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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
[An 8-year-old boy with anti-NMDA receptor encephalitis, successfully treated with cyclophosphamide]
Tadahiro Mitani1, Yoshimitsu Ohtsuka, Kei Yamamoto
1Division of Neurology, Kanagawa Children's Medical Center, Yokohama, Kanagawa.
Abstract:
We report on an 8-year-old boy with non-paraneoplastic anti-NMDA receptor (NMDAR) encephalitis, who presented with psychotic symptoms and involuntary movement following an intractable seizure. His serum and CSF tested positive for anti-NMDAR antibodies. He received an initial immunotherapy consisting of methylprednisolone pulse therapy (mPSL) and intravenous immunoglobulin therapy (IVIg), without any clinical improvement. He had three cycles of monthly cyclophosphamide pulse therapy (500 mg/m2), and his clinical condition started to improve gradually two weeks after the first cycle, without any side effects. Six months after onset, he tested normal upon standard neurological examination. Cyclophosphamide therapy should be considered for children with anti-NMDAR encephalitis, as well as mPSL and IVIg.
Insights
Cyclophosphamide therapy offers a promising treatment for pediatric anti-NMDA receptor (NMDAR) encephalitis. This approach, following initial immunotherapy, led to significant clinical improvement in an 8-year-old boy with NMDAR encephalitis.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Anti-NMDA receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
- Pediatric cases often present with complex symptoms including seizures, psychosis, and movement disorders.
Observation:
- An 8-year-old boy with non-paraneoplastic anti-NMDAR encephalitis showed no improvement with standard therapies like methylprednisolone pulse (mPSL) and intravenous immunoglobulin (IVIg).
- The patient experienced psychotic symptoms and involuntary movements secondary to intractable seizures.
Findings:
- Following three cycles of monthly cyclophosphamide pulse therapy, the patient exhibited gradual clinical improvement.
- Neurological function normalized six months post-treatment, with no reported side effects.
Implications:
- Cyclophosphamide pulse therapy is a viable and effective treatment option for pediatric anti-NMDAR encephalitis.
- This suggests cyclophosphamide should be considered alongside mPSL and IVIg in the management of pediatric anti-NMDAR encephalitis.
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