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
Clinical Department of Pediatrics I, Neuropediatrics, Medical University of Innsbruck, Innsbruck, Austria.
Department of Pediatric Neurology and Developmental Medicine and Social Pediatrics, Dr. von Haunersches Children's Hospital, University of Munich, Munich, Germany.
Eur J Paediatr Neurol. 2017 May;21(3):457-464. doi: 10.1016/j.ejpn.2016.11.016. Epub 2016 Dec 5.
Anti-N-methyl d-aspartate receptor (NMDAR) encephalitis is a rare disorder characterized by seizures, neuropsychiatric symptoms, dyskinesia and autonomic instability.
Aim of the present study was to evaluate the seizure phenotypes and electroencephalogram (EEG) features in children with anti-NMDAR encephalitis.
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.
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.
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.
抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种罕见疾病，其特征为癫痫发作、神经精神症状、运动障碍和自主神经功能不稳定。
本研究旨在评估抗NMDAR脑炎患儿的癫痫发作表型和脑电图（EEG）特征。
对来自欧洲九个中心的17例抗NMDAR脑炎患儿的癫痫发作类型、电临床特征和临床特点进行回顾性病例系列分析。
近半数（8/17）患儿出现精神症状，4/17患者癫痫发作是首发症状，5/17患者两种症状同时出现。在随后病程中，16/17患儿出现癫痫发作。首次脑电图检查发现11/17患者存在广泛性慢波，3/17患者存在局灶性慢波，仅3/17患儿脑电图背景活动正常。17例患者中有9例（53%）检测到极慢波刷（EDB）模式。
除精神症状外，抗NMDAR脑炎患儿在初次就诊时脑电图常显示广泛性慢波，无论有无癫痫发作。半数患儿存在EDB，这可能是早期发现这种免疫介导疾病的有用工具。
