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Anti-NMDAR encephalitis with GFAPα IgG: a case report

BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an inflammatory disease of the central nervous system (CNS) in which antibodies within the serum and cerebrospinal fluid (CSF) target NMDA receptors. Glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune disease...

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Autores principales: Bai, Peng, Liu, Bin, Li, Min, Chun, Mei, Ma, Xiangling, Zhen, Jin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9652802/
https://www.ncbi.nlm.nih.gov/pubmed/36371153
http://dx.doi.org/10.1186/s12883-022-02961-2
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author Bai, Peng
Liu, Bin
Li, Min
Chun, Mei
Ma, Xiangling
Zhen, Jin
author_facet Bai, Peng
Liu, Bin
Li, Min
Chun, Mei
Ma, Xiangling
Zhen, Jin
author_sort Bai, Peng
collection PubMed
description BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an inflammatory disease of the central nervous system (CNS) in which antibodies within the serum and cerebrospinal fluid (CSF) target NMDA receptors. Glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune disease affecting the central nervous system (CNS). Meningoencephalitis can affect any anatomical region rostrocaudally, from the optic nerve to the spinal cord. The clinical implications of NMDAR antibodies overlapping with other antibodies against glial or neuronal cell surface proteins have not been investigated. CASE PRESENTATION: A 35-year-old male presented with headaches along with amnesia, slurred and awkward speech, psychiatric symptoms, cognitive decline, and insomnia. His medical history revealed ankylosing spondylitis for six months. Ancillary findings included CSF pleocytosis and elevated protein levels. T2-weighted fluid attenuation inversion recovery was used to image high-intensity lesions of the bilateral paraventricular, radiate corona, semioval centre, and right subcortical regions. The CSF was positive for NMDAR and GFAP antibodies through transfected cell-based assays. A diagnosis of anti-GFAP encephalitis was made, although the prominent clinical features were of anti-NMDAR encephalitis. CONCLUSIONS: Herein, we describe a case of anti-NMDAR encephalitis with overlapping symptoms of GFAP antibody positivity. Patients with unusual symptoms of anti-NMDAR encephalitis should also be tested for anti-GFAP antibodies. However, because this was a single case study, caution should be exercised when interpreting the observations. Since the patient was diagnosed with autoimmune encephalitis, intravenous methylprednisolone was administered, which yielded a positive outcome.
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spelling pubmed-96528022022-11-15 Anti-NMDAR encephalitis with GFAPα IgG: a case report Bai, Peng Liu, Bin Li, Min Chun, Mei Ma, Xiangling Zhen, Jin BMC Neurol Case Report BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an inflammatory disease of the central nervous system (CNS) in which antibodies within the serum and cerebrospinal fluid (CSF) target NMDA receptors. Glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune disease affecting the central nervous system (CNS). Meningoencephalitis can affect any anatomical region rostrocaudally, from the optic nerve to the spinal cord. The clinical implications of NMDAR antibodies overlapping with other antibodies against glial or neuronal cell surface proteins have not been investigated. CASE PRESENTATION: A 35-year-old male presented with headaches along with amnesia, slurred and awkward speech, psychiatric symptoms, cognitive decline, and insomnia. His medical history revealed ankylosing spondylitis for six months. Ancillary findings included CSF pleocytosis and elevated protein levels. T2-weighted fluid attenuation inversion recovery was used to image high-intensity lesions of the bilateral paraventricular, radiate corona, semioval centre, and right subcortical regions. The CSF was positive for NMDAR and GFAP antibodies through transfected cell-based assays. A diagnosis of anti-GFAP encephalitis was made, although the prominent clinical features were of anti-NMDAR encephalitis. CONCLUSIONS: Herein, we describe a case of anti-NMDAR encephalitis with overlapping symptoms of GFAP antibody positivity. Patients with unusual symptoms of anti-NMDAR encephalitis should also be tested for anti-GFAP antibodies. However, because this was a single case study, caution should be exercised when interpreting the observations. Since the patient was diagnosed with autoimmune encephalitis, intravenous methylprednisolone was administered, which yielded a positive outcome. BioMed Central 2022-11-12 /pmc/articles/PMC9652802/ /pubmed/36371153 http://dx.doi.org/10.1186/s12883-022-02961-2 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Bai, Peng
Liu, Bin
Li, Min
Chun, Mei
Ma, Xiangling
Zhen, Jin
Anti-NMDAR encephalitis with GFAPα IgG: a case report
title Anti-NMDAR encephalitis with GFAPα IgG: a case report
title_full Anti-NMDAR encephalitis with GFAPα IgG: a case report
title_fullStr Anti-NMDAR encephalitis with GFAPα IgG: a case report
title_full_unstemmed Anti-NMDAR encephalitis with GFAPα IgG: a case report
title_short Anti-NMDAR encephalitis with GFAPα IgG: a case report
title_sort anti-nmdar encephalitis with gfapα igg: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9652802/
https://www.ncbi.nlm.nih.gov/pubmed/36371153
http://dx.doi.org/10.1186/s12883-022-02961-2
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