Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report
Abstract 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...
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BMC
2022-05-01
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| Series: | BMC Pediatrics |
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| Online Access: | https://doi.org/10.1186/s12887-022-03356-5 |
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| author | Wei Han Jiannan Ma Li Jiang Min Cheng |
| author_facet | Wei Han Jiannan Ma Li Jiang Min Cheng |
| author_sort | Wei Han |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-12T08:13:25Z |
| format | Article |
| id | doaj.art-5389cfe1ec6547498c0879b35c2a4593 |
| institution | Directory Open Access Journal |
| issn | 1471-2431 |
| language | English |
| last_indexed | 2024-12-12T08:13:25Z |
| publishDate | 2022-05-01 |
| publisher | BMC |
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| series | BMC Pediatrics |
| spelling | doaj.art-5389cfe1ec6547498c0879b35c2a45932022-12-22T00:31:41ZengBMCBMC Pediatrics1471-24312022-05-012211410.1186/s12887-022-03356-5Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case reportWei Han0Jiannan Ma1Li Jiang2Min Cheng3Department of Neurology Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of PediatricsDepartment of Neurology Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of PediatricsDepartment of Neurology Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of PediatricsDepartment of Neurology Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of PediatricsAbstract 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.https://doi.org/10.1186/s12887-022-03356-5Autoimmune encephalitisAMPAR encephalitisGluR1 receptorRhabdomyolysis |
| spellingShingle | Wei Han Jiannan Ma Li Jiang Min Cheng Fulminant anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR1 antibodies encephalitis in a Chinese boy: a case report BMC Pediatrics Autoimmune encephalitis AMPAR encephalitis GluR1 receptor Rhabdomyolysis |
| 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 | Autoimmune encephalitis AMPAR encephalitis GluR1 receptor Rhabdomyolysis |
| url | https://doi.org/10.1186/s12887-022-03356-5 |
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