Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis

Background: Anti-N-methyl d-aspartate receptor (NMDAR) encephalitis is a rare disorder characterized by seizures, neuropsychiatric symptoms, dyskinesia and autonomic instability. Objective: Aim of the present study was to evaluate the seizure phenotypes and electroencephalogram (EEG) features in chi...

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Main Authors: Haberlandt, Edda (Author) , Plecko, Barbara (Author) , Schubert-Bast, Susanne (Author)
Format: Article (Journal)
Language:English
Published: 2017
In: European journal of paediatric neurology
Year: 2016, Volume: 21, Issue: 3, Pages: 457-464
ISSN:1532-2130
DOI:10.1016/j.ejpn.2016.11.016
Online Access:Verlag, Volltext: http://dx.doi.org/10.1016/j.ejpn.2016.11.016
Verlag, Volltext: http://www.sciencedirect.com/science/article/pii/S109037981630246X
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Author Notes:E. Haberlandt, M. Ensslen, U. Gruber-Sedlmayr, B. Plecko, M. Brunner-Krainz, M. Schimmel, S. Schubert-Bast, U. Neirich, H. Philippi, G. Kurleman, M. Tardieu, G. Wohlrab, I. Borggraefe, K. Rostásy

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245 1 0 |a Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis  |c E. Haberlandt, M. Ensslen, U. Gruber-Sedlmayr, B. Plecko, M. Brunner-Krainz, M. Schimmel, S. Schubert-Bast, U. Neirich, H. Philippi, G. Kurleman, M. Tardieu, G. Wohlrab, I. Borggraefe, K. Rostásy 
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520 |a Background: Anti-N-methyl d-aspartate receptor (NMDAR) encephalitis is a rare disorder characterized by seizures, neuropsychiatric symptoms, dyskinesia and autonomic instability. Objective: Aim of the present study was to evaluate the seizure phenotypes and electroencephalogram (EEG) features in children with anti-NMDAR encephalitis. Methods: Seizure types, electroclinical features and clinical characteristics of 17 children with anti-NMDAR encephalitis were analysed in a retrospective case series from nine centres in Europe. Results: Nearly half (8/17) of the children presented with psychiatric symptoms, whereas in 4/17 patients seizures were the first symptom and in 5/17 both symptoms occurred at the same time. During the following course seizures were reported in 16/17 children. The first EEG detected generalized slowing in 11/17 patients, focal slowing in 3/17 and normal background activity in only 3/17 children. The extreme delta brush (EDB) pattern was detected in 9/17 (53%) patients. Conclusion: In addition to psychiatric symptoms, children with anti-NMDAR encephalitis often show generalized slowing in EEG with or without seizures at initial presentation. EDB is present in half of all children and is potentially a helpful tool for early detection of this immune-mediated disease. 
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