Seizure Semiology in Antibody-Associated Autoimmune Encephalitis

Kaaden, Tillman and Madlener, Marie and Angstwurm, Klemens and Bien, Christian G. and Bogarin, Yuri and Doppler, Kathrin and Finke, Alexander and Gerner, Stefan T. and Reimann, Gernot and Haeusler, Martin and Handreka, Robert and Hellwig, Kerstin and Kaufmann, Max and Kellinghaus, Christoph and Koertvelyessy, Peter and Kraft, Andrea and Lewerenz, Jan and Menge, Til and Paliantonis, Asterios and von Podewils, Felix and Pruess, Harald and Rauer, Sebastian and Ringelstein, Marius and Rostasy, Kevin and Schirotzek, Ingo and Schwabe, Julia and Sokolowski, Piotr and Suesse, Marie and Suehs, Kurt-Wolfram and Surges, Rainer and Tauber, Simone C. and Thaler, Franziska and Bergh, Florian Then and Urbanek, Christian and Wandinger, Klaus-P and Wildemann, Brigitte and Mues, Sigrid and Zettl, Uwe and Leypoldt, Frank and Melzer, Nico and Geis, Christian and Malter, Michael and Kunze, Albrecht (2022) Seizure Semiology in Antibody-Associated Autoimmune Encephalitis. NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION, 9 (6): e200034. ISSN 2332-7812,

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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.

Item Type: Article
Uncontrolled Keywords: GLUTAMIC-ACID DECARBOXYLASE; FACIOBRACHIAL DYSTONIC SEIZURES; RECEPTOR ANTIBODIES; EPILEPSY; AUTOANTIBODIES; IMMUNOTHERAPY;
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Neurologie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 13 Dec 2023 07:11
Last Modified: 13 Dec 2023 07:11
URI: https://pred.uni-regensburg.de/id/eprint/58793

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