Anti-N-methyl-D-aspartate receptor encephalitis: a newly recognized inflammatory brain disease in children.
Anti-N-methyl-D-aspartate receptor encephalitis: a newly recognized inflammatory brain disease in children.
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DOI：
10.1002/art.30437
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发表时间：
2011-08
影响因子：
--
通讯作者：
Benseler, Susanne M.
中科院分区：
文献类型：
--
作者：
Luca, Nadia;Daengsuwan, Tassalapa;Dalmau, Josep;Jones, Kevin;deVeber, Gabrielle;Kobayashi, Jeffrey;Laxer, Ronald M.;Benseler, Susanne M.
抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种新近发现的抗神经元抗体介导的炎症性脑病，可导致既往健康儿童严重的精神和神经功能缺损。本研究的目的是报告诊断为抗NMDAR脑炎的儿童的特征性临床特征和结局。在12个月的时间内，对表现出与抗NMDAR脑炎一致的新获得的精神和/或神经功能缺损以及CNS炎症证据的连续性儿童进行筛查。如果儿童在血清和/或脑脊液（CSF）中有抗NMDAR抗体的确证证据，则将其纳入研究。报告了临床表现和检查结果的详细信息。记录治疗类型和持续时间以及末次随访时的结局。筛查了7名儿童，确定了3名患有抗NMDAR脑炎的儿童。所有患者均表现为神经或精神（“神经精神”）异常、癫痫发作、言语障碍、睡眠障碍和意识水平波动。两名年龄较大的患者也有更突出的精神病学特征，而年龄较小的孩子则有明显的自主神经不稳定和明显的不自主运动障碍。没有一个有潜在的肿瘤。免疫抑制治疗导致接近或完全恢复;然而，2例患者早期复发，需要重新治疗。抗NMDAR脑炎是儿童神经精神缺陷的一个重要原因，必须包括在CNS血管炎和其他炎性脑病的鉴别诊断中。早期诊断和治疗对神经功能恢复至关重要。
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a newly recognized anti-neuronal antibody-mediated inflammatory brain disease causing severe psychiatric and neurological deficits in previously healthy children. The aim of this study was to report characteristic clinical features and outcomes of children diagnosed with anti-NMDAR encephalitis. Consecutive children presenting with newly acquired psychiatric and/or neurologic deficits consistent with anti-NMDAR encephalitis and evidence of CNS inflammation were screened over a 12-month period. Children were included in the study if they had confirmatory evidence of anti-NMDAR antibodies in the serum and/or cerebrospinal fluid (CSF). Details of clinical presentation and results of investigations were reported. Type and duration of treatment and outcomes at last follow-up were documented. Seven children were screened and three children with anti-NMDAR encephalitis were identified. All patients presented with neurological or psychiatric (‘neuropsychiatric’) abnormalities, seizures, speech disorder, sleep disturbance, and fluctuating level of consciousness. The two older patients also had more prominent psychiatric features, while the younger child had significant autonomic instability and prominent involuntary movement disorder. None had an underlying tumor. Immunosuppressive therapies resulted in near or complete recovery; however, two of the patients had early relapse requiring re-treatment. Anti-NMDAR encephalitis is an important cause of neuropsychiatric deficits in children that must be included in the differential diagnosis of CNS vasculitis and other inflammatory brain diseases. Early diagnosis and treatment are essential for neurologic recovery.
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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：
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通讯作者：
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影响因子：
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影响因子：
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作者：
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影响因子：
48
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通讯作者：
Lynch, David R.
DOI：
10.1111/j.1469-8749.2009.03542.x
发表时间：
2010-05-01
影响因子：
3.8
作者：
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Deonna, Thierry