Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents.
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DOI：
10.1002/ana.21756
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发表时间：
2009-07
影响因子：
11.2
通讯作者：
Dalmau, Josep
中科院分区：
文献类型：
--
作者：
Florance, Nicole R.;Davis, Rebecca L.;Lam, Christopher;Szperka, Christina;Zhou, Lei;Ahmad, Saba;Campen, Cynthia J.;Moss, Heather;Peter, Nadja;Gleichman, Amy J.;Glaser, Carol A.;Lynch, David R.;Rosenfeld, Myrna R.;Dalmau, Josep
目的探讨≤ 18岁儿童抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎的临床特点。信息由作者或转诊医生获得。使用异位表达NR 1的HEK 293细胞，通过免疫细胞化学和酶联免疫吸附试验（ELISA）测定抗体。在8个月的时间内，确定了81例抗NMDAR脑炎患者（12例男性）。32例（40%）为≤18岁（最小23个月，中位14岁）; 6例为男性。卵巢畸胎瘤的发生率在>18岁女性中为56%，在≤18岁女孩中为31%（p = 0.05），在≤14岁女孩中为9%（p = 0.008）。所有男性患者均无肿瘤。在32例≤18岁的患者中，87.5%的患者表现为行为或性格改变，有时与癫痫发作和频繁的睡眠功能障碍有关; 9.5%的患者表现为运动障碍或肌张力障碍; 3%的患者表现为言语减少。入院时，53%有严重的语言障碍。最终，77%的患者出现癫痫发作，84%出现刻板动作，86%出现自主神经不稳定，23%出现换气不足。对免疫疗法的反应是缓慢和可变的。总体而言，74%的患者在免疫治疗或肿瘤切除后完全或实质性恢复。神经系统复发发生在25%。在末次随访时，切除畸胎瘤的患者（5/8）完全恢复的频率高于未切除畸胎瘤的患者（4/23; p = 0.03）。抗NMDAR脑炎在儿童中越来越多地被认识到，占所有病例的40%。年轻患者患肿瘤的可能性较小。行为和语言问题、癫痫发作和异常运动是常见的早期症状。表型与成人相似，但自主神经功能障碍和通气不足在儿童中不太常见或严重。
To report the clinical features of anti–N-methyl-D-aspartate receptor (NMDAR) encephalitis in patients ≤ 18 years old. Information was obtained by the authors or referring physicians. Antibodies were determined by immunocytochemistry and enzyme-linked immunosorbent assay (ELISA) using HEK293 cells ectopically expressing NR1. Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were ≤18 years old (youngest 23 months, median 14 years); 6 were male. The frequency of ovarian teratomas was 56% in women >18 years old, 31% in girls ≤18 years old (p = 0.05), and 9% in girls ≤14 years old ( p = 0.008). None of the male patients had tumors. Of 32 patients ≤18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p = 0.03). Anti-NMDAR encephalitis is increasingly recognized in children, comprising 40% of all cases. Younger patients are less likely to have tumors. Behavioral and speech problems, seizures, and abnormal movements are common early symptoms. The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children.
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DOI：
10.1038/ncpneuro0493
发表时间：
2007-05-01
期刊：
NATURE CLINICAL PRACTICE NEUROLOGY
影响因子：
--
作者：
Sansing, Lauren H.;Tuzun, Erdem;Dalmau, Josep
通讯作者：
Dalmau, Josep
影响因子：
48
作者：
Dalmau, Josep;Gleichman, Amy J.;Hughes, Ethen G.;Rossi, Jeffrey E.;Peng, Xiaoyu;Lai, Meizan;Dessain, Scott K.;Rosenfeld, Mynna R.;Balice-Gordon, Rita;Lynch, David R.
通讯作者：
Lynch, David R.
影响因子：
11
作者：
Graus, F;Delattre, JY;Voltz, R
通讯作者：
Voltz, R
影响因子：
3.9
作者：
Tonomura, Yasuyo;Kataoka, Hiroshi;Ueno, Satoshi
通讯作者：
Ueno, Satoshi
影响因子：
6
作者：
Wirtz, PW;Nijnuis, MG;Verschuuren, JJ
通讯作者：
Verschuuren, JJ