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Autoimmune limbic encephalitis (ALE) represents a heterogeneous disease associated with antibodies targeting extracellular (ALE extra ) epitopes, intracellular (ALE intra ) epitopes, anti-glutamic acid decarboxylase65 ALE (ALE GAD65 ), and ALE without detectable antibodies (ALE abneg ). Combining analysis of cellular parameters, investigated by flow cytometry, and soluble parameters in the blood and cerebrospinal fluid (CSF) from a large cohort of 148 ALE patients (33 ALE extra , 12 ALE intra , 28 ALE-GAD65, 37 ALE abneg ) in comparison to paradigmatic examples for neuro-inflammatory (51 relapsing remitting MS patients (RRMS)), and neuro-degenerative (34 Alzheimer's disease patients (AD)) diseases revealed discrete immune signatures in ALE subgroups. Identification of ALE-subtype specific markers facilitated classification of rare ALE-associated tumors, which may prompt further diagnostic efforts in clinical practice. While ALE intra exhibited features of neuro-inflammation, ALE extra displayed features of neuro-inflammation as well as neuro-degeneration. Moreover, ALE GAD65 and ALE abneg lacked hallmarks of inflammation. This may explain the low efficacy of anti-inflammatory treatment regimens in ALE GAD65 and presumably also ALE abneg ., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: [NMel has received honoraria for lecturing and travel expenses for attending meetings from Biogen Idec, GlaxoSmith Kline, Teva, Novartis Pharma, Bayer Healthcare, Genzyme, Alexion Pharmaceuticals, Fresenius Medical Care, Diamed, UCB Pharma, AngeliniPharma, BIAL and Sanofi-Aventis, has received royalties for consulting from UCB Pharma, Alexion Pharmaceuticals and Sanofi-Aventis and has received financial research support from Euroimmun, Fresenius Medical Care, Diamed, Alexion Pharmaceuticals, and Novartis Pharma. SR received travel grants from Merck Healthcare Germany GmbH, Bristol Myers Squibb, and Alexion Pharmaceuticals. She served on a scientific advisory board from Merck Healthcare Germany GmbH and received honoraria for lecturing from Roche. Her research was supported by Novartis, ‘Stiftung zur Förderung junger Neurowissenschaftler‘, and ‚Else Kröner-Fresenius-Stiftung‘. SGM receives honoraria for lecturing, and travel expenses for attending meetings from Academy 2, Argenx, Alexion, Almirall, Amicus Therapeutics Germany, Bayer Health Care, Biogen, BioNtech, BMS, Celgene, Datamed, Demecan, Desitin, Diamed, Diaplan, DIU Dresden, DPmed, Gen Medicine and Healthcare products, Genzyme, Hexal AG, IGES, Impulze GmbH, Janssen Cilag, KW Medipoint, MedDay Pharmaceuticals, Merck Serono, MICE, Mylan, Neuraxpharm, Neuropoint, Novartis, Novo Nordisk, ONO Pharma, Oxford PharmaGenesis, QuintilesIMS, Roche, Sanofi-Aventis, Springer Medizin Verlag, STADA, Chugai Pharma, Teva, UCB, Viatris, Wings for Life international and Xcenda. His research is funded by the German Ministry for Education and Research (BMBF), Bundesinstitut für Risikobewertung (BfR), Deutsche Forschungsgemeinschaft (DFG), Else Kröner Fresenius Foundation, Gemeinsamer Bundesausschuss (G-BA), German Academic Exchange Service, Hertie Foundation, Interdisciplinary Center for Clinical Studies (IZKF) Muenster, German Foundation Neurology and Alexion, Almirall, Amicus Therapeutics Germany, Biogen, Diamed, DGM e.v., Fresenius Medical Care, Genzyme, Gesellschaft von Freunden und Förderern der Heinrich-Heine-Universität Düsseldorf e.V., HERZ Burgdorf, Merck Serono, Novartis, ONO Pharma, Roche, and Teva. SK has received research funding from Biogen and speaker honoraria from UCB, Jazz Pharmaceutical and Eisai.]., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Seizure clusters, prolonged seizures, and status epilepticus are life-threatening neurological emergencies leading to irreversible neuronal damage. Benzodiazepines are current evidence-based rescue therapy options; however, recent investigations indicated the prescription of mainly unsuitable benzodiazepines and inappropriate use of rescue medication., Objective: To examine current use, satisfaction, and adverse events concerning rescue medication in patients with epilepsy in Germany., Patients and Methods: The study was conducted at epilepsy centres in Frankfurt am Main, Greifswald, Marburg, and Münster between 10/2020 and 12/2020. Patients with an epilepsy diagnosis were assessed based on a questionnaire examining a 12-month period., Results: In total, 486 patients (mean age: 40.5, range 18-83, 58.2 % female) participated in this study, of which 125 (25.7 %) reported the use of rescue medication. The most frequently prescribed rescue medications were lorazepam tablets (56.8 %, n = 71 out of 125), buccal midazolam (19.2 %, n = 24), and rectal diazepam (10.4 %, n = 13). Seizures continuing for over several minutes (43.2 %, n = 54), seizure clusters (28.0 %, n = 35), and epileptic auras (28.0 %, n = 35) were named as indications, while 28.0 % (n = 35) stated they administered the rescue medication for every seizure. Of those continuing to have seizures, 46.0 % did not receive rescue medication. On average, rescue medication prescription occurred 7.1 years (SD 12.7, range 0-66) after an epilepsy diagnosis., Conclusions: Unsuitable oral benzodiazepines remain widely prescribed for epilepsy patients as rescue medication. Patients also reported inappropriate use of medication. A substantial proportion of patients who were not seizure-free did not receive rescue medication prescriptions. Offering each patient at risk for prolonged seizures or clusters of seizures an individual rescue treatment with instructions on using it may decrease mortality and morbidity and increase quality of life. ., Competing Interests: Conflict of interest statement M. Maltseva received a grant from the German Research Foundation outside the submitted work. F. v. Podewils has received speaker honoraria from Bial, Eisai, GW Pharmaceutical companies, Angelini Pharma, Zogenix, and UCB Pharma and scientific advisory board honoraria from GW Pharmaceutical companies, UCB Pharma, and Angelini Pharma. L. Habermehl has received support from Jazz Pharma, Zogenix, and Angelini Pharma in the form of reimbursed travelling expenses and training costs. L. Langenbruch reports speaker's honoraria from Eisai, Biogen, and GW Pharmaceuticals. S. Knake reports personal fees from Arvelle Therapeutics, Bial, Desitin Arzneimittel, Eisai, Epilog, UCB Pharma, and Zogenix. F. Rosenow reports personal fees from Angelini Pharma, Desitin Pharma, Eisai GmbH, and UCB Pharma and grants from the Detlev-Wrobel-Fonds for Epilepsy Research, the Deutsche Forschungsgemeinschaft (DFG), the Federal Ministry of Education and Research (BMBF), the LOEWE Programme of the State of Hesse, and the European Union, and from the Dr Senckenbergische Stiftung, the Reiss Stiftung, and the Ernst Max von Grunelius Foundation Frankfurt. L. Kämppi reports speakers’ honoraria from UCB and Eisai, congress/travel support from Angelini Pharma, and personal grants from the Michael Foundation, Finnish Neurology Association, and HUS Neurocenter. C. Mann was supported by the Hessian state to promote female scientists and reports speakers’ honoraria from UCB and travel support from Zogenix outside the submitted work. A. Strzelczyk reports personal fees and grants from Angelini Pharma, Biocodex, Desitin Arzneimittel, Eisai, Jazz Pharmaceuticals, Marinus Pharma, Precisis, Takeda, UCB Pharma, and UNEEG Medical. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Objective: Epilepsy is a chronic condition that can affect patients of all ages. Women with epilepsy (WWE) require access to specific counseling and information regarding issues related to contraception, pregnancy, and hormonal effects on seizure control and bone mineral density. This study investigated the knowledge among WWE regarding their condition, and whether epilepsy-specific knowledge has improved over the last 15 years., Methods: A total of 280 WWE aged 18 to 82 years participated in this multicenter, questionnaire-based study. The study was conducted at four epilepsy centers in Germany, between October 2020 and December 2020. Sociodemographic and epilepsy-specific data for participating women were analyzed and compared with the results of a similar survey performed in 2003-2005 among 365 WWE in Germany., Results: The questionnaire-based survey revealed considerable knowledge deficits without significant improvements over the last 15 years, particularly among those with less education and with regards to information on the more pronounced effects of epilepsy in older WWE (>50 years), including interactions with menopause and osteoporosis. In WWE ≤29 years, a significant increase in the knowledge score was observed in 2020 compared with this age group in 2005 (mean 7.42 vs. 6.5, p = .036). Mothers frequently reported epilepsy-related concerns regarding childrearing, particularly of seizures scaring their child and the need to rely on other people., Conclusion: WWE continue to demonstrate inadequate epilepsy-related knowledge. Despite increasing information availability and the aspiration toward better awareness among medical professionals, overall knowledge has not increased sufficiently compared with the levels observed in recent studies., (Copyright © 2022. Published by Elsevier Ltd.)