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Published on: July 24, 2016
Limbic encephalitis in children and adolescents.
E Haberlandt1, T Bast, A Ebner
1Medical University of Innsbruck, Clinical Department of Pediatrics IV, Division of Neuropediatrics and Inherited Metabolic Diseases, Innsbruck, Austria.
Archives of Disease in Childhood
|October 21, 2010
Summary
Limbic encephalitis, though rare in children, shares adult disease characteristics. Early immunotherapy may improve outcomes for pediatric patients with this autoimmune neurological disorder.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Limbic encephalitis is uncommon in individuals under 18 and often lacks a formal diagnosis.
- The condition presents with a clinico-radiological syndrome affecting limbic areas.
Purpose of the Study:
- To investigate the presentation and outcomes of pediatric patients with limbic encephalitis.
- To identify specific neuronal autoantibodies (Abs) associated with the condition in this age group.
Main Methods:
- Retrospective study of 10 patients (<18 years) diagnosed with limbic encephalitis over 3.5 years.
- Involved 12 European neuropaediatric and neurological departments.
- Neuronal autoantibodies were determined in specialized labs; MRI confirmed mediotemporal encephalitis.
Main Results:
- Median age at onset was 14 years; 8 patients had defined autoantibodies (GAD Abs, VGKC Abs, Hu Abs, Ma1/2 Abs).
- A neuroblastoma was found in one patient with Hu Abs.
- After a median of 15 months follow-up with immunotherapy, 2 patients recovered, 8 remained impaired, and 1 died.
Conclusions:
- Pediatric limbic encephalitis mirrors adult forms, suggesting similar pathogenic mechanisms.
- Most cases were non-paraneoplastic, necessitating evaluation of more aggressive immunotherapies.
- Improved treatment strategies are crucial for better patient outcomes.
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