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A Simple Cell-based Immunofluorescence Assay to Detect Autoantibody Against the N-Methyl-D-Aspartate NMDA Receptor in Blood
Published on: January 9, 2018
Solitary juxtacortical lesion associated with anti-N-methyl-D-aspartate receptor encephalitis: a case report
Rupan Gao1, Xiang Zhang2, Abhijeet Kumar Bhekharee3
1Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai, China.
Background:
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe autoimmune encephalitis mediated by anti-NMDA receptor antibodies. Brain MRI manifestations vary and are non-specific. If there are any lesions, they tend to be diffusely or multifocally distributed. Solitary lesion is relatively rare.
Case Presentation:
We report a 16-year-old girl who initially presented with focal seizures but developed severe psychiatric and extrapyramidal symptoms later on. Brain MRI revealed a solitary juxtacortical demyelinating lesion in the left frontal lobe. No enhancement was noted. Electroencephalogram captured epileptiform discharges in the same region. NMDAR IgGs were tested positive in the serum and cerebrospinal fluid. Corticosteroid and intravenous IgG were administered and the patient completely recovered. Brain MRI revealed a fainter lesion in the left frontal lobe.
Conclusion:
In very rare instances, anti-NMDA receptor encephalitis can present with a solitary brain lesion. A full panel of antibodies for autoimmune encephalitis is the key leading to the diagnosis.
Insights
Anti-NMDA receptor encephalitis, a severe autoimmune condition, can rarely present with a single brain lesion. Prompt diagnosis via antibody testing is crucial for effective treatment and recovery.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe autoimmune disorder.
- It is characterized by antibodies targeting NMDA receptors in the brain.
- Typical brain MRI findings are non-specific and often diffuse or multifocal.
Observation:
- A 16-year-old female presented with focal seizures, progressing to psychiatric and extrapyramidal symptoms.
- Brain MRI revealed a unique solitary juxtacortical demyelinating lesion in the left frontal lobe without enhancement.
- EEG showed epileptiform discharges localized to the lesion area.
Findings:
- Serum and cerebrospinal fluid tested positive for NMDAR IgGs.
- The patient received corticosteroid and intravenous immunoglobulin therapy.
- Complete recovery was observed, with MRI showing a fainter lesion post-treatment.
Implications:
- This case highlights that anti-NMDA receptor encephalitis can manifest with a solitary brain lesion, a rare presentation.
- Comprehensive antibody testing for autoimmune encephalitis is critical for accurate diagnosis.
- Early diagnosis and treatment lead to favorable outcomes in anti-NMDA receptor encephalitis.
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Seizures: Classification
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types: