Seizure Semiology in Antibody-Associated Autoimmune Encephalitis

被引：25

作者：

Kaaden, Tillman ^{[1
]}

Madlener, Marie ^{[2
,3
]}

Angstwurm, Klemens ^{[4
]}

Bien, Christian G. ^{[5
]}

Bogarin, Yuri ^{[6
]}

Doppler, Kathrin ^{[7
]}

Finke, Alexander ^{[8
]}

Gerner, Stefan T. ^{[9
]}

Reimann, Gernot ^{[10
]}

Haeusler, Martin ^{[11
]}

Handreka, Robert ^{[12
]}

Hellwig, Kerstin ^{[13
]}

Kaufmann, Max ^{[14
]}

Kellinghaus, Christoph ^{[15
]}

Koertvelyessy, Peter ^{[16
]}

Kraft, Andrea ^{[17
]}

Lewerenz, Jan ^{[18
]}

Menge, Til ^{[19
]}

Paliantonis, Asterios ^{[20
]}

von Podewils, Felix ^{[21
]}

Pruess, Harald ^{[22
]}

Rauer, Sebastian ^{[23
]}

Ringelstein, Marius ^{[19
,24
]}

Rostasy, Kevin ^{[25
]}

Schirotzek, Ingo ^{[26
]}

Schwabe, Julia ^{[27
]}

Sokolowski, Piotr ^{[28
]}

Suesse, Marie ^{[21
]}

Suehs, Kurt-Wolfram ^{[29
]}

Surges, Rainer ^{[30
]}

Tauber, Simone C. ^{[31
]}

Thaler, Franziska ^{[32
]}

Bergh, Florian Then ^{[33
]}

Urbanek, Christian ^{[34
]}

Wandinger, Klaus-P ^{[35
]}

Wildemann, Brigitte ^{[36
]}

Mues, Sigrid ^{[37
,38
]}

Zettl, Uwe ^{[39
]}

Leypoldt, Frank ^{[40
]}

Melzer, Nico ^{[23
,41
]}

Geis, Christian ^{[1
]}

Malter, Michael ^{[2
,3
]}

Kunze, Albrecht ^{[1
,42
]}

机构：

[1] Univ Hosp Jena, Hans Berger Dept Neurol, Jena, Germany

[2] Univ Cologne, Fac Med, Dept Neurol, Cologne, Germany

[3] Univ Cologne, Univ Hosp Cologne, Cologne, Germany

[4] Univ Regensburg, Dept Neurol, Regensburg, Germany

[5] Bielefeld Univ, Med Sch, Dept Epileptol Krankenhaus Mara, Campus Bielefeld Bethel, Bielefeld, Germany

[6] Asklepios Hosp Teupitz, Dept Psychiat & Psychotherapy, Teupitz, Germany

[7] Univ Wurzburg, Dept Neurol, Wurzburg, Germany

[8] Hosp Luneburg, Dept Neurol, Luneburg, Germany

[9] Univ Hosp Erlangen, Dept Neurol, Erlangen, Germany

[10] Klinikum Dortmund, Dept Neurol, Dortmund, Germany

[11] RWTH Univ Hosp Aachen, Dept Neuropediat, Aachen, Germany

[12] Carl Thiem Klinikum Cottbus, Dept Neurol, Cottbus, Germany

[13] Univ Bochum, Dept Neurol, Bochum, Germany

[14] Univ Klinikum Hamburg Eppendorf, Zentrum Mol Neurobiol Hamburg, Inst Neuroimmunol & Multiple Sklerose, Hamburg, Germany

[15] Klinikum Osnabruck, Dept Neurol, Osnabruck, Germany

[16] Univ Magdeburg, Dept Neurol, Magdeburg, Germany

[17] Martha Maria Hosp Halle, Dept Neurol, Halle, Germany

[18] Univ Ulm, Dept Neurol, Ulm, Germany

[19] Heinrich Heine Univ Dusseldorf, LVR Klinikum, Ctr Neurol & Neuropsychiat, Dept Neurol, Dusseldorf, Germany

[20] Klinikum Hirslanden, Dept Neurol, Zurich, Switzerland

[21] Univ Greifswald, Dept Neurol, Greifswald, Germany

[22] Charite, Dept Neurol & Expt Neurol, Berlin, Germany

[23] Univ Freiburg, Dept Neurol, Freiburg, Germany

[24] Heinrich Heine Univ Dusseldorf, Med Fac, Dept Neurol, Dusseldorf, Germany

[25] Vest Kinder & Jugendklin Datteln, Dept Neuropediat, Datteln, Germany

[26] Univ Hosp Giessen & Marburg, Dept Neurol, Giessen, Germany

[27] Asklepios Hosp Nord, Dept Neurol, Hamburg, Germany

[28] Hosp Hubertusburg, Dept Neurol, Wermsdorf, Germany

[29] Univ Hosp Hannover, Dept Neurol, Hannover, Germany

[30] Univ Hosp Bonn, Dept Epileptol, Bonn, Germany

[31] RWTH Univ Hosp Aachen, Dept Neurol, Aachen, Germany

[32] Ludwig Maximilians Univ Munchen, Univ Hosp, Inst Clin Neuroimmunol, Munich, Germany

[33] Univ Leipzig, Dept Neurol, Leipzig, Germany

[34] Hosp Ludwigshafen, Dept Neurol, Ludwigshafen, Germany

[35] Univ Hosp Schleswig Holstein, Inst Clin Chem, Kiel, Germany

[36] Heidelberg Univ, Dept Neurol, Heidelberg, Germany

[37] Tech Univ Dresden, Univ Hosp, Dept Neurol, Dresden, Germany

[38] Univ Klinikum Knappschaftskrankenhaus Bochum Lang, Klin Neurol, Bochum, Germany

[39] Univ Rostock, Dept Neurol, Rostock, Germany

[40] Christian Albrechts Univ Kiel, Dept Neurol, Kiel, Germany

[41] Univ Hosp Muenster, Dept Neurol, Inst Translat Neurol, Munster, Germany

[42] Zentralklin Bad Berka, Dept Neurol, Bad Berka, Germany

来源：

NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION | 2022年 / 9卷 / 06期

关键词：

GLUTAMIC-ACID DECARBOXYLASE; FACIOBRACHIAL DYSTONIC SEIZURES; RECEPTOR ANTIBODIES; EPILEPSY; AUTOANTIBODIES; IMMUNOTHERAPY;

D O I：

10.1212/NXI.0000000000200034

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

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