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CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis

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CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis. / Dürr, Marc; Nissen, Gunnar; Sühs, Kurt-Wolfram; Schwenkenbecher, Philipp; Geis, Christian; Ringelstein, Marius; Hartung, Hans-Peter; Friese, Manuel A; Kaufmann, Max; Malter, Michael P; Madlener, Marie; Thaler, Franziska S; Kümpfel, Tania; Senel, Makbule; Häusler, Martin G; Schneider, Hauke; Bergh, Florian Then; Kellinghaus, Christoph; Zettl, Uwe K; Wandinger, Klaus-Peter; Melzer, Nico; Gross, Catharina C; Lange, Peter; Dreyhaupt, Jens; Tumani, Hayrettin; Leypoldt, Frank; Lewerenz, Jan; German Network for Research on Autoimmune Encephalitis (GENERATE).

in: NEUROL-NEUROIMMUNOL, Jahrgang 8, Nr. 6, 11.2021, S. e1086.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Dürr, M, Nissen, G, Sühs, K-W, Schwenkenbecher, P, Geis, C, Ringelstein, M, Hartung, H-P, Friese, MA, Kaufmann, M, Malter, MP, Madlener, M, Thaler, FS, Kümpfel, T, Senel, M, Häusler, MG, Schneider, H, Bergh, FT, Kellinghaus, C, Zettl, UK, Wandinger, K-P, Melzer, N, Gross, CC, Lange, P, Dreyhaupt, J, Tumani, H, Leypoldt, F, Lewerenz, J & German Network for Research on Autoimmune Encephalitis (GENERATE) 2021, 'CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis', NEUROL-NEUROIMMUNOL, Jg. 8, Nr. 6, S. e1086. https://doi.org/10.1212/NXI.0000000000001086

APA

Dürr, M., Nissen, G., Sühs, K-W., Schwenkenbecher, P., Geis, C., Ringelstein, M., Hartung, H-P., Friese, M. A., Kaufmann, M., Malter, M. P., Madlener, M., Thaler, F. S., Kümpfel, T., Senel, M., Häusler, M. G., Schneider, H., Bergh, F. T., Kellinghaus, C., Zettl, U. K., ... German Network for Research on Autoimmune Encephalitis (GENERATE) (2021). CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis. NEUROL-NEUROIMMUNOL, 8(6), e1086. https://doi.org/10.1212/NXI.0000000000001086

Vancouver

Dürr M, Nissen G, Sühs K-W, Schwenkenbecher P, Geis C, Ringelstein M et al. CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis. NEUROL-NEUROIMMUNOL. 2021 Nov;8(6):e1086. https://doi.org/10.1212/NXI.0000000000001086

Bibtex

@article{130e80d384634e0ca8a91a20d72c015f,
title = "CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis",
abstract = "BACKGROUND AND OBJECTIVES: CSF in antibody-defined autoimmune encephalitis (AE) subtypes shows subtype-dependent degrees of inflammation ranging from rare and often mild to frequent and often robust. AEs with NMDA receptor antibodies (NMDAR-E) and leucine-rich glioma-inactivated protein 1 antibodies (LGI1-E) represent opposite ends of this spectrum: NMDAR-E with typically frequent/robust and LGI1-E with rare/mild CSF inflammation. For a more in-depth analysis, we characterized CSF findings in acute, therapy-naive NMDAR-E and LGI1-E in a multicentric, retrospective, cross-sectional setting.METHODS: Eighty-two patients with NMDAR-E and 36 patients with LGI1-E from the GErman NEtwork for Research of AuToimmune Encephalitis (GENERATE) with lumbar puncture within 90 days of onset and before immunotherapy were included. CSF parameters comprised leukocytes, oligoclonal bands (OCBs), and CSF/serum ratios for albumin, immunoglobulin G (IgG), A (IgA), and M (IgM), the latter 3 converted to Z scores according to Reiber formulas. The MRZ reaction was tested in 14 patients with NMDAR-E and 6 patients with LGI1-E, respectively.RESULTS: CSF was abnormal in 94% of NMDAR-E but only in 36% of LGI1-E patients. Robust quantitative intrathecal immunoglobulin synthesis (IIS, IgG > IgM >> IgA) was characteristic for NMDAR-E, but absent in LGI-E. In NMDAR-E, CSF leukocytes were higher when IIS was present or more pronounced. In addition, in NMDAR-E, CSF leukocytes were lower and IIS occurred less often and if so to a lesser degree at older age. Patients with NMDAR-E with severe functional impairment more often had positive OCBs. In CSF obtained later than 3 weeks of onset, leukocytes were lower. In parallel, the correlation of leukocytes with IIS disappeared as IIS was partially independent of disease duration. The MRZ reaction was positive in 5 (36%) patients with NMDAR-E. All these associations were completely absent in LGI1-E. Here, younger patients showed more blood-CSF barrier dysfunction. In LGI1-E, but not in NMDAR-E, the blood-CSF barrier was more dysfunctional when CSF leukocytes were higher.DISCUSSION: NMDAR-E and LGI-E differ in their typical extent of CSF inflammation. In addition, the patterns formed by the different inflammatory CSF parameters and their relationship with disease severity, age, and disease duration are subtype-characteristic. Moreover, signs for multiple sclerosis-like chronic inflammation are present in a subgroup of patients with NMDAR-E. These CSF patterns might be markers for the different immunopathogeneses of LGI1-E and NMDAR-E.",
author = "Marc D{\"u}rr and Gunnar Nissen and Kurt-Wolfram S{\"u}hs and Philipp Schwenkenbecher and Christian Geis and Marius Ringelstein and Hans-Peter Hartung and Friese, {Manuel A} and Max Kaufmann and Malter, {Michael P} and Marie Madlener and Thaler, {Franziska S} and Tania K{\"u}mpfel and Makbule Senel and H{\"a}usler, {Martin G} and Hauke Schneider and Bergh, {Florian Then} and Christoph Kellinghaus and Zettl, {Uwe K} and Klaus-Peter Wandinger and Nico Melzer and Gross, {Catharina C} and Peter Lange and Jens Dreyhaupt and Hayrettin Tumani and Frank Leypoldt and Jan Lewerenz and {German Network for Research on Autoimmune Encephalitis (GENERATE)}",
note = "Copyright {\textcopyright} 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.",
year = "2021",
month = nov,
doi = "10.1212/NXI.0000000000001086",
language = "English",
volume = "8",
pages = "e1086",
journal = "NEUROL-NEUROIMMUNOL",
issn = "2332-7812",
publisher = "Lippincott Williams and Wilkins",
number = "6",

}

RIS

TY - JOUR

T1 - CSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis

AU - Dürr, Marc

AU - Nissen, Gunnar

AU - Sühs, Kurt-Wolfram

AU - Schwenkenbecher, Philipp

AU - Geis, Christian

AU - Ringelstein, Marius

AU - Hartung, Hans-Peter

AU - Friese, Manuel A

AU - Kaufmann, Max

AU - Malter, Michael P

AU - Madlener, Marie

AU - Thaler, Franziska S

AU - Kümpfel, Tania

AU - Senel, Makbule

AU - Häusler, Martin G

AU - Schneider, Hauke

AU - Bergh, Florian Then

AU - Kellinghaus, Christoph

AU - Zettl, Uwe K

AU - Wandinger, Klaus-Peter

AU - Melzer, Nico

AU - Gross, Catharina C

AU - Lange, Peter

AU - Dreyhaupt, Jens

AU - Tumani, Hayrettin

AU - Leypoldt, Frank

AU - Lewerenz, Jan

AU - German Network for Research on Autoimmune Encephalitis (GENERATE)

N1 - Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

PY - 2021/11

Y1 - 2021/11

N2 - BACKGROUND AND OBJECTIVES: CSF in antibody-defined autoimmune encephalitis (AE) subtypes shows subtype-dependent degrees of inflammation ranging from rare and often mild to frequent and often robust. AEs with NMDA receptor antibodies (NMDAR-E) and leucine-rich glioma-inactivated protein 1 antibodies (LGI1-E) represent opposite ends of this spectrum: NMDAR-E with typically frequent/robust and LGI1-E with rare/mild CSF inflammation. For a more in-depth analysis, we characterized CSF findings in acute, therapy-naive NMDAR-E and LGI1-E in a multicentric, retrospective, cross-sectional setting.METHODS: Eighty-two patients with NMDAR-E and 36 patients with LGI1-E from the GErman NEtwork for Research of AuToimmune Encephalitis (GENERATE) with lumbar puncture within 90 days of onset and before immunotherapy were included. CSF parameters comprised leukocytes, oligoclonal bands (OCBs), and CSF/serum ratios for albumin, immunoglobulin G (IgG), A (IgA), and M (IgM), the latter 3 converted to Z scores according to Reiber formulas. The MRZ reaction was tested in 14 patients with NMDAR-E and 6 patients with LGI1-E, respectively.RESULTS: CSF was abnormal in 94% of NMDAR-E but only in 36% of LGI1-E patients. Robust quantitative intrathecal immunoglobulin synthesis (IIS, IgG > IgM >> IgA) was characteristic for NMDAR-E, but absent in LGI-E. In NMDAR-E, CSF leukocytes were higher when IIS was present or more pronounced. In addition, in NMDAR-E, CSF leukocytes were lower and IIS occurred less often and if so to a lesser degree at older age. Patients with NMDAR-E with severe functional impairment more often had positive OCBs. In CSF obtained later than 3 weeks of onset, leukocytes were lower. In parallel, the correlation of leukocytes with IIS disappeared as IIS was partially independent of disease duration. The MRZ reaction was positive in 5 (36%) patients with NMDAR-E. All these associations were completely absent in LGI1-E. Here, younger patients showed more blood-CSF barrier dysfunction. In LGI1-E, but not in NMDAR-E, the blood-CSF barrier was more dysfunctional when CSF leukocytes were higher.DISCUSSION: NMDAR-E and LGI-E differ in their typical extent of CSF inflammation. In addition, the patterns formed by the different inflammatory CSF parameters and their relationship with disease severity, age, and disease duration are subtype-characteristic. Moreover, signs for multiple sclerosis-like chronic inflammation are present in a subgroup of patients with NMDAR-E. These CSF patterns might be markers for the different immunopathogeneses of LGI1-E and NMDAR-E.

AB - BACKGROUND AND OBJECTIVES: CSF in antibody-defined autoimmune encephalitis (AE) subtypes shows subtype-dependent degrees of inflammation ranging from rare and often mild to frequent and often robust. AEs with NMDA receptor antibodies (NMDAR-E) and leucine-rich glioma-inactivated protein 1 antibodies (LGI1-E) represent opposite ends of this spectrum: NMDAR-E with typically frequent/robust and LGI1-E with rare/mild CSF inflammation. For a more in-depth analysis, we characterized CSF findings in acute, therapy-naive NMDAR-E and LGI1-E in a multicentric, retrospective, cross-sectional setting.METHODS: Eighty-two patients with NMDAR-E and 36 patients with LGI1-E from the GErman NEtwork for Research of AuToimmune Encephalitis (GENERATE) with lumbar puncture within 90 days of onset and before immunotherapy were included. CSF parameters comprised leukocytes, oligoclonal bands (OCBs), and CSF/serum ratios for albumin, immunoglobulin G (IgG), A (IgA), and M (IgM), the latter 3 converted to Z scores according to Reiber formulas. The MRZ reaction was tested in 14 patients with NMDAR-E and 6 patients with LGI1-E, respectively.RESULTS: CSF was abnormal in 94% of NMDAR-E but only in 36% of LGI1-E patients. Robust quantitative intrathecal immunoglobulin synthesis (IIS, IgG > IgM >> IgA) was characteristic for NMDAR-E, but absent in LGI-E. In NMDAR-E, CSF leukocytes were higher when IIS was present or more pronounced. In addition, in NMDAR-E, CSF leukocytes were lower and IIS occurred less often and if so to a lesser degree at older age. Patients with NMDAR-E with severe functional impairment more often had positive OCBs. In CSF obtained later than 3 weeks of onset, leukocytes were lower. In parallel, the correlation of leukocytes with IIS disappeared as IIS was partially independent of disease duration. The MRZ reaction was positive in 5 (36%) patients with NMDAR-E. All these associations were completely absent in LGI1-E. Here, younger patients showed more blood-CSF barrier dysfunction. In LGI1-E, but not in NMDAR-E, the blood-CSF barrier was more dysfunctional when CSF leukocytes were higher.DISCUSSION: NMDAR-E and LGI-E differ in their typical extent of CSF inflammation. In addition, the patterns formed by the different inflammatory CSF parameters and their relationship with disease severity, age, and disease duration are subtype-characteristic. Moreover, signs for multiple sclerosis-like chronic inflammation are present in a subgroup of patients with NMDAR-E. These CSF patterns might be markers for the different immunopathogeneses of LGI1-E and NMDAR-E.

U2 - 10.1212/NXI.0000000000001086

DO - 10.1212/NXI.0000000000001086

M3 - SCORING: Journal article

C2 - 34697224

VL - 8

SP - e1086

JO - NEUROL-NEUROIMMUNOL

JF - NEUROL-NEUROIMMUNOL

SN - 2332-7812

IS - 6

ER -