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Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study

Autoimmune encephalitis (AIE) poses a diagnostic challenge due to its heterogeneous clinical presentation, which overlaps with various neurological and psychiatric diseases. During the diagnostic work-up, cerebrospinal fluid (CSF) is routinely obtained, allowing for differential diagnostics as well...

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Autores principales: Zrzavy, Tobias, Höftberger, Romana, Wimmer, Isabella, Berger, Thomas, Rommer, Paulus, Macher, Stefan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8019787/
https://www.ncbi.nlm.nih.gov/pubmed/33828556
http://dx.doi.org/10.3389/fimmu.2021.646940
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author Zrzavy, Tobias
Höftberger, Romana
Wimmer, Isabella
Berger, Thomas
Rommer, Paulus
Macher, Stefan
author_facet Zrzavy, Tobias
Höftberger, Romana
Wimmer, Isabella
Berger, Thomas
Rommer, Paulus
Macher, Stefan
author_sort Zrzavy, Tobias
collection PubMed
description Autoimmune encephalitis (AIE) poses a diagnostic challenge due to its heterogeneous clinical presentation, which overlaps with various neurological and psychiatric diseases. During the diagnostic work-up, cerebrospinal fluid (CSF) is routinely obtained, allowing for differential diagnostics as well as for the determination of antibody subclasses and specificities. In this monocentric cohort study, we describe initial and serial CSF findings of 33 patients diagnosed with antibody-associated AIE (LGI1 (n=8), NMDA (n=7), CASPR2 (n=3), IgLON5 (n=3), AMPAR (n=1), GAD65/67 (n=4), Yo (n=3), Ma-1/2 (n=2), CV2 (n=2)). Routine CSF parameters of 12.1% of AIE patients were in normal ranges, while 60.6% showed elevated protein levels and 45.4% had intrathecal oligoclonal bands (OCBs). Repeated CSF analyses showed a trend towards normalization of initial pathological CSF findings, while relapses were more likely to be associated with increased cell counts and total protein levels. OCB status conversion in anti-NMDARE patients coincided with clinical improvement. In summary, we show that in routine CSF analysis at diagnosis, a considerable number of patients with AIE did not exhibit alteration in the CSF and therefore, diagnosis may be delayed if antibody testing is not performed. Moreover, OCB status in anti-NMDAR AIE patients could represent a potential prognostic biomarker, however further studies are necessary to validate these exploratory findings.
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spelling pubmed-80197872021-04-06 Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study Zrzavy, Tobias Höftberger, Romana Wimmer, Isabella Berger, Thomas Rommer, Paulus Macher, Stefan Front Immunol Immunology Autoimmune encephalitis (AIE) poses a diagnostic challenge due to its heterogeneous clinical presentation, which overlaps with various neurological and psychiatric diseases. During the diagnostic work-up, cerebrospinal fluid (CSF) is routinely obtained, allowing for differential diagnostics as well as for the determination of antibody subclasses and specificities. In this monocentric cohort study, we describe initial and serial CSF findings of 33 patients diagnosed with antibody-associated AIE (LGI1 (n=8), NMDA (n=7), CASPR2 (n=3), IgLON5 (n=3), AMPAR (n=1), GAD65/67 (n=4), Yo (n=3), Ma-1/2 (n=2), CV2 (n=2)). Routine CSF parameters of 12.1% of AIE patients were in normal ranges, while 60.6% showed elevated protein levels and 45.4% had intrathecal oligoclonal bands (OCBs). Repeated CSF analyses showed a trend towards normalization of initial pathological CSF findings, while relapses were more likely to be associated with increased cell counts and total protein levels. OCB status conversion in anti-NMDARE patients coincided with clinical improvement. In summary, we show that in routine CSF analysis at diagnosis, a considerable number of patients with AIE did not exhibit alteration in the CSF and therefore, diagnosis may be delayed if antibody testing is not performed. Moreover, OCB status in anti-NMDAR AIE patients could represent a potential prognostic biomarker, however further studies are necessary to validate these exploratory findings. Frontiers Media S.A. 2021-03-22 /pmc/articles/PMC8019787/ /pubmed/33828556 http://dx.doi.org/10.3389/fimmu.2021.646940 Text en Copyright © 2021 Zrzavy, Höftberger, Wimmer, Berger, Rommer and Macher http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Zrzavy, Tobias
Höftberger, Romana
Wimmer, Isabella
Berger, Thomas
Rommer, Paulus
Macher, Stefan
Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study
title Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study
title_full Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study
title_fullStr Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study
title_full_unstemmed Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study
title_short Longitudinal CSF Findings in Autoimmune Encephalitis—A Monocentric Cohort Study
title_sort longitudinal csf findings in autoimmune encephalitis—a monocentric cohort study
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8019787/
https://www.ncbi.nlm.nih.gov/pubmed/33828556
http://dx.doi.org/10.3389/fimmu.2021.646940
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