Child Neurology: Parainfectious Anti-NMDAR Encephalitis With Anti-MOG and Anti-AQP4 Seropositivity in an Infant
Kriti Nautiyal1, Sayoni Roy Chowdhury2, Rashmi Meena2
1Department of Paediatrics. Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India.
Abstract:
Overlap syndromes of anti-N-methyl-d-aspartate (NMDA) receptor encephalitis and myelin oligodendrocyte glycoprotein (MOG) or aquaporin-4 (AQP4)-mediated demyelination, either concurrently or sequentially, have been increasingly recognized. We report a 7-month-old infant who presented with 7 days of fever, seizures, irritability, and signs of meningeal irritation. Initial workup suggested acute meningoencephalitis; therefore, the child was treated with antibiotics, antivirals, empirical antitubercular therapy, and steroids. After transient improvement, the child's condition deteriorated including the development of new-onset choreo-ballistic-dystonic movements, encephalopathy, sleep disturbances, and dysautonomia. CSF analysis revealed positivity for parvovirus B19 and Epstein-Barr virus, along with autoantibodies against the NMDA receptor. In addition, serum testing was positive for anti-MOG and anti-AQP4 antibodies. Despite prompt immunotherapy with methylprednisolone, intravenous immunoglobulin, rituximab, and cyclophosphamide, the child showed only moderate improvement and later developed drug-refractory infantile epileptic spasm syndrome. The coexistence of multiple autoantibodies for immune-mediated encephalitis in this case illustrates potential links to underlying disease mechanisms as well as underscores the therapeutic challenges posed by triple seropositivity (anti-NMDA receptor, anti-MOG, and anti-AQP4 IgG autoantibodies) in an infant.
Insights
This case report details a rare infant encephalitis with triple autoantibody positivity (anti-NMDA receptor, anti-MOG, anti-AQP4). It highlights diagnostic and therapeutic challenges in pediatric autoimmune neurological diseases.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Autoimmune Encephalitis
Background:
- Overlap syndromes involving anti-NMDA receptor encephalitis and MOG/AQP4-mediated demyelination are increasingly recognized.
- Autoimmune encephalitis presents diagnostic and therapeutic challenges, particularly in infants.
Purpose of the Study:
- To report a rare case of a 7-month-old infant with concurrent anti-NMDA receptor encephalitis, anti-MOG, and anti-AQP4 antibodies.
- To illustrate the diagnostic complexities and treatment difficulties associated with triple autoantibody positivity in pediatric autoimmune neurological disorders.
Main Methods:
- Clinical presentation of fever, seizures, irritability, and movement disorders in an infant.
- Cerebrospinal fluid (CSF) and serum analysis for viral pathogens and specific autoantibodies (anti-NMDA receptor, anti-MOG, anti-AQP4).
- Administration of multi-agent immunotherapy including steroids, IVIG, rituximab, and cyclophosphamide.
Main Results:
- The infant presented with meningoencephalitis, choreo-ballistic-dystonic movements, encephalopathy, and dysautonomia.
- CSF was positive for parvovirus B19 and Epstein-Barr virus; serum tested positive for anti-NMDA receptor, anti-MOG, and anti-AQP4 IgG autoantibodies.
- Despite prompt immunotherapy, the infant showed only moderate improvement and developed refractory infantile epileptic spasm syndrome.
Conclusions:
- The coexistence of multiple autoantibodies in this infant highlights potential links to underlying disease mechanisms in autoimmune encephalitis.
- Triple seropositivity (anti-NMDA receptor, anti-MOG, anti-AQP4 IgG) presents significant therapeutic challenges in pediatric cases.
- This case underscores the need for comprehensive diagnostic evaluation and tailored treatment strategies for complex autoimmune neurological conditions in infants.
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