Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report
Abstract Background Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis (Anti-AMPAR-E) is a very rare subtype of autoimmune encephalitis, typically presenting with memory decline, seizures, and changes in psychosis and behavior. Anti-AMPAR-E is often associated with the p...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2025-01-01
|
| Series: | BMC Neurology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12883-025-04040-8 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832571590222544896 |
|---|---|
| author | Chunxia Yan Lingling Bai Jingwei Du Zonglei Chong Guangjun Xu Xiaoqian Yang |
| author_facet | Chunxia Yan Lingling Bai Jingwei Du Zonglei Chong Guangjun Xu Xiaoqian Yang |
| author_sort | Chunxia Yan |
| collection | DOAJ |
| description | Abstract Background Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis (Anti-AMPAR-E) is a very rare subtype of autoimmune encephalitis, typically presenting with memory decline, seizures, and changes in psychosis and behavior. Anti-AMPAR-E is often associated with the presence of neoplasms and generally has a poor prognosis. Currently, cases of severe anti-AMPAR-E, particularly those accompanied by hyperammonemia, are exceedingly rare. Case presentation A 66-year-old man was admitted to the hospital, complaining of deterioration in memory and confusion for at least 10 days and worsening for 3 days. The patient’s condition rapidly progressed to coma, which persisted for 2 months, manifesting as a fulminant course. At that time, his Glasgow Coma Scale (GCS) score was 6, and AMPAR antibodies were strongly positive in both serum and cerebrospinal fluid (CSF). Additionally, his serum ammonia levels consistently exceeded reference values during his hospital stay. Consequently, he was diagnosed with severe anti-AMPAR-E with prolonged hyperammonemia and treated with intravenous methylprednisolone pulse (IVMP) therapy, intravenous immunoglobulin (IVIG), and rituximab therapy until he regained consciousness. However, 10 months after discharge, he was readmitted to the hospital due to seizures and subsequently diagnosed with lung cancer. The patient eventually passed away at home. Conclusions Even if the short-term prognosis is good, regular tumor-related screening is essential for patients with severe anti-AMPAR-E to detect potential tumors early and improve long-term outcomes. Moreover, it is necessary to perform repeated ammonia level assessments and to adequately treat hyperammonemia. |
| format | Article |
| id | doaj-art-71f2133fac2d4ef4ba1d8bc99810e647 |
| institution | Kabale University |
| issn | 1471-2377 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neurology |
| spelling | doaj-art-71f2133fac2d4ef4ba1d8bc99810e6472025-02-02T12:30:09ZengBMCBMC Neurology1471-23772025-01-012511810.1186/s12883-025-04040-8Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case reportChunxia Yan0Lingling Bai1Jingwei Du2Zonglei Chong3Guangjun Xu4Xiaoqian Yang5Department of Neurology, Liaocheng People ’ s HospitalDepartment of Neurology, Liaocheng People ’ s HospitalDepartment of Neurology, Liaocheng People ’ s HospitalDepartment of Neurosurgery, Liaocheng People’s HospitalDepartment of Neurology, Liaocheng People ’ s HospitalDepartment of Neurology, Liaocheng People ’ s HospitalAbstract Background Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis (Anti-AMPAR-E) is a very rare subtype of autoimmune encephalitis, typically presenting with memory decline, seizures, and changes in psychosis and behavior. Anti-AMPAR-E is often associated with the presence of neoplasms and generally has a poor prognosis. Currently, cases of severe anti-AMPAR-E, particularly those accompanied by hyperammonemia, are exceedingly rare. Case presentation A 66-year-old man was admitted to the hospital, complaining of deterioration in memory and confusion for at least 10 days and worsening for 3 days. The patient’s condition rapidly progressed to coma, which persisted for 2 months, manifesting as a fulminant course. At that time, his Glasgow Coma Scale (GCS) score was 6, and AMPAR antibodies were strongly positive in both serum and cerebrospinal fluid (CSF). Additionally, his serum ammonia levels consistently exceeded reference values during his hospital stay. Consequently, he was diagnosed with severe anti-AMPAR-E with prolonged hyperammonemia and treated with intravenous methylprednisolone pulse (IVMP) therapy, intravenous immunoglobulin (IVIG), and rituximab therapy until he regained consciousness. However, 10 months after discharge, he was readmitted to the hospital due to seizures and subsequently diagnosed with lung cancer. The patient eventually passed away at home. Conclusions Even if the short-term prognosis is good, regular tumor-related screening is essential for patients with severe anti-AMPAR-E to detect potential tumors early and improve long-term outcomes. Moreover, it is necessary to perform repeated ammonia level assessments and to adequately treat hyperammonemia.https://doi.org/10.1186/s12883-025-04040-8Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitisAutoimmune encephalitisHyperammonemia |
| spellingShingle | Chunxia Yan Lingling Bai Jingwei Du Zonglei Chong Guangjun Xu Xiaoqian Yang Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report BMC Neurology Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis Autoimmune encephalitis Hyperammonemia |
| title | Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report |
| title_full | Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report |
| title_fullStr | Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report |
| title_full_unstemmed | Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report |
| title_short | Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report |
| title_sort | severe anti α amino 3 hydroxy 5 methyl 4 isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia a case report |
| topic | Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis Autoimmune encephalitis Hyperammonemia |
| url | https://doi.org/10.1186/s12883-025-04040-8 |
| work_keys_str_mv | AT chunxiayan severeantiaamino3hydroxy5methyl4isoxazolepropionicacidreceptorencephalitiswithprolongedhyperammonemiaacasereport AT linglingbai severeantiaamino3hydroxy5methyl4isoxazolepropionicacidreceptorencephalitiswithprolongedhyperammonemiaacasereport AT jingweidu severeantiaamino3hydroxy5methyl4isoxazolepropionicacidreceptorencephalitiswithprolongedhyperammonemiaacasereport AT zongleichong severeantiaamino3hydroxy5methyl4isoxazolepropionicacidreceptorencephalitiswithprolongedhyperammonemiaacasereport AT guangjunxu severeantiaamino3hydroxy5methyl4isoxazolepropionicacidreceptorencephalitiswithprolongedhyperammonemiaacasereport AT xiaoqianyang severeantiaamino3hydroxy5methyl4isoxazolepropionicacidreceptorencephalitiswithprolongedhyperammonemiaacasereport |