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: | , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
2017
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| 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 |
| Summary: | 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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| Item Description: | Gesehen am 04.09.2018 Available online 5 December 2016 |
| Physical Description: | Online Resource |
| ISSN: | 1532-2130 |
| DOI: | 10.1016/j.ejpn.2016.11.016 |