Aged laboratory mice and rats are a unique model for better understanding how the young brain differs from the aged brain and how these differences may provide insights into the mechanisms of seizures and epilepsy in older adults. Furthermore, aged rodents represent an untapped resource for discovering novel treatments for late-onset epilepsy. In this short review, I discuss representative preclinical studies to illustrate how aged rodents have enhanced our understanding of the increased susceptibility of the aged brain to seizures, epileptogenesis, and the effects of antiseizure medications.
Background: The transition to adulthood is a vulnerable phase in the development of people and illness-related factors can substantially disrupt the individual developmental chances of people with epilepsy. Treatment concepts for young adults with epilepsy should therefore not only cover medical aspects. Research question: Presentation of problems based on characteristic disease courses, systematic presentation of relevant disease-related aspects in transfer and transition in epilepsy. Targeted illumination of factors that are relevant with respect to the care, adherence and participation of young adults with epilepsy. Material and methods: Summary and discussion of original and review articles as well as expert recommendations. Results: As disease-associated factors in epilepsy can have long-term effects on the individual development, a structured transition to adult medicine is crucial. Various key factors should be taken into account in the treatment concept, including not only the course of the epilepsy syndrome and the effect of seizure suppressants but also social aspects and individual life planning. Discussion: The individual treatment concept for young adults with epilepsy should take both medical and nonmedical aspects into account. Optimal communication between healthcare providers and high treatment adherence increase the chances of a favorable disease progression and optimal psychosocial development.
Epilepsien beginnen oft früh im Leben und können eine erhebliche Belastung für die betroffenen Kinder und ihre Familien darstellen. Darstellung der Epilepsiesyndrome, die typischerweise in den ersten 2 Lebensjahren beginnen. Der Fokus liegt auf Klassifikation und Definition der Epilepsiesyndrome. Grundsätzlich erfolgt die Einteilung der Epilepsiesyndrome mit Beginn in den ersten 2 Lebensjahren in 2 große Gruppen: Zum einen die selbstlimitierenden Epilepsien mit einer großen Chance für eine spontane Remission und guter Prognose, zum anderen die entwicklungsbedingten und epileptischen Enzephalopathien. Bei Letzteren kommt es sowohl aufgrund der Ätiologie unabhängig von der epileptischen Aktivität als auch als Folge der epileptischen Aktivität zu Entwicklungsbeeinträchtigungen. Die meisten Epilepsiesyndrome mit einer spezifischen Ätiologie, die im Neugeborenen- oder Kleinkindalter beginnen, werden den entwicklungsbedingten und epileptischen Enzephalopathien zugeordnet. Die Kenntnis der Klassifikation der Epilepsien mit Beginn in den ersten 2 Lebensjahren ermöglicht eine individualisierte Beratung und Behandlung und hilft, den Bedürfnissen von jungen Kindern mit Epilepsie und ihren Familien besser gerecht zu werden.
Epilepsy surgery in early childhood represents a time-critical intervention for selected children with drug-resistant epilepsy, in whom ongoing epileptic activity may disrupt neuronal networks and brain development. In this age group, surgical decision-making differs from that in older children and adults, as indications, risks, and outcomes are closely intertwined with neurodevelopmental vulnerability and plasticity. This narrative review provides a clinically oriented overview of epilepsy surgery in children aged 3 years and younger. We discuss age-specific considerations regarding drug resistance, etiological spectrum, presurgical evaluation, and surgical strategies, with particular emphasis on the rationale for early intervention. Available data indicate that epilepsy surgery in early childhood can achieve durable seizure control in a substantial proportion of carefully selected patients, particularly when performed in specialized centers. Surgery may stabilize rather than reverse developmental impairment. Persistent seizures, widespread or evolving epileptogenic networks, and diffuse underlying pathological changes remain key determinants of suboptimal outcome. Ethical considerations and shared decision-making are integral to early epilepsy surgery, requiring transparent communication of findings, options, risks, and uncertainty as well as a careful balancing of irreversible surgical effects against the ongoing harm of uncontrolled epilepsy. Early referral and individualized, multidisciplinary evaluation are essential to optimize outcomes in this vulnerable population.
An epilepsy syndrome is defined by a characteristic combination of clinical features, including seizure type(s), ictal and interictal EEG patterns, age at onset, clinical history, and neurological findings, often supported by specific structural, genetic, metabolic, immune, or infectious etiologies. This article describes the most common seizure types in early childhood epilepsy syndromes and how they differ from one another. In addition, the most common and relevant epilepsy syndromes in early childhood are discussed in terms of their electroclinical characteristics. The diagnosis of an epilepsy syndrome in this age group frequently carries prognostic and treatment implications. Therefore, knowledge of the electroclinical phenotype and its differentiation from other types is important.
In diesem Artikel werden monogene Ursachen von Epilepsiesyndromen betrachtet, die typischerweise vor dem zweiten Geburtstag beginnen. Der Artikel orientiert sich an der Veröffentlichung der ILAE (International League against Epilepsy) zu Klassifikation und Definition der Epilepsiesyndrome mit Beginn bei Neugeborenen und Kleinkindern aus dem Jahr 2022. Hierbei bezieht sich der Artikel auf die entwicklungsbedingten und epileptischen Enzephalopathien („developmental and epileptic encephalopathies“, DEE), die selbstlimitierenden Epilepsien im frühen Kindesalter sowie eine Auswahl an spezifischen Epilepsiesyndromen. Empfehlungen zur genetischen Diagnostik bei frühkindlicher Epilepsie und Epilepsiesydromen sind dem kürzlich veröffentlichten „Update zu Empfehlungen des Experten-Panels Epilepsie und Genetik der Deutschen Gesellschaft für Epileptologie“ (ZEPI-D-25-00043) zu entnehmen.
In der Entwicklung des Menschen stellt der Übergang in das Erwachsenenalter eine vulnerable Phase dar, krankheitsbedingte Faktoren können dabei die individuellen Entwicklungschancen von Menschen mit Epilepsie maßgeblich stören. Therapiekonzepte bei jungen Erwachsenen mit Epilepsie sollten daher nicht nur medizinische Aspekte umfassen. Problemdarstellung anhand charakteristischer Krankheitsverläufe, systematische Darstellung relevanter krankheitsbezogener Aspekte in Transfer und Transition bei Epilepsie. Gezielte Beleuchtung von Faktoren, die im Hinblick auf Versorgung, Adhärenz und Teilhabe junger Erwachsener mit Epilepsie relevant sind. Zusammenfassung und Diskussion von Original- und Übersichtsarbeiten sowie Expertenempfehlungen. Da krankheitsassoziierte Faktoren bei Epilepsie langfristige Auswirkungen auf die individuelle Entwicklung haben können, ist ein strukturierter Übergang in die Erwachsenenmedizin von entscheidender Bedeutung. Dabei sollten verschiedene Schlüsselfaktoren im therapeutischen Konzept berücksichtigt werden, die neben dem Verlauf des Epilepsiesyndroms und der Wirkung der Anfallssuppressiva auch soziale Aspekte und individuelle Lebensplanungen umfassen. Das individuelle Behandlungskonzept bei jungen Erwachsenen mit Epilepsie sollte medizinische und auch nichtmedizinische Aspekte berücksichtigen. Ein optimaler Austausch unter Gesundheitsdienstleistern und eine hohe Therapieadhärenz steigern die Chance auf einen guten Krankheitsverlauf und eine optimale psychosoziale Entwicklung.
Some 200 years ago, in 1825, French doctors first described their impressions of hippocampal alterations in epilepsy during autopsies. However, recognizing the fundamental role the mesial lobe would play in epileptology did not occur until many decades to follow. Following a gap in the scientific literature, Theodor Meynert in 1867 raised the topic of Ammon’s horn sclerosis (AHS) again, and a body of studies followed, many of them in the German-speaking literature. Some studies remain influential to the present day, such as the contribution by Sommer (1880), whose name is linked to sector CA1 of Ammon’s horn, or the work of Bratz (1899), who included the first detailed histological illustration of AHS. A row of early studies on AHS reflects the growing body of scientific evidence on the role of hippocampal pathology in a subtype of epilepsy, which was enigmatic in the past but today is well-known as temporal lobe epilepsy. Here, we present, illustrate, and discuss important early studies on AHS between 1825 and the turn of the century.
The TSC Center Initiative was launched by the self-help organization Tuber & ouml;se Sklerose Deutschland e. V. (Tuberous Sclerosis Germany, TSDEV). The TSC centers have proven to be well-utilized high-performance medical facilities for patients with tuberous sclerosis. The TSC centers are certified by TSDEV according to established rules. The TSC centers are maintained by various providers with different care models. Financial compensation for outpatient services in TSC centers is often insufficient.
Background Drug-resistant focal epilepsy can successfully be treated by surgical elimination of the epileptogenic brain area. Magnetoencephalography (MEG) can contribute to localizing the epileptogenic zone, providing complementary information to electroencephalography (EEG). In recent years, optically pumped magnetometers (OPMs) have been developed as a new type of MEG sensor which promise to improve the signal-to-noise ratio as well as localization accuracy and to reduce operating costs. Objective We aimed at demonstrating the feasibility of OPM-MEG in adults with drug-resistant epilepsy and at confirming the plausibility of the results obtained. Material and methods In this study, three adult volunteers with drug-resistant frontal lobe epilepsy, temporal lobe epilepsy and unclassified epilepsy, respectively, underwent a 45-min MEG scan with 120 OPMs. We averaged the recorded epileptic spikes and used them for MEG source localization. The results were compared with those of EEG source localization and the results of other presurgical diagnostic modalities. Results All test persons tolerated the OPM-MEG measurements without any problems. In all cases, epileptic spikes were recorded and reconstructed through MEG source localization. In all three cases, the results were congruent with those of EEG source localization and other clinical diagnostics. Conclusion The use of OPM-MEG is feasible in adults with pharmacoresistant epilepsy and yields plausible results. As a next step, we are planning a clinical-technical study to determine the diagnostic value of OPM-MEG during presurgical epilepsy evaluation, especially in comparison to EEG and conventional MEG.
We report a retrospective multicenter study on ketogenic diets (KD) administered for at least 3 months in 32 patients under 18 years of age with tuberous sclerosis complex (TSC). The primary endpoint was seizure reduction after 6 months under KD. Secondary endpoints were seizure reduction at other time points, tolerability and retention rate. The KD was administered for a median of 9.2 months (range 3-132 months). After 6 months 27 patients were still on the diet. A relevant reduction of seizure frequency was found in 15 (58%) of 26 analyzable cases and 6 became seizure-free (23%). The efficacy of the KD declined over time. At the end of the observation period the KD had been stopped in 27 patients. The documented reasons were lack of efficacy (6), noncompliance (8), side effects (5) and surgery (3). Mild and/or transient side effects were documented in 8 patients, 5 other patients discontinued KD due to side effects (2 cases of elevated transaminases and 1 case each of pancreatitis, vomiting and kidney stones). Patients in the subgroup with classical KD (N = 19) were significantly younger than those with a modified Atkins diet (N = 13). Efficacy and tolerability did not differ between these subgroups. There was no difference between patients receiving concomitant mTOR inhibition (N = 13) and patients without mTOR inhibition throughout the entire duration of KD (N = 12). KD is an effective and well-tolerated epilepsy therapy in TSC. A combination with mTOR inhibitors is possible. The effectiveness of KD in our cohort is comparable to that generally expected for epilepsy; however, we found no evidence that KD was more effective in patients with TSC than in cohorts with other etiologies.
