Neuropediatrics 2013; 44 - FV14_06
DOI: 10.1055/s-0033-1337736

Anti-NMDAR encephalitis in children: Delineation of the epileptic phenotypes and EEG findings

E Haberlandt 1, M Ensslen 2, U Gruber-Sedlmayr 3, B Plecko 4, M Schimmel 5, S Schubert-Bast 6, E Schuler 6, M Tardieu 7, G Wohlrab 4, I Borggräfe 2, K Rostásy 1
  • 1Departement of Pediatrics I, Division of Pediatric Neurology, Medical University Innsbruck, Austria
  • 2Haunersches Kinderspital, Universität München, München, Germany
  • 3Department of Pediatrics, Medical University of Graz, Graz, Austria
  • 4Department of Neuropediatrics, University Hospital, Zürich, Switzerland
  • 5Klinikum Augsburg, Augsburg, Germany
  • 6Sektion Neuropädiatrie, University Hospital, Heidelberg, Germany
  • 7Service de Neurologie Pédiatrique, Hoptal Bicetre, Paris, France

Aims: In adults with anti-NMDAR encephalitis, the epileptic phenotypes and EEG findings have been described in detail, but this delineation is lacking so far in children.

Methods: Retrospective study of the clinical features, EEG-findings, and outcome of 15 children with anti-NMDAR encephalitis from 7 different neuropediatric centers.

Results: From August 2002 until January 2012, 15 children, 4 male (26%) and 11 female (73%) patients, with median age at disease onset of 6 10/12 (range 1 9/12 to 17 3/12) were included. All children fulfilled the criteria of anti-NMDAR encephalitis proved by antibodies in either CSF or plasma. Mean follow-up period was 14 months (range 2 to 24 months). Of these 15 patients, 3 (20%) presented initially with seizures, 8 (53%) with psychiatric symptoms, and 4 (27%) had seizures combined with psychiatric symptoms at onset. Only 1 out of 15 (6%) patients had no seizures. Generalized seizures (GS) without any other seizure type were observed in 5 (33%), whereas only focal seizures (FS) appeared in 3 (20%), and 6 (40%) presented with both seizure types. In children younger than 12 years, 4 of the 15 (27%) patients had only GS and 3 (20%) had only FS, whereas children older than 12 years presented only in one case with GS only (1/15 6, 7%), 3 (20%) patients had two seizure types in both age groups. Status epilepticus was seen in 2 of the 15 (13%) patients. The initial EEG showed slowing in 12 out of 15 (80%) and was normal only in 3 out of 15 (20%). There was no interictal epileptic discharge in 9 out of 15 (60%), in 3 out of 15 (20%) generalized EP, in 3/15 (20%) focal EP was described. After a median follow-up of 14 months (range 2 to 24 months), 2 (13%) patients had still seizures, 5 (36%) had a complete recovery, and 9 (64%) suffered from moderate or severe deficits.

Conclusion: Although adults with anti-NMDAR encephalitis often have focal forms of epilepsy, in children there was no dominance of any seizures type. Slowing in EEG is obvious in 80%, whereas EP at the beginning of the disease is rare.