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


Book Volume: 9

Journal Issue: 6

DOI: 10.1212/NXI.0000000000200034


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

How to cite


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).


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

BibTeX: Download