Kaaden, Tillman, Madlener, Marie, Angstwurm, Klemens, Bien, Christian G., Bogarin, Yuri, Doppler, Kathrin ORCID: 0000-0003-2883-0009, Finke, Alexander, Gerner, Stefan T., Reimann, Gernot, Haeusler, Martin, Handreka, Robert, Hellwig, Kerstin, Kaufmann, Max ORCID: 0000-0002-1954-4425, Kellinghaus, Christoph, Koertvelyessy, Peter, Kraft, Andrea, Lewerenz, Jan, Menge, Til, Paliantonis, Asterios, von Podewils, Felix, Pruess, Harald, Rauer, Sebastian, Ringelstein, Marius, Rostasy, Kevin, Schirotzek, Ingo, Schwabe, Julia, Sokolowski, Piotr, Suesse, Marie, Suehs, Kurt-Wolfram, Surges, Rainer, Tauber, Simone C., Thaler, Franziska, Bergh, Florian Then, Urbanek, Christian, Wandinger, Klaus-P, Wildemann, Brigitte, Mues, Sigrid, Zettl, Uwe, Leypoldt, Frank, Melzer, Nico ORCID: 0000-0002-2420-701X, Geis, Christian, Malter, Michael ORCID: 0000-0003-2535-4553 and Kunze, Albrecht (2022). Seizure Semiology in Antibody-Associated Autoimmune Encephalitis. Neurol.-Neuroimmunol. Neuroinflammation, 9 (6). PHILADELPHIA: LIPPINCOTT WILLIAMS & WILKINS. 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: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Kaaden, TillmanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Madlener, MarieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Angstwurm, KlemensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bien, Christian G.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bogarin, YuriUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doppler, KathrinUNSPECIFIEDorcid.org/0000-0003-2883-0009UNSPECIFIED
Finke, AlexanderUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gerner, Stefan T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reimann, GernotUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haeusler, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Handreka, RobertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hellwig, KerstinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kaufmann, MaxUNSPECIFIEDorcid.org/0000-0002-1954-4425UNSPECIFIED
Kellinghaus, ChristophUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koertvelyessy, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kraft, AndreaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lewerenz, JanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Menge, TilUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Paliantonis, AsteriosUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Podewils, FelixUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pruess, HaraldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rauer, SebastianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ringelstein, MariusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rostasy, KevinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schirotzek, IngoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schwabe, JuliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sokolowski, PiotrUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Suesse, MarieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Suehs, Kurt-WolframUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Surges, RainerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tauber, Simone C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thaler, FranziskaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bergh, Florian ThenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Urbanek, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wandinger, Klaus-PUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wildemann, BrigitteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mues, SigridUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zettl, UweUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Leypoldt, FrankUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Melzer, NicoUNSPECIFIEDorcid.org/0000-0002-2420-701XUNSPECIFIED
Geis, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Malter, MichaelUNSPECIFIEDorcid.org/0000-0003-2535-4553UNSPECIFIED
Kunze, AlbrechtUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-698615
DOI: 10.1212/NXI.0000000000200034
Journal or Publication Title: Neurol.-Neuroimmunol. Neuroinflammation
Volume: 9
Number: 6
Date: 2022
Publisher: LIPPINCOTT WILLIAMS & WILKINS
Place of Publication: PHILADELPHIA
ISSN: 2332-7812
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
GLUTAMIC-ACID DECARBOXYLASE; FACIOBRACHIAL DYSTONIC SEIZURES; RECEPTOR ANTIBODIES; EPILEPSY; AUTOANTIBODIES; IMMUNOTHERAPYMultiple languages
Clinical Neurology; NeurosciencesMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/69861

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