N-Methyl-D-Aspartate Receptor Antibodies in Pediatric Dyskinetic Encephalitis Lethargica

N-Methyl-D-Aspartate Receptor Antibodies in Pediatric Dyskinetic Encephalitis Lethargica
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DOI:
10.1002/ana.21807
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发表时间:
2009-11-01
影响因子:
11.2
通讯作者:
Vincent, Angela
Vincent, Angela
中科院分区:
医学1区
文献类型:
--
作者:
Dale, Russell C.;Irani, Sarosh R.;Vincent, Angela

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昏睡性脑炎是一种伴有精神、睡眠和锥体外系运动障碍的脑炎。运动障碍和帕金森病的形式已被描述。EL与抗N-甲基-D-天冬氨酸受体(NMDAR-Ab)脑炎具有相同的临床特征。我们研究了20例当代EL儿科患者的血清。10份血清(来自2名男性和8名女性,年龄1.3-13岁)和616份脑脊液样本NMDAR抗体阳性。NMDAR-Ab阳性的患者有运动障碍、激越、癫痫发作和失眠,而帕金森综合征和嗜睡在NMDAR-Ab阴性的儿童中占主导地位。我们无法识别任何肿瘤。EL的运动障碍形式是NMDAR-Ab脑炎,可以影响非常年幼的儿童。
Encephalitis lethargica (EL) describes an encephalitis with psychiatric, sleep, and extrapyramidal movement disorders. Dyskinetic and Parkinsonian forms have been described. EL shares clinical features with the anti-N-methyl-D-aspartate receptor (NMDAR-Ab) encephalitis. We studied 20 sera from pediatric patients with contemporary EL. Ten sera (from 2 males and 8 females, aged 1.3-13 years) and 616 cerebrospinal fluid samples were positive for NMDAR-Ab. NMDAR-Ab-positive patients had dyskinesias, agitation, seizures, and insomnia, whereas Parkinsonism and somnolence dominated in the NMDAR-Ab-negative children. We were unable to identify any tumors. The dyskinetic form of EL is an NMDAR-Ab encephalitis and can affect very young children.