheless, at our Epilepsy Center, we routinely assess photosensitivity in all newly referred adult patients. In this investigation, we sought to address the diagnostic yield under the prerequisites described. METHODS: We reanalyzed all routine EEG recordings among referrals to the department of adults during the first six months of 2019, including a simultaneous video that is always coregistered in our center. The prevalence of abnormal findings during photic stimulation was assessed. RESULTS: Intermittent photic stimulation was performed on 344 patients. Photoparoxysmal response were detected in five subjects (1.5%). All patients were female. Four patients were diagnosed with idiopathic generalized epilepsy, and one with Doose syndrome. Photomyogenic responses were recorded in 1.1% and only in patients with psychogenic nonepileptic seizures. In two subjects with psychogenic nonepileptic seizures, the typical seizure was provoked by intermittent photic stimulation (8.7% of all subjects with psychogenic nonepileptic seizures in this cohort). Photoparoxysmal response was not detected in any subject with focal epilepsy, syncope, or other nonepileptic paroxysmal events. In every case of photoparoxysmal responses, increased photosensitivity had already been reported before recording. CONCLUSIONS: In our study, photoparoxysmal responses was a rare phenomenon among adults with a preponderance of females and idiopathic generalized epilepsies. Intermittent photic stimulation may be helpful in provoking typical psychogenic nonepileptic seizures and thus abbreviate the diagnostic process. Provided a careful history, routine intermittent photic stimulation in adults with epilepsy does not appear to be mandatory.