This 78-year-old female patient presented to the outpatient department because of recent myoclonic and tonic-clonic seizures that had occurred more than 1 year ago but did not relapse under newly instigated levetiracetam treatment, despite ongoing epileptiform discharges in the EEG. A routine electroencephalogram (EEG) during the outpatient examination confirmed the generalized spike and wave discharges typical for idiopathic generalized epilepsy. Due to this unexpected constellation an extensive survey of the medical history was undertaken revealing childhood absence epilepsy that had been treated with antiseizure medication until 12 years of age. Since then the patient had remained seizure-free for 70 years even without medication; however, several EEG updates during that time for different but not epilepsy-related reasons repeatedly confirmed generalized epileptiform EEG discharges with no seizure relapses. To our best knowledge this case demonstrates one of the longest seizure-free intervals in a case of idiopathic generalized epilepsy prior to a seizure relapse ever reported in the literature.

Eine 78-jährige Patientin stellte sich wegen kürzlich aufgetretener myoklonischer und tonisch-klonischer Anfälle ambulant vor, die zwar mehr als ein Jahr zuvor aufgetreten waren und sich unter einer neu begonnenen Levetiracetam-Therapie nicht wiederholt hatten, bei der aber bei EEG(Elektroenzephalogramm)-Kontrollen noch epilepsietypische Potenziale aufgefallen waren. Ein Routine-EEG im Rahmen unserer ambulanten Untersuchung bestätigte generalisierte Spike-and-Wave-Entladungen, typisch für eine idiopathische generalisierte Epilepsie. Aufgrund dieser unerwarteten Konstellation wurde die Anamnese nochmals sehr gründlich geführt. Es ergab sich, dass in der frühen Kindheit eine Absencenepilepsie bestanden hatte und bis zum 12. Lebensjahr medikamentös behandelt worden war. Seitdem blieb die Patientin über 70 Jahre auch ohne Medikation anfallsfrei. Mehrere EEG-Kontrollen in dieser Zeit aus anderer neurologischer Indikation heraus hatten jedoch immer wieder generalisierte epilepsietypische Muster gezeigt, ohne dass es zu Rückfällen kam. Nach unserem Wissen handelt es sich um eines der längsten dokumentierten anfallsfreien Intervalle bei idiopathischer generalisierter Epilepsie vor einem Rückfall.

IntroductionPlanning stereo-electroencephalography (sEEG) for focal drug-resistant epilepsy with subtle or absent lesions requires accurate non-invasive spatial information about the hypothetical organization of the epileptic focus. The targeting of individual trajectories for a limited number of invasive depth electrodes is particularly challenging in patients who have undergone prior epilepsy surgery. This study investigated how information from multimodal imaging can guide sEEG planning and enable successful epilepsy surgery in patients with non-lesional focal epilepsy.MethodsWe studied 15 patients who appeared non-lesional on conventional MRI and were suspected to have mono-focal epilepsy. These patients underwent sEEG implantation between October 2019 and October 2022, based on findings from non-invasive video-EEG monitoring and multimodal imaging. Among the participants, four had undergone prior epilepsy surgery, including three who had previously undergone invasive EEG. All patients underwent high-resolution 3 T MRI and MRI morphometry (MAP) as part of their non-invasive presurgical diagnostics. Electric and magnetic source imaging were performed in patient subgroups. sEEG planning incorporated findings from the available imaging methods registered within the stereotactic planning system.ResultsA median of nine sEEG electrodes (range: 7–11) were implanted in each patient, targeting both primary and secondary hypotheses about the epileptic focus location. sEEG recordings revealed a monofocal seizure onset in 12 out of 15 patients, all of whom subsequently underwent epilepsy surgery. No bleeding complications occurred. Of these patients, nine achieved Engel 1 postsurgical outcomes, while three had Engel ≥2 outcomes. Surgery was not performed in three patients due to multifocal epilepsy (n = 2) or an unidentified seizure onset zone (SOZ, n = 1). Concordance across multiple imaging modalities was associated with favorable surgical outcomes.ConclusionIn patients with focal epilepsy and subtle or absent lesions, sophisticated sEEG diagnostics guided by advanced multimodal imaging can successfully identify the seizure onset zone. When focal onset is confirmed and multifocal epilepsy is excluded through sEEG, subsequent epilepsy surgery often results in seizure-free outcomes.

Pharmacotherapy and the desire to have children is a challenging topic that mainly but not exclusively affects women of childbearing age, who make up one third of all female patients with epilepsy. This article presents the current study data, critically examines the resulting recommendations for people with epilepsy and a desire to have children and discusses own experiences in the treatment of women of childbearing age or pregnant women with epilepsy. In the synopsis, the findings on the subject of increased risk of malformations could be largely confirmed and a few new data will change our approach in certain points. An anticipatory selection of antiseizure medication in women of childbearing age, proactive information and pragmatic management of unintended pregnancy should help decrease the risk of teratogenicity and to reduce complications for mother and child during pregnancy.

PurposeBrivaracetam 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.MethodsWe 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.ResultsBetween 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.ConclusionOur 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.

Pharmakotherapie und Kinderwunsch sind ein anspruchsvolles Thema, das hauptsächlich, aber nicht ausschließlich Frauen im gebärfähigen Alter betrifft, die ein Drittel aller Patientinnen mit Epilepsie ausmachen. Im folgenden Beitrag werden aktuelle Studiendaten dargestellt, daraus resultierende Empfehlungen bei Menschen mit Epilepsie und Kinderwunsch kritisch beleuchtet und eigene Erfahrungen in der Behandlung mit Frauen im gebärfähigen Alter bzw. schwangeren Frauen mit Epilepsie diskutiert. In der Zusammenschau können die Erkenntnisse zum Thema erhöhtes Fehlbildungsrisiko weitgehend bestätigt werden, wenige neue Daten werden unsere Vorgehensweise in gewissen Punkten ändern. Vorausschauende Auswahl der anfallssuppressiven Medikamente bei Frauen im gebärfähigen Alter, proaktive Aufklärung und der pragmatische Umgang bei ungeplanter Schwangerschaft sollen dazu beitragen, das Teratogenitätsrisiko zu senken bzw. Komplikationen für Mutter und Kind in der Schwangerschaft zu reduzieren.

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.

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.

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.

Introduction: Video-electroencephalography monitoring (VEM) is the current gold standard for in-hospital epilepsy diagnosis as well as for the diagnostics of many other paroxysmal and recurrent neurological symptoms. Consequently, high-performance VEM systems (VEMS) have become indispensable for performing modern in-hospital differential and presurgical epilepsy diagnostics. As a central diagnostic tool and interface between technology and doctors, VEMS also represent a relevant safety factor in inpatient diagnostics. Recently, complaints about malfunctions of VEMS have been reported repeatedly, some of which hinder the clinical diagnosis of epilepsy patients. Therefore, we investigated the reliability of, customer satisfaction with and potential for improvement of the VEMS used in German epilepsy centres. Materials and Methods: Between December 2020 and January 2021, a survey was conducted among German epilepsy centres. Using a short questionnaire including established business to customer metrics, such as the Net Promoter Score, the Customer Satisfaction Score, the Customer Effort Score and the Things Gone Wrong Metric, the reliability, customer satisfaction and potential for general or specific improvements of VEMS were accessed without aiming to compare individual manufacturers or systems in detail. Results: A total of 16 of 27 contacted epilepsy centres (59%) participated in the survey. Customer satisfaction with VEMS was low with only 13 % of clients that would actively recommend their system to other clinicians or epileptologists. Only 50 % of users were satisfied with the overall performance of their VEMS, and only 18 % were satisfied with the manufacturer's after-sales service. Major software or hardware problems were reported in the diagnostic of approximately every 10th patient. Common problem areas mentioned were the VEMS user interface, the stability of software, a shortage of regular updates and a lack of customer-oriented improvements. The greatest potential for improvement was found in software and hardware stability as well as in customer service. Conclusion: As far as it can reasonably be assessed from this German multicenter survey, the level of satisfaction among German epilepsy centres regarding their VEMS is soberingly low. This unsatisfaction seems to be mainly based on frequent and sometimes serious malfunctions of hardware and software on the one hand and a subjective dissatisfaction with the manufacturer’s aftermarket customer service on the other hand. The fact that on average the diagnosis of every 10th patient is hampered by hardware or software problems should be an alarming sign, regardless of the general satisfaction with VEMS. Besides the obvious potential for improvement, there is an urgent need for national and international functional and safety standards for VEMS.

Photosensitivity is a phenomenon which is defined more precisely by intermittent photic stimulation that elicits a photoparoxysmal response (PPR). Data about the prognosis of people with PPR are scarce. Pure PPR mostly appears in later childhood and adolescence, has a female preponderance and disappears in many people during the third decade. However, whether or not it disappears is mainly dependent whether or not it is associated with an underlying disease with a benign spontaneous course like Rolandic epilepsy, a good therapy prognosis like juvenile myoclonic epilepsy (which is the epilepsy syndrome with the highest proportion of PPR) or a bad prognosis like progressive myoclonic epilepsies. If PPR persists in epilepsy patients stimulus avoidance and suppression is an appropriate additional approach beyond antiepileptic drug treatment. A typical case is reported. Keywords photosensitivity photoparoxysmal response prognosis

OBJECTIVE:To analyze satisfaction with and reliability of video-electroencephalography-monitoring systems (VEMS) in epilepsy diagnostics.METHODS:A survey was conducted between December 2020 and January 2021 among German epilepsy centers using well-established customer satisfaction (CS) and quality assurance metrics.RESULTS:Among 16 participating centers, CS with VEMS was low, with only 13% of customers actively recommending their system. Only 50% of users were satisfied with the overall performance of their VEMS, and a low 18% were satisfied with the manufacturer's customer service. User interface, software stability, lack of regular updates, and missing customer-oriented improvements were reported as frequent problems jeopardizing diagnosis in approximately every 10th patient. The greatest potential for improvement was identified for software and hardware stability as well as customer service.CONCLUSION:Satisfaction with VEMS and their customer service was low, and diagnostics were regularly affected by software or hardware errors. Even if this can be partly explained by the technical complexity of VEMS, there is an urgent need for improvements with regard to the reliability and durability of system components as well as signal synchrony and data management.SIGNIFICANCE:This analysis highlights low consumer satisfaction of users with VEMS and uncovers frequent problems and potential for improvement.

Nationalen und internationalen Empfehlungen zufolge erfordert präoperative Diagnostik und insbesondere Epilepsiechirurgie zuvor den Beleg der Pharmakoresistenz. Allerdings gibt es Befundkonstellationen, in denen Epilepsiechirurgie nicht eine Alternative, sondern wegen der wesentlich besseren Nutzen-Risiko-Relation die Therapie der Wahl darstellt. Anhand einer Kasuistik möchten wir das Dogma der Pharmakoresistenz als Voraussetzung für Epilepsiechirurgie infrage stellen.

We present the case of a young male patient who suffered from a rare type of progressive myoclonic epilepsy. The long diagnostic pathway including the results of somatosensory evoked potentials (SEP), which finally enabled the precise diagnosis to be found and the individual course of treatment are described. We briefly review the underlying Lafora disease and discuss the term giant SEPs and its importance in the context of this disease.

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.

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.

With an elimination half-life of 105 hours, perampanel (PER) allows a once-daily dosing regimen. In pivotal trials, when PER was tapered, it was therefore usually discontinued abruptly. Thus, in our hospital we have always practiced abrupt cessation. In this case series, we investigated how long PER serum concentrations still remain measurable after abrupt discontinuation of PER and whether withdrawal symptoms, such as an increase in seizures or status epilepticus, occur. PER serum levels and the clinical course of 15 adult in-patients were monitored for three weeks based on a retrospective study design following abrupt discontinuation of PER. After one week, PER was still detected in 13 of 15 patients, after two weeks in 10, and after three weeks in three. Neither a severe increase in seizure frequency nor status epilepticus occurred. However, modifications of the concomitant antiseizure drugs were necessary. The abrupt discontinuation of PER leads to a slow decrease in plasma concentration, thus resembling self-evident gradual discontinuation of PER. In some cases, PER may still be measurable and thus clinically active even weeks after its discontinuation. Efficacy and safety of other antiseizure drugs can be estimated appropriately only thereafter.

National and international guidelines inevitably require the proof of antiseizure drug resistance prior to presurgical diagnostics and especially prior to epilepsy surgery; however, there are individual constellations where epilepsy surgery is not an alternative but the first-line treatment if one considers the risk-benefit ratio. By means of a case report we want to discuss and partly question whether the dogma of antiseizure drug resistance as the prerequisite of epilepsy surgery is always justified.