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Published in: BMC Neurology 1/2022

Open Access 01-12-2022 | Anti-NMDA Receptor Encephalitis | Case report

Anti-NMDAR encephalitis with GFAPα IgG: a case report

Authors: Peng Bai, Bin Liu, Min Li, Mei Chun, Xiangling Ma, Jin Zhen

Published in: BMC Neurology | Issue 1/2022

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Abstract

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.
Literature
1.
go back to reference Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36.CrossRefPubMedPubMedCentral Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36.CrossRefPubMedPubMedCentral
2.
go back to reference Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391–404.CrossRefPubMedPubMedCentral Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391–404.CrossRefPubMedPubMedCentral
3.
go back to reference Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, Aksamit AJ, et al. Autoimmune glial fibrillary acidic protein astrocytopathy: a novel Meningoencephalomyelitis. JAMA Neurol. 2016;73(11):1297–307.CrossRefPubMed Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, Aksamit AJ, et al. Autoimmune glial fibrillary acidic protein astrocytopathy: a novel Meningoencephalomyelitis. JAMA Neurol. 2016;73(11):1297–307.CrossRefPubMed
5.
go back to reference Hacohen Y, Absoud M, Hemingway C, Jacobson L, Lin JP, Pike M, et al. NMDA receptor antibodies associated with distinct white matter syndromes. Neurol Neuroimmunol Neuroinflamm. 2014;1(1):e2.CrossRefPubMedPubMedCentral Hacohen Y, Absoud M, Hemingway C, Jacobson L, Lin JP, Pike M, et al. NMDA receptor antibodies associated with distinct white matter syndromes. Neurol Neuroimmunol Neuroinflamm. 2014;1(1):e2.CrossRefPubMedPubMedCentral
6.
go back to reference Martinez-Hernandez E, Guasp M, García-Serra A, Maudes E, Ariño H, Sepulveda M, et al. Clinical significance of anti-NMDAR concurrent with glial or neuronal surface antibodies. Neurology. 2020;94(22):e2302–10.CrossRefPubMed Martinez-Hernandez E, Guasp M, García-Serra A, Maudes E, Ariño H, Sepulveda M, et al. Clinical significance of anti-NMDAR concurrent with glial or neuronal surface antibodies. Neurology. 2020;94(22):e2302–10.CrossRefPubMed
7.
go back to reference Sarigecili E, Cobanogullari MD, Komur M, Okuyaz C. A rare concurrence: antibodies against myelin oligodendrocyte glycoprotein and N-methyl-d-aspartate receptor in a child. Mult Scler Relat Disord. 2019;28:101–3.CrossRefPubMed Sarigecili E, Cobanogullari MD, Komur M, Okuyaz C. A rare concurrence: antibodies against myelin oligodendrocyte glycoprotein and N-methyl-d-aspartate receptor in a child. Mult Scler Relat Disord. 2019;28:101–3.CrossRefPubMed
8.
go back to reference Flanagan EP, Hinson SR, Lennon VA, Fang B, Aksamit AJ, Morris PP, et al. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients. Ann Neurol. 2017;81(2):298–309.CrossRefPubMed Flanagan EP, Hinson SR, Lennon VA, Fang B, Aksamit AJ, Morris PP, et al. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients. Ann Neurol. 2017;81(2):298–309.CrossRefPubMed
9.
go back to reference Titulaer MJ, Höftberger R, Iizuka T, Leypoldt F, McCracken L, Cellucci T, et al. Overlapping demyelinating syndromes and anti–N-methyl-D-aspartate receptor encephalitis. Ann Neurol. 2014;75(3):411–28.CrossRefPubMedPubMedCentral Titulaer MJ, Höftberger R, Iizuka T, Leypoldt F, McCracken L, Cellucci T, et al. Overlapping demyelinating syndromes and anti–N-methyl-D-aspartate receptor encephalitis. Ann Neurol. 2014;75(3):411–28.CrossRefPubMedPubMedCentral
10.
go back to reference Kimura A, Takekoshi A, Yoshikura N, Hayashi Y, Shimohata T. Clinical characteristics of autoimmune GFAP astrocytopathy. J Neuroimmunol. 2019;332:91–8.CrossRefPubMed Kimura A, Takekoshi A, Yoshikura N, Hayashi Y, Shimohata T. Clinical characteristics of autoimmune GFAP astrocytopathy. J Neuroimmunol. 2019;332:91–8.CrossRefPubMed
11.
go back to reference Long Y, Liang J, Xu H, Huang Q, Yang J, Gao C, et al. Autoimmune glial fibrillary acidic protein astrocytopathy in Chinese patients: a retrospective study. Eur J Neurol. 2018;25(3):477–83.CrossRefPubMed Long Y, Liang J, Xu H, Huang Q, Yang J, Gao C, et al. Autoimmune glial fibrillary acidic protein astrocytopathy in Chinese patients: a retrospective study. Eur J Neurol. 2018;25(3):477–83.CrossRefPubMed
12.
go back to reference Zekeridou A, McKeon A, Flanagan EP. A path to understanding autoimmune GFAP astrocytopathy. Eur J Neurol. 2018;25(3):421–2.CrossRefPubMed Zekeridou A, McKeon A, Flanagan EP. A path to understanding autoimmune GFAP astrocytopathy. Eur J Neurol. 2018;25(3):421–2.CrossRefPubMed
13.
go back to reference Yamakawa M, Hogan KO, Leever J, Jassam YN. Autopsy case of Meningoencephalomyelitis associated with glial fibrillary acidic protein antibody. Neurol Neuroimmunol Neuroinflamm. 2021;8(6):e1081. Yamakawa M, Hogan KO, Leever J, Jassam YN. Autopsy case of Meningoencephalomyelitis associated with glial fibrillary acidic protein antibody. Neurol Neuroimmunol Neuroinflamm. 2021;8(6):e1081.
16.
go back to reference Wang M, Tan J, Zhou Z, Wang Y, Bako SY, Yang Y, et al. Relapsing MOG-IgG-associated diseases coexisting with anti-NMDAR encephalitis: a case report and literature review. J Integr Neurosci. 2022;21(3):82.CrossRefPubMed Wang M, Tan J, Zhou Z, Wang Y, Bako SY, Yang Y, et al. Relapsing MOG-IgG-associated diseases coexisting with anti-NMDAR encephalitis: a case report and literature review. J Integr Neurosci. 2022;21(3):82.CrossRefPubMed
Metadata
Title
Anti-NMDAR encephalitis with GFAPα IgG: a case report
Authors
Peng Bai
Bin Liu
Min Li
Mei Chun
Xiangling Ma
Jin Zhen
Publication date
01-12-2022
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2022
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-022-02961-2

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