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

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

doi:10.1016/j.ejpn.2016.11.016

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

Eur J Paediatr Neurol. 2017 May;21(3):457-464. doi: 10.1016/j.ejpn.2016.11.016. Epub 2016 Dec 5.

Authors

E Haberlandt¹, M Ensslen², U Gruber-Sedlmayr³, B Plecko⁴, M Brunner-Krainz³, M Schimmel⁵, S Schubert-Bast⁶, U Neirich⁷, H Philippi⁸, G Kurleman⁹, M Tardieu¹⁰, G Wohlrab⁴, I Borggraefe², K Rostásy¹¹

Affiliations

¹ Clinical Department of Pediatrics I, Neuropediatrics, Medical University of Innsbruck, Innsbruck, Austria. Electronic address: edda.haberlandt@tirol-kliniken.at.
² Department of Pediatric Neurology and Developmental Medicine and Social Pediatrics, Dr. von Haunersches Children's Hospital, University of Munich, Munich, Germany.
³ Department of Pediatrics, Medical University of Graz, Graz, Austria.
⁴ Department of Neuropaediatrics and Neurophysiology, University Children's Hospital, Zuerich, Switzerland.
⁵ Department of Pediatrics, Klinikum Augsburg, Augsburg, Germany.
⁶ Department of Pediatric Neurology, University Children's Hospital Heidelberg, Heidelberg, Germany.
⁷ Department of Pediatrics, Neurology, Stiftungskrankenhäuser Frankfurt am Main, Clementine Kinderhospital, Frankfurt am Main, Germany.
⁸ Sozialpädiatrisches Zentrum Frankfurt Mitte, Frankfurt am Main, Germany.
⁹ Department of General Pediatrics, Neuropediatrics, University Children's Hospital Münster, Münster, Germany.
¹⁰ Assistance publique hôpitaux de Paris, Hôpitaux universitaires Paris-Sud, Université Paris-Sud, Paris, France.
¹¹ Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Witten, Germany.

PMID: 28017557
DOI: 10.1016/j.ejpn.2016.11.016

Abstract

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.

Keywords: Anti-NMDAR encephalitis; Children; Delta brush; Epilepsy; Extreme; Focal seizures; Generalized seizures.

Publication types

Multicenter Study

MeSH terms

Adolescent
Anti-N-Methyl-D-Aspartate Receptor Encephalitis / complications
Anti-N-Methyl-D-Aspartate Receptor Encephalitis / diagnosis*
Anti-N-Methyl-D-Aspartate Receptor Encephalitis / physiopathology*
Child
Child, Preschool
Delta Rhythm / physiology*
Early Diagnosis
Electroencephalography
Female
Humans
Male
Phenotype
Retrospective Studies
Seizures / complications
Seizures / diagnosis*
Seizures / physiopathology*