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Limbic encephalitis in children and adolescents
  1. E Haberlandt1,
  2. T Bast2,
  3. A Ebner3,
  4. H Holthausen4,
  5. G Kluger4,
  6. R Kravljanac5,
  7. J Kröll-Seger6,
  8. G Kurlemann7,
  9. C Makowski8,
  10. K Rostasy1,
  11. E Tuschen-Hofstätter9,
  12. G Weber10,
  13. A Vincent11,
  14. CG Bien12
  1. 1Medical University of Innsbruck, Clinical Department of Pediatrics IV, Division of Neuropediatrics and Inherited Metabolic Diseases, Innsbruck, Austria
  2. 2University Children's Hospital, Heidelberg, Germany
  3. 3Epilepsy Centre Bethel, Clinic Mara, Bielefeld, Germany
  4. 4Neuropediatric Department, BHZ Vogtareuth, Vogtareuth, Germany
  5. 5University of Belgrade, Institute for Mother and Child Health, Belgrade, Serbia
  6. 6Swiss Epilepsy Centre, Zurich, Switzerland
  7. 7University of Muenster, Department of Neuropediatrics, Muenster, Germany
  8. 8Munich University of Technology, Department of Pediatrics, Munich, Germany
  9. 9St. Marien-Hospital Bonn, Pediatric Department, Bonn, Germany
  10. 10Klinikum Duisburg, Department of Pediatrics, Duisburg, Germany
  11. 11University of Oxford, L6 West Wing and Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
  12. 12University of Bonn Medical Centre, Department of Epileptology, Bonn, Germany
  1. Correspondence to Dr Christian G Bien, Department of Epileptology, University of Bonn Medical Centre, Sigmund-Freud-Str. 25, 53105 Bonn, Germany; christian.bien{at}ukb.uni-bonn.de

Abstract

Objective Limbic encephalitis is rare in people <18 years of age and rarely given a formal diagnosis.

Design Retrospective study on presentation and outcome of children and adolescents with the clinico-radiological syndrome of limbic encephalitis tested for specific neuronal autoantibodies (Abs) over 3.5 years.

Setting Assessment, diagnosis, treatment and follow-up at 12 neuropaediatric and neurological departments in Europe, with Abs determined in Bonn, Germany and Oxford, UK.

Patients Ten patients <18 years of age who presented with a disorder mainly affecting the limbic areas of <5 years' duration with MRI evidence of mediotemporal encephalitis (hyperintense T2/FLAIR signal, resolving over time).

Results Median age at disease onset was 14 years (range 3–17). Eight patients had defined Abs: one each with Hu or Ma1/2 Abs, four with high titre glutamic acid decarboxylase (GAD) Abs, two of whom had low voltage-gated potassium channel (VGKC) Abs and two with only low titre VGKC Abs. A tumour was only found in the patient with Hu Abs (a neuroblastoma). After a median follow-up of 15 months with corticosteroid or intravenous immunoglobulin treatment, starting after a median of 4 months, two patients recovered, eight remained impaired and one died.

Conclusions Limbic encephalitis is a disease that can occur in childhood or adolescence with many of the hallmarks of the adult disorder, suggesting that both result from similar pathogenic processes. Since most of the cases were non-paraneoplastic, as now also recognised in adults, more systematic and aggressive immunotherapies should be evaluated in order to improve outcomes.

https://doi.org/10.1136/adc.2010.183897

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    Footnotes

    • Competing interests Dr Vincent and her department in Oxford receive royalties and payments for antibody assays for the diagnosis of different neurological disorders. Dr Vincent is a consultant for Athena Diagnostics Inc (USA) and RSR Ltd (UK), and holds a patent on VGKC-antibody targets.

    • Provenance and peer review Not commissioned; externally peer reviewed.