Epileptic Phenotypes, Electroclinical Features and Clinical Characteristics in 17 Children with Anti-NMDAR Encephalitis

• Published in: European journal of paediatric neurology 2017-05, Vol.21 (3), p.457-464
• Main Authors: Haberlandt, E., MD, Ensslen, M, Gruber-Sedlmayr, U, Plecko, B, Brunner-Krainz, M, Schimmel, M, Schubert-Bast, S, Neirich, U, Philippi, H, Kurleman, G, Tardieu, M, Wohlrab, G, Borggraefe, I, Rostásy, K
• Format: Article
• Language: English
• Subjects: 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; Anti-NMDAR encephalitis; Child; Child, Preschool; Children; Delta brush; Delta Rhythm - physiology; Early Diagnosis; Electroencephalography; Epilepsy; Extreme; Female; Focal seizures; Generalized seizures; Humans; Male; Neurology; Pediatrics; Phenotype; Retrospective Studies; Seizures - complications; Seizures - diagnosis; Seizures - physiopathology
• ISSN: 1090-3798; 1532-2130
• 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.