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Updated: May 23, 2026

A Simple Cell-based Immunofluorescence Assay to Detect Autoantibody Against the N-Methyl-D-Aspartate (NMDA) Receptor in Blood
Published on: January 9, 2018
[Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]
N Nunez-Enamorado1, A Camacho-Salas, S Belda-Hofheinz
1Hospital Universitario 12 de Octubre, Madrid, Espana. noenu_enamorado@yahoo.es
Insights
Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is increasingly diagnosed in children. Early recognition and immunotherapy, particularly plasmapheresis, are crucial for treating this potentially reversible neurological disorder.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
Background:
- Autoimmune encephalitis targeting N-methyl-D-aspartate (NMDA) receptors is increasingly recognized in pediatric populations.
- Suspected in children presenting with psychiatric symptoms, encephalopathy, movement disorders, or seizures.
Observation:
- A 2.5-year-old boy presented with subacute encephalopathy, seizures, behavioral changes, neurological regression, dyskinesias, and insomnia.
- Initial treatments with corticosteroids and IVIG were ineffective; however, plasmapheresis led to rapid and significant recovery.
- Cerebrospinal fluid was normal, MRI showed focal periventricular lesions and leptomeningeal enhancement, and EEG indicated diffuse delta activity with epileptiform discharges.
Findings:
- The patient tested positive for antibodies against the NMDA receptor.
- Plasmapheresis resulted in a swift and dramatic clinical improvement.
- Long-term follow-up showed mild residual behavioral and language alterations without relapse or need for maintenance therapy.
Implications:
- Anti-NMDA receptor encephalitis is a treatable condition, emphasizing the importance of early diagnosis.
- Immunotherapy, including plasmapheresis, is effective for non-paraneoplastic cases, offering rapid recovery.
- This case highlights the potential for significant neurological recovery with prompt and appropriate treatment in pediatric autoimmune encephalitis.
Introduction:
Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults.
Case Report:
We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment.
Conclusions:
Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.
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