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. 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.
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.
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 patientsseizures 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.