Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients.
Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients.
复制标题
DOI：
10.1016/j.jpeds.2012.10.011
复制
发表时间：
2013-04
期刊：
影响因子：
--
通讯作者：
Spanish Anti-N-methyl-D-Aspartate Receptor (NMDAR) Encephalitis Work Group
中科院分区：
文献类型：
--
作者：
Armangue T;Titulaer MJ;Málaga I;Bataller L;Gabilondo I;Graus F;Dalmau J;Spanish Anti-N-methyl-D-Aspartate Receptor (NMDAR) Encephalitis Work Group
报告20例抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎患儿的临床特点。回顾过去4年在西班牙的一个中心进行的临床数据、长期随访和免疫学研究。患者的中位年龄为13岁（范围，8个月-18岁），70%为女性。在12名患者（60%）的初始症状是神经系统，通常是运动障碍或癫痫发作，在其他40%的精神。发病一个月后，所有患者都出现不自主运动以及行为和言语的改变。所有患者均接受类固醇，静脉注射免疫球蛋白（IVIG）或血浆置换，7利妥昔单抗或环磷酰胺。中位随访时间为17.5个月，85%的患者基本恢复，10%中度或重度功能障碍，1例死亡。3例患者既往发作与抗NMDAR脑炎相符，其中2例在诊断疾病后复发。卵巢畸胎瘤被确定为两名患者，一个在脑炎发作，另一个一年后。两个新的观察结果（各一名患者）包括，鉴定被认为是这种疾病特征的脑电图模式（“极端δ刷”），以及单纯疱疹脑炎（HSE）后抗NMDAR脑炎的发展。儿童抗NMDAR脑炎的初始症状与成人不同（儿童中神经性症状较多，精神性症状较少），单症状疾病的发展极为罕见（复发除外），大多数患者对治疗有反应。我们的研究表明HSE后舞蹈手足徐动症和抗NMDAR脑炎之间存在联系。
To report the clinical features of 20 pediatric patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Review of clinical data, long-term follow-up, and immunological studies performed in a single center in Spain in the last 4 years. The median age of the patients was 13 years (range, 8 months-18 years), 70% were female. In 12 patients (60%) the initial symptoms were neurologic, usually dyskinesias or seizures, and in the other 40% psychiatric. One month into the disease, all patients had involuntary movements and alterations of behavior and speech. All patients received steroids, intravenous immunoglobulin (IVIG) or plasma exchange, and 7 rituximab or cyclophosphamide. With a median follow up of 17.5 months, 85% had substantial recovery, 10% moderate or severe deficits, and 1 died. Three patients had previous episodes compatible with anti-NMDAR encephalitis, 2 of them with additional relapses after the diagnosis of the disorder. Ovarian teratoma was identified in two patients, one at onset of encephalitis and the other one year later. Two novel observations (one patient each) include, the identification of an electroencephalographic pattern (“extreme delta brush”) considered characteristic of this disorder, and the development of anti-NMDAR encephalitis after herpes simplex encephalitis (HSE). The initial symptoms of pediatric anti-NMDAR encephalitis vary from those of the adults (more neurologic and less psychiatric in children), the development of a mono-symptomatic illness is extremely rare (except in relapses), and most patients respond to treatment. Our study suggests a link between post-HSE choreoathetosis and anti-NMDAR encephalitis.
登录
查看更多内容
DOI：
10.1093/brain/awq113
发表时间：
2010-06
期刊：
Brain : a journal of neurology
影响因子：
--
作者：
Irani SR;Bera K;Waters P;Zuliani L;Maxwell S;Zandi MS;Friese MA;Galea I;Kullmann DM;Beeson D;Lang B;Bien CG;Vincent A
通讯作者：
Vincent A
影响因子：
--
作者：
Luca, Nadia;Daengsuwan, Tassalapa;Dalmau, Josep;Jones, Kevin;deVeber, Gabrielle;Kobayashi, Jeffrey;Laxer, Ronald M.;Benseler, Susanne M.
通讯作者：
Benseler, Susanne M.
影响因子：
56.3
作者：
Granerod, Julia;Ambrose, Helen E.;Crowcroft, Natasha S.
通讯作者：
Crowcroft, Natasha S.
影响因子：
3.6
作者：
Bseikri, Mustafa R.;Barton, Jubilee R.;Roy-Burman, Arup
通讯作者：
Roy-Burman, Arup
影响因子：
11.8
作者：
Gable, Mary S.;Sheriff, Heather;Glaser, Carol A.
通讯作者：
Glaser, Carol A.