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Published on: November 23, 2013
The growing spectrum of antibody-associated inflammatory brain diseases in children
Sandra Bigi1, Manisha Hladio1, Marinka Twilt1
1Department of Pediatrics (S.B.), Division of Neurology; Department of Pediatrics (M.H.), Division of Rheumatology; and Department of Emergency Medicine and Research Institute (S.M.B.), The Hospital for Sick Children, University of Toronto, Ontario, Canada; Department of Pediatrics (S.B.), Division of Child Neurology, University Children's Hospital, Berne, Switzerland; Department of Pediatric Rheumatology (M.T.), Aarhus University Hospital, Aarhus, Denmark; Catalan Institution of Research and Advanced Studies (ICREA) and Biomedical Research Institute August Pi i Sunyer (IDIBAPS) (J.D.), Hospital Clinic, University of Barcelona, Spain; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; and Section of Rheumatology (S.M.B.), Department of Pediatrics, Alberta Children's Hospital, University of Calgary, Alberta, Canada.
Insights
Pediatric antibody-associated inflammatory brain diseases (AB-associated IBrainD) are an emerging concern, with 27% of affected children experiencing lasting neurologic deficits. Early diagnosis and immunosuppressive treatment are crucial for better outcomes in these complex cases.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Antibody-associated inflammatory brain diseases (AB-associated IBrainD) are increasingly recognized in pediatric populations.
- Understanding the clinical spectrum and outcomes is vital for effective management.
Purpose of the Study:
- To characterize the clinical presentation, diagnostic process, management strategies, and neurologic outcomes of pediatric AB-associated IBrainD.
- To highlight the importance of recognizing diverse clinical patterns for timely intervention.
Main Methods:
- Retrospective cohort study of pediatric patients (≤18 years) diagnosed with AB-associated IBrainD.
- Data collection included clinical features, laboratory results, neuroimaging, and treatment regimens.
- Study conducted at a single center from January 2005 to June 2013.
Main Results:
- 16 out of 169 children (10%) had AB-associated IBrainD, with a median age of 13.3 years.
- Common diagnoses included anti-NMDA receptor encephalitis (56%), aquaporin-4 autoimmunity (25%), Hashimoto encephalitis (13%), and anti-GAD65 encephalitis (6%).
- 27% of children experienced function-limiting neurologic sequelae at a median follow-up of 1.7 years; one patient died.
Conclusions:
- Pediatric AB-associated IBrainD constitute a significant and growing subgroup of inflammatory brain diseases.
- Prompt diagnosis and initiation of immunosuppressive therapy are essential for improving neurologic outcomes.
- Awareness of distinct clinical presentations aids in early detection and management.
Objective:
To describe the clinical spectrum, diagnostic evaluation, current management, and neurologic outcome of pediatric antibody-associated inflammatory brain diseases (AB-associated IBrainD).
Methods:
We performed a single-center retrospective cohort study of consecutive patients aged ≤18 years diagnosed with an AB-associated IBrainD at The Hospital for Sick Children, Toronto, Ontario, Canada, between January 2005 and June 2013. Standardized clinical data, laboratory test results, neuroimaging features, and treatment regimens were captured.
Results:
Of 169 children (93 female, 55%) diagnosed with an IBrainD, 16 (10%) had an AB-associated IBrainD. Median age at presentation was 13.3 years (range 3.1-17.9); 11 (69%) were female. Nine patients (56%) had anti-NMDA receptor encephalitis, 4 (25%) had aquaporin-4 autoimmunity, 2 (13%) had Hashimoto encephalitis, and 1 (6%) had anti-glutamic acid decarboxylase 65 (GAD65) encephalitis. The key presenting features in children with anti-NMDA receptor encephalitis, Hashimoto encephalopathy, and anti-GAD65 encephalitis included encephalopathy, behavioral symptoms, and seizures; patients with aquaporin-4 autoimmunity showed characteristic focal neurologic deficits. Six patients (38%) required intensive care unit admission at presentation. Median time from symptom onset to diagnosis was 55 days (range 6-358). All but 1 patient received immunosuppressive therapy. One child with anti-NMDA receptor encephalitis died due to multiorgan failure. At last follow-up, after a median follow-up time of 1.7 years (range 0.8-3.7), 27% of the children had function-limiting neurologic sequelae.
Conclusions:
Children with AB-associated IBrainD represent an increasing subgroup among IBrainD; 1 in 4 children has function-limiting residual neurologic deficits. Awareness of the different clinical patterns is important in order to facilitate timely diagnosis and initiate immunosuppressive treatment.
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