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Recurrent Anti-AMPA Receptor Limbic Encephalitis: A Case Report and Literature Review
Yuanyuan Fang1, Dengji Pan1, Hao Huang1
1Department of Neurology, Tongji Medical College, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, China.
Abstract:
Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor encephalitis is a relatively rare anti-neuronal surface antigen autoimmune encephalitis (LE). We described a case of a 47-year-old Chinese man having anti-AMPA receptor limbic encephalitis initially presented with cognitive decline, undetectable antibodies, and normal imaging findings in magnetic resonance image (MRI) and then developed into typical autoimmune limbic encephalitis a few months later with a course of multiple relapses. In addition, we found progressive brain atrophy in our case, which was a rare presentation of LE. This report also summarized the characteristics of nine reported cases of anti-AMPA receptor limbic encephalitis with relapse up to date. This case highlighted that autoimmune limbic encephalitis is an important differential diagnosis for patients with typical symptoms even when the MRI and antibodies are normal, and more attention should be paid to the relapse of anti-AMPA receptor encephalitis.
Insights
Anti-AMPA receptor encephalitis, a rare autoimmune condition, can present subtly with cognitive decline and normal initial tests. This case highlights the importance of considering relapses and progressive brain atrophy in diagnosis.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Autoimmune encephalitis targeting neuronal surface antigens, such as Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors, represents a significant neurological challenge.
- Limbic encephalitis (LE) associated with anti-AMPA receptor antibodies is relatively rare, often presenting with complex neurological symptoms.
Observation:
- A case study details a 47-year-old Chinese man with anti-AMPA receptor limbic encephalitis, initially showing cognitive decline, undetectable antibodies, and normal MRI.
- The patient later developed typical autoimmune limbic encephalitis with multiple relapses and exhibited progressive brain atrophy, a rare manifestation.
Findings:
- The study reviewed nine reported cases of relapsing anti-AMPA receptor limbic encephalitis, alongside the presented case.
- Progressive brain atrophy was identified as a rare but notable feature in this form of autoimmune encephalitis.
Implications:
- Autoimmune limbic encephalitis should be considered in differential diagnoses for patients with suggestive symptoms, even with normal initial MRI and antibody tests.
- Increased clinical vigilance for relapses in anti-AMPA receptor encephalitis is crucial for timely diagnosis and management.
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