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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 a toddler: A diagnostic challenge
Yasmin Khundakji1, Amira Masri2, Najwa Khuri-Bulos3
1Faculty of Medicine, The University of Jordan, Amman, Jordan.
Insights
Anti N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare autoimmune disorder in young children. This case highlights diagnostic challenges and successful treatment in a Jordanian toddler, emphasizing the need for more research in this demographic.
Area of Science:
- Pediatric Neurology
- Autoimmune Encephalitis
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a significant cause of pediatric encephalitis.
- Infants and toddlers are underrepresented in NMDAR encephalitis research, especially from the Middle East.
Observation:
- A 21-month-old Jordanian female toddler presented with behavioral changes and autistic features, mimicking other conditions.
- Concurrent urinary tract infection and gastroenteritis complicated the initial diagnosis.
Findings:
- The toddler was diagnosed with NMDAR encephalitis.
- Treatment included methylprednisolone, intravenous immunoglobulin (IVIg), plasma exchange, and rituximab, leading to gradual improvement.
Implications:
- This case underscores the diagnostic challenges of NMDAR encephalitis in very young children.
- It highlights the importance of considering NMDAR encephalitis in toddlers with atypical presentations and the need for more regional data.
Abstract:
Anti N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder and is considered to be one of the most common causes of encephalitis in children. Despite the fact that around half of all reported cases are of children, the number of studies that report infants and toddlers is very small. Furthermore, reports on children from the Middle East particularly are extremely rare. We report a 21-month-old Jordanian female toddler with NMDAR encephalitis, who initially presented with behavioral changes and some autistic features. She presented a diagnostic challenge due to a concurrent urinary tract infection and gastroenteritis. Multiple investigations were conducted and she was treated with methylprednisolone and intravenous immunoglobulin (IVIg) empirically as well as plasma exchange and rituximab once the diagnosis was confirmed. Her condition improved gradually. We discuss her clinical picture and the diagnostic challenges within this age group; we also review the current related literature.
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