Child Neurology: Parainfectious Anti-NMDAR Encephalitis With Anti-MOG and Anti-AQP4 Seropositivity in an Infant

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.

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Publication Details

Journal
Neurology
Published
2025-09-15
DOI
https://doi.org/10.1212/wnl.0000000000214125
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
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article

Child Neurology: Parainfectious Anti-NMDAR Encephalitis With Anti-MOG and Anti-AQP4 Seropositivity in an Infant

Suvasini Sharma, Sayoni Roy Chowdhury, Nihaal Reddy, Varinder Singh et al.
Neurology
Autoimmune Neurological Disorders and Treatments
article

Child Neurology: Parainfectious Anti-NMDAR Encephalitis With Anti-MOG and Anti-AQP4 Seropositivity in an Infant

Suvasini Sharma, Sayoni Roy Chowdhury, Nihaal Reddy, Varinder Singh, Rakesh Kumar Meena, Kriti Nautiyal
article en

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.

NeurologyVol. 105(7)
Lady Hardinge Medical College (IN), Rainbow Children's Hospital (IN)
Openalex Percentile: Top 64%
Autoimmune Neurological Disorders and Treatments
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Child Neurology: Parainfectious Anti-NMDAR Encephalitis With Anti-MOG and Anti-AQP4 Seropositivity in an Infant — Suvasini Sharma, Sayoni Roy Chowdhury, et al. · Neurology (2025) | TGRS Research Map | TGRS