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Reversal of profound cortical atrophy with immunotherapy in pediatric anti-NMDA receptor encephalitis
Andy Liu1, Maeve C Lucas2, Lilia Kazerooni2
1Keck School of Medicine of the University of Southern California, Los Angeles, LA, United States of America.
Abstract:
Anti-NMDA receptor (NMDAr) encephalitis is an autoimmune condition marked by neuropsychiatric symptoms and NMDAr IgG autoantibodies in the serum and/or cerebrospinal fluid. While MRI findings are often nonspecific or normal, the reversibility of imaging abnormalities with immunotherapy remains underexplored. We report a 3-year-old girl who presented with seizures, altered mental status, and encephalopathy diagnosed with medically refractory anti-NMDAR encephalitis. Initial neuroimaging was unremarkable, but subsequent MRIs revealed T2 hyperintensities and leptomeningeal enhancement. After delayed treatment with IVIG, methylprednisolone, Rituximab, and plasmapheresis, the patient showed clinical improvement. Observational MRI scans after treatment demonstrated reduced parenchymal volume loss and stabilization of T2 changes over 4.5 years. This case highlights the potential for clinical and radiographic improvement after treatment in anti-NMDAR encephalitis and the benefit of immunomodulatory therapy even years after presentation.
Insights
This case study shows that even years after presentation, immunotherapy can lead to clinical and radiographic improvements in anti-NMDA receptor (NMDAR) encephalitis, a condition causing neuropsychiatric symptoms.
Area of Science:
- Neuroimmunology
- Pediatric Neurology
- Neuroradiology
Background:
- Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune disorder causing severe neuropsychiatric symptoms.
- Neuroimaging findings in NMDAR encephalitis are often nonspecific and their reversibility with treatment is not well understood.
- Early diagnosis and treatment are crucial, but outcomes can vary.
Purpose of the Study:
- To report a case of anti-NMDAR encephalitis in a pediatric patient with delayed diagnosis and treatment.
- To investigate the potential for clinical and radiographic recovery with aggressive immunotherapy.
- To highlight the long-term effects of immunomodulatory therapy on imaging abnormalities.
Main Methods:
- Case report of a 3-year-old girl with medically refractory anti-NMDAR encephalitis.
- Serial MRI scans to monitor brain abnormalities.
- Treatment with intravenous immunoglobulin (IVIG), methylprednisolone, Rituximab, and plasmapheresis.
Main Results:
- Initial MRI was normal; subsequent scans showed T2 hyperintensities and leptomeningeal enhancement.
- The patient experienced clinical improvement after multi-agent immunotherapy.
- Long-term follow-up (4.5 years) revealed reduced parenchymal volume loss and stabilized T2 changes on MRI.
Conclusions:
- Aggressive immunomodulatory therapy can lead to significant clinical and radiographic improvement in anti-NMDAR encephalitis, even with delayed treatment.
- Reversibility of imaging abnormalities is possible, suggesting a dynamic process responsive to treatment.
- This case underscores the importance of sustained immunotherapy in managing NMDAR encephalitis.
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