PT  - JOURNAL ARTICLE
AU  - Kaaden, Tillman
AU  - Madlener, Marie
AU  - Angstwurm, Klemens
AU  - Bien, Christian G.
AU  - Bogarin, Yuri
AU  - Doppler, Kathrin
AU  - Finke, Alexander
AU  - Gerner, Stefan T.
AU  - Reimann, Gernot
AU  - Häusler, Martin
AU  - Handreka, Robert
AU  - Hellwig, Kerstin
AU  - Kaufmann, Max
AU  - Kellinghaus, Christoph
AU  - Koertvelyessy, Peter
AU  - Kraft, Andrea
AU  - Lewerenz, Jan
AU  - Menge, Til
AU  - Paliantonis, Asterios
AU  - von Podewils, Felix
AU  - Prüss, Harald
AU  - Rauer, Sebastian
AU  - Ringelstein, Marius
AU  - Rostásy, Kevin
AU  - Schirotzek, Ingo
AU  - Schwabe, Julia
AU  - Sokolowski, Piotr
AU  - Suesse, Marie
AU  - Sühs, Kurt-Wolfram
AU  - Surges, Rainer
AU  - Tauber, Simone C.
AU  - Thaler, Franziska
AU  - Bergh, Florian Then
AU  - Urbanek, Christian
AU  - Wandinger, Klaus-P.
AU  - Wildemann, Brigitte
AU  - Mues, Sigrid
AU  - Zettl, Uwe
AU  - Leypoldt, Frank
AU  - Melzer, Nico
AU  - Geis, Christian
AU  - Malter, Michael
AU  - Kunze, Albrecht
AU  - ,
TI  - Seizure Semiology in Antibody-Associated Autoimmune Encephalitis
AID  - 10.1212/NXI.0000000000200034
DP  - 2022 Nov 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e200034
VI  - 9
IP  - 6
4099  - http://nn.neurology.org/content/9/6/e200034.short
4100  - http://nn.neurology.org/content/9/6/e200034.full
SO  - Neurol Neuroimmunol Neuroinflamm2022 Nov 01; 9
AB  - 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 déjà-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.ab=antibody; AE=autoimmune encephalitis; FBDS=faciobrachial dystonic seizures; GAD=glutamic acid decarboxylase; GENERATE=German Network for Research on Autoimmune Encephalitis; ILAE=International League Against Epilepsy; LGI1=leucine-rich glioma-inactivated protein 1; mRS=modified Rankin score; NMDAR=N-methyl-d-aspartate receptor; OR=odds ratio; SE=status epilepticus