RT Journal Article
SR Electronic
T1 Seizure Semiology in Antibody-Associated Autoimmune Encephalitis
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e200034
DO 10.1212/NXI.0000000000200034
VO 9
IS 6
A1 Kaaden, Tillman
A1 Madlener, Marie
A1 Angstwurm, Klemens
A1 Bien, Christian G.
A1 Bogarin, Yuri
A1 Doppler, Kathrin
A1 Finke, Alexander
A1 Gerner, Stefan T.
A1 Reimann, Gernot
A1 Häusler, Martin
A1 Handreka, Robert
A1 Hellwig, Kerstin
A1 Kaufmann, Max
A1 Kellinghaus, Christoph
A1 Koertvelyessy, Peter
A1 Kraft, Andrea
A1 Lewerenz, Jan
A1 Menge, Til
A1 Paliantonis, Asterios
A1 von Podewils, Felix
A1 Prüss, Harald
A1 Rauer, Sebastian
A1 Ringelstein, Marius
A1 Rostásy, Kevin
A1 Schirotzek, Ingo
A1 Schwabe, Julia
A1 Sokolowski, Piotr
A1 Suesse, Marie
A1 Sühs, Kurt-Wolfram
A1 Surges, Rainer
A1 Tauber, Simone C.
A1 Thaler, Franziska
A1 Bergh, Florian Then
A1 Urbanek, Christian
A1 Wandinger, Klaus-P.
A1 Wildemann, Brigitte
A1 Mues, Sigrid
A1 Zettl, Uwe
A1 Leypoldt, Frank
A1 Melzer, Nico
A1 Geis, Christian
A1 Malter, Michael
A1 Kunze, Albrecht
A1 ,
YR 2022
UL http://nn.neurology.org/content/9/6/e200034.abstract
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