ArticleBMC neurology2021
Clinical characteristics and prognosis of anti-alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic acid receptor encephalitis.
Article in BMC neurology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 17 citations in OpenAlex.
- Serial Cerebral Blood Flow Changes in AMPAR Encephalitis Presenting with Left Hemiplegia and Nonfluent Aphasia.Internal medicine (Tokyo, Japan) · 2026Article
- AMPAR immunization induces progressive autoimmune encephalitis with autoreactive B cells in the brain.bioRxiv : the preprint server for biology · 2026Article
- Clinical and neuroradiological findings in patients with Anti-AMPAR encephalitis: associations with outcomes.BMC neurology · 2026Review
- Article
- Cortical ribbon sign in AMPAR antibody-associated encephalitis mimicking Creutzfeldt-Jakob disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Severe anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with prolonged hyperammonemia: a case report.BMC neurology · 2025Article
- Clinical characteristics and outcome in patients with anti-AMPAR2 encephalitis: a case series and literature review.Frontiers in human neuroscience · 2025Article
- Autoimmune Encephalitis in a Patient with a Solitary Intracranial Plasmacytoma.Internal medicine (Tokyo, Japan) · 2024Article
- Clinical features, cerebrospinal fluid changes, and prognosis in Chinese patients with autoimmune encephalitis.Acta neurologica Belgica · 2024Article
- Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis developed after ovarian cancer cytoreduction surgery: a case report and literature review.BMC women's health · 2023Review
- Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment.Biomedicines · 2023Review
- Performance of the clinical assessment scale for autoimmune encephalitis in a pediatric autoimmune encephalitis cohort.Frontiers in immunology · 2022Review
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundEncephalitis associated with antibodies against alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) is an extremely rare type of antibody-mediated encephalitis. This research aims to investigate the clinical characteristics and prognosis of anti-AMPAR encephalitis.
methodsThis retrospective study enrolled nine patients with anti-AMPAR encephalitis. Demographic information, clinical manifestations, laboratory and radiological findings, treatment and response were collected and analyzed. These patients were followed up with an average period of 72 weeks to gather prognostic information.
resultsNine patients (7 females and 2 males) were enrolled with a mean age at disease onset of 59 years old. Three clinical pictures, including limbic encephalitis (n = 7; 78%), pure amnesia (n = 1; 11%) and fulminant encephalitis (n = 1; 11%) were identified. New symptoms of dysphagia and deafness were identified in the clinical spectrum of anti-AMPAR encephalitis. All patients had positive blood AMPAR antibodies, and six of them (67%) had paired positive antibodies in cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) was abnormal in 75% of the patients with no specific patterns recognized. Six patients (67%) had tumors, including lung cancers and thymomas. After immunotherapy and oncotherapy, partial improvement of neurological symptoms was observed among all 6 patients with available records during their hospitalization. After a mean follow-up of 72 weeks, 3 patients had marked decrease of modified Rankin Scale (mRS) score, 1 patient had unchanged mRS score, 4 patients died and the other one was lost.
conclusionsAnti-AMPAR encephalitis mainly presents as limbic encephalitis, and is paraneoplastic in 67% of cases. Thus, intensive screening for tumors is recommended for all anti-AMPAR patients. Although patients showed a good short-term therapeutic response, the overall prognosis was not satisfactory.
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