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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
Anti-NMDA receptor encephalitis in children: the disorder, its diagnosis, and treatment
Harry E Peery1, Gregory S Day, Asif Doja
1Department of Obstetrics and Gynecology, Division of Reproductive Biology, McMaster University, Hamilton, Ontario, Canada.
Insights
Anti-NMDA receptor encephalitis is a severe autoimmune disorder affecting the brain, often misdiagnosed initially. Early diagnosis and immunosuppressant therapy improve outcomes for this condition.
Area of Science:
- Neuroscience
- Immunology
- Pediatrics
Background:
- Anti-NMDA receptor encephalitis is a severe, newly identified neuroautoimmune syndrome.
- It typically presents with a prodromal infection, followed by psychiatric and neurological symptoms.
- The condition is more prevalent in females and can lead to significant disability.
Purpose of the Study:
- To describe the clinical course, diagnosis, and treatment of anti-NMDA receptor encephalitis.
- To highlight diagnostic challenges and potential complications.
- To emphasize the importance of early diagnosis and treatment for improved prognosis.
Main Methods:
- Review of clinical presentations and diagnostic findings.
- Identification of antibodies against the NR1 subunit of the NMDA receptor in serum and CSF.
- Analysis of tumor association, particularly teratomas.
Main Results:
- The syndrome progresses through distinct phases: prodromal, psychotic/seizure, akinetic, and hyperkinetic.
- Diagnosis is confirmed by specific antibody detection and oligoclonal bands in CSF.
- Tumors are found in 25% of adolescents, and relapses occur in 20-25% of children.
Conclusions:
- Early diagnosis and immunosuppressant therapy are crucial for improving outcomes.
- Recovery can be prolonged, and patients may not always return to their premorbid health status.
- Understanding the clinical spectrum and diagnostic markers is essential for effective management.
Abstract:
Anti-NMDA receptor encephalitis is a newly characterized severe neuroautoimmune syndrome with a progressive, clinical course. Most often seen in females, it usually begins with a prodromal phase suggestive of an acute or subclinical upper respiratory tract infection that lasts for up to 2 weeks. This is followed by a psychotic and seizure phase in which the child may rapidly develop seizures, behavioral changes, and, less commonly in children, psychiatric symptoms, resulting in frequent misdiagnoses. The child may become mute and unresponsive but awake during the akinetic phase. Autonomic instability characterizes the hyperkinetic phase. A teratoma or, more rarely, another tumor type is found in 25% of affected adolescents beyond the first decade of life. The finding of oligoclonal protein electrophoresis (>80%) and antibodies in serum and cerebrospinal fluid directed against the NR1 subunit of the NMDA receptor confirms the diagnosis. Prognosis is improved with the appropriate use of immunosuppressant therapies. Relapses in children may be multiple and occur in 20-25% of cases. Recovery is slow and may take 3 years or longer. Even so, the child may not always regain its premorbid level of health.
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