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Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report

BACKGROUND: Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis is a rare autoimmune synaptic encephalitis associated with autoantibodies that cause a selective decrease in surface expression and changes in receptor localization. Anti-AMPAR encephalitis is poorly...

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Autores principales: Han, Wei, Ma, Jiannan, Jiang, Li, Cheng, Min
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9110937/
https://www.ncbi.nlm.nih.gov/pubmed/35581601
http://dx.doi.org/10.1186/s12887-022-03356-5
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author Han, Wei
Ma, Jiannan
Jiang, Li
Cheng, Min
author_facet Han, Wei
Ma, Jiannan
Jiang, Li
Cheng, Min
author_sort Han, Wei
collection PubMed
description BACKGROUND: Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis is a rare autoimmune synaptic encephalitis associated with autoantibodies that cause a selective decrease in surface expression and changes in receptor localization. Anti-AMPAR encephalitis is poorly recognized, especially in children, and its clinical phenotype is incompletely described. CASE PRESENTATION: We report a case of anti-AMPAR GluR1 antibody-mediated autoimmune encephalitis in a 12-year-old male. The patient manifested as a fulminant course, with ataxia, cerebellar degeneration at the onset, and rapidly evolved into hyperthermia, coma and rhabdomyolysis. Antibodies against AMPAR GluR1 receptors were detected in the cerebrospinal fluid by cell-based assay. Diffuse slow waves were found by electroencephalograph, and the left cerebellar vermis and hemisphere were affected on brain magnetic resonance imaging (MRI). The patient was treated with intravenous immunoglobulin (IVIG), methylprednisolone combined with plasma exchange. Symptoms were alleviated after immunotherapy and the patient sustained clinical improvement. This is the first time that acute rhabdomyolysis symptom has been identified in a pediatric patient with anti-AMPAR encephalitis. CONCLUSIONS: This case expands the clinical spectrum of anti-AMPAR encephalitis and highlights that despite poor clinical manifestation at the outset, recovery remains possible. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12887-022-03356-5.
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spelling pubmed-91109372022-05-17 Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report Han, Wei Ma, Jiannan Jiang, Li Cheng, Min BMC Pediatr Case Report BACKGROUND: Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis is a rare autoimmune synaptic encephalitis associated with autoantibodies that cause a selective decrease in surface expression and changes in receptor localization. Anti-AMPAR encephalitis is poorly recognized, especially in children, and its clinical phenotype is incompletely described. CASE PRESENTATION: We report a case of anti-AMPAR GluR1 antibody-mediated autoimmune encephalitis in a 12-year-old male. The patient manifested as a fulminant course, with ataxia, cerebellar degeneration at the onset, and rapidly evolved into hyperthermia, coma and rhabdomyolysis. Antibodies against AMPAR GluR1 receptors were detected in the cerebrospinal fluid by cell-based assay. Diffuse slow waves were found by electroencephalograph, and the left cerebellar vermis and hemisphere were affected on brain magnetic resonance imaging (MRI). The patient was treated with intravenous immunoglobulin (IVIG), methylprednisolone combined with plasma exchange. Symptoms were alleviated after immunotherapy and the patient sustained clinical improvement. This is the first time that acute rhabdomyolysis symptom has been identified in a pediatric patient with anti-AMPAR encephalitis. CONCLUSIONS: This case expands the clinical spectrum of anti-AMPAR encephalitis and highlights that despite poor clinical manifestation at the outset, recovery remains possible. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12887-022-03356-5. BioMed Central 2022-05-17 /pmc/articles/PMC9110937/ /pubmed/35581601 http://dx.doi.org/10.1186/s12887-022-03356-5 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
Han, Wei
Ma, Jiannan
Jiang, Li
Cheng, Min
Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report
title Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report
title_full Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report
title_fullStr Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report
title_full_unstemmed Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report
title_short Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report
title_sort fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor glur1 antibodies encephalitis in a chinese boy: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9110937/
https://www.ncbi.nlm.nih.gov/pubmed/35581601
http://dx.doi.org/10.1186/s12887-022-03356-5
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