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Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis.
Eur J Paediatr Neurol. 2017 May; 21(3):457-464.EJ

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.

Authors+Show Affiliations

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

Pub Type(s)

Journal Article
Multicenter Study

Language

eng

PubMed ID

28017557

Citation

Haberlandt, E, et al. "Epileptic Phenotypes, Electroclinical Features and Clinical Characteristics in 17 Children With anti-NMDAR Encephalitis." European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society, vol. 21, no. 3, 2017, pp. 457-464.
Haberlandt E, Ensslen M, Gruber-Sedlmayr U, et al. Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis. Eur J Paediatr Neurol. 2017;21(3):457-464.
Haberlandt, E., 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. (2017). Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis. European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society, 21(3), 457-464. https://doi.org/10.1016/j.ejpn.2016.11.016
Haberlandt E, et al. Epileptic Phenotypes, Electroclinical Features and Clinical Characteristics in 17 Children With anti-NMDAR Encephalitis. Eur J Paediatr Neurol. 2017;21(3):457-464. PubMed PMID: 28017557.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis. AU - Haberlandt,E, AU - Ensslen,M, AU - Gruber-Sedlmayr,U, AU - Plecko,B, AU - Brunner-Krainz,M, AU - Schimmel,M, AU - Schubert-Bast,S, AU - Neirich,U, AU - Philippi,H, AU - Kurleman,G, AU - Tardieu,M, AU - Wohlrab,G, AU - Borggraefe,I, AU - Rostásy,K, Y1 - 2016/12/05/ PY - 2015/10/27/received PY - 2016/09/03/revised PY - 2016/11/25/accepted PY - 2016/12/27/pubmed PY - 2017/12/22/medline PY - 2016/12/27/entrez KW - Anti-NMDAR encephalitis KW - Children KW - Delta brush KW - Epilepsy KW - Extreme KW - Focal seizures KW - Generalized seizures SP - 457 EP - 464 JF - European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society JO - Eur. J. Paediatr. Neurol. VL - 21 IS - 3 N2 - 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. SN - 1532-2130 UR - https://www.unboundmedicine.com/medline/citation/28017557/Epileptic_phenotypes_electroclinical_features_and_clinical_characteristics_in_17_children_with_anti_NMDAR_encephalitis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1090-3798(16)30246-X DB - PRIME DP - Unbound Medicine ER -