Seizure Semiology in Antibody-Associated Autoimmune Encephalitis

Kaaden T, Madlener M, Angstwurm K, Bien CG, Bogarin Y, Doppler K, Finke A, Gerner S, Reimann G, Haeusler M, Handreka R, Hellwig K, Kaufmann M, Kellinghaus C, Koertvelyessy P, Kraft A, Lewerenz J, Menge T, Paliantonis A, Von Podewils F, Pruess H, Rauer S, Ringelstein M, Rostasy K, Schirotzek I, Schwabe J, Sokolowski P, Suesse M, Suehs KW, Surges R, Tauber SC, Thaler F, Bergh FT, Urbanek C, Wandinger KP, Wildemann B, Mues S, Zettl U, Leypoldt F, Melzer N, Geis C, Malter M, Kunze A (2022)

Publication Type: Journal article

Publication year: 2022

Journal

Book Volume: 9

Journal Issue: 6

DOI: 10.1212/NXI.0000000000200034

Abstract

Background and Objectives To assess seizure characteristics in antibody (ab)-associated autoimmune encephalitis (ab + AE) with the 3 most prevalent abs against N-methyl-d-aspartate receptor (NMDAR), leucine-rich glioma-inactivated protein 1 (LGI1), and glutamic acid decarboxylase (GAD). Methods Multicenter nationwide prospective cohort study of the German Network for Research in Autoimmune Encephalitis. Results Three hundred twenty patients with ab + AE were eligible for analysis: 190 NMDAR+, 89 LGI1+, and 41 GAD+. Seizures were present in 113 (60%) NMDAR+, 69 (78%) LGI1+, and 26 (65%) GAD+ patients and as leading symptoms for diagnosis in 53 (28%) NMDAR+, 47 (53%) LGI+, and 20 (49%) GAD+ patients. Bilateral tonic-clonic seizures occurred with almost equal frequency in NMDAR+ (38/51, 75%) and GAD+ (14/20, 70%) patients, while being less common in LGI1+ patients (27/59, 46%). Focal seizures occurred less frequently in NMDAR+ (67/113; 59%) than in LGI1+ (54/69, 78%) or in GAD+ patients (23/26; 88%). An aura with deja-vu phenomenon was nearly specific in GAD+ patients (16/20, 80%). Faciobrachial dystonic seizures (FBDS) were uniquely observed in LGI1+ patients (17/59, 29%). Status epilepticus was reported in one-third of NMDAR+ patients, but only rarely in the 2 other groups. The occurrence of seizures was associated with higher disease severity only in NMDAR+ patients. Discussion Seizures are a frequent and diagnostically relevant symptom of ab + AE. Whereas NMDAR+ patients had few localizing semiological features, semiology in LGI1+ and GAD+ patients pointed toward a predominant temporal seizure onset. FBDS are pathognomonic for LGI1 + AE. Status epilepticus seems to be more frequent in NMDAR + AE.

Authors with CRIS profile

Involved external institutions

Universitätsklinikum Jena Germany (DE) Universität zu Köln Germany (DE) Universität Regensburg Germany (DE) Universität Bielefeld Germany (DE) Asklepios Kliniken Germany (DE) Julius-Maximilians-Universität Würzburg Germany (DE) Städtisches Klinikum Lüneburg Germany (DE) Klinikum Dortmund Germany (DE) Universitätsklinikum Aachen (UKA) Germany (DE) Carl-Thiem-Klinikum Germany (DE) Ruhr-Universität Bochum (RUB) Germany (DE) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) Klinikum Osnabrück Germany (DE) Otto-von-Guericke-Universität Magdeburg Germany (DE) Krankenhaus Martha-Maria Halle-Dölau Germany (DE) Universität Ulm Germany (DE) Heinrich-Heine-Universität Düsseldorf Germany (DE) Hirslanden AG / Privatklinikgruppe Hirslanden Switzerland (CH) Universität Greifswald Germany (DE) Charité - Universitätsmedizin Berlin Germany (DE) Albert-Ludwigs-Universität Freiburg Germany (DE) Vestische Kinder- und Jugendklinik Datteln Germany (DE) Universitätsklinikum Gießen und Marburg (UKGM) Germany (DE) Universitätsklinikum Bonn Germany (DE) Ludwig-Maximilians-Universität (LMU) Germany (DE) Fachkrankenhaus Hubertusburg Germany (DE) Medizinische Hochschule Hannover (MHH) / Hannover Medical School Germany (DE) Universität Leipzig Germany (DE) Klinikum der Stadt Ludwigshafen am Rhein gGmbH Germany (DE) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) Ruprecht-Karls-Universität Heidelberg Germany (DE) Technische Universität Dresden Germany (DE) Universität Rostock Germany (DE) Christian-Albrechts-Universität zu Kiel Germany (DE)

How to cite

APA:

Kaaden, T., Madlener, M., Angstwurm, K., Bien, C.G., Bogarin, Y., Doppler, K.,... Kunze, A. (2022). Seizure Semiology in Antibody-Associated Autoimmune Encephalitis. Neurology, Neuroimmunology and Neuroinflammation, 9(6). https://doi.org/10.1212/NXI.0000000000200034

MLA:

Kaaden, Tillman, et al. "Seizure Semiology in Antibody-Associated Autoimmune Encephalitis." Neurology, Neuroimmunology and Neuroinflammation 9.6 (2022).

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