抗N-甲基-D-天冬氨酸受体脑炎中的眼阵挛-肌阵挛综合征
Opsoclonus-myoclonus syndrome in anti-N-methyl-D-aspartate receptor encephalitis.
作者信息
Kurian Mary, Lalive Patrice H, Dalmau Josep O, Horvath Judit
机构信息
Department of Neurosciences, Faculty of Medicine, University of Geneva, Geneva, Switzerland.
出版信息
Arch Neurol. 2010 Jan;67(1):118-21. doi: 10.1001/archneurol.2009.299.
BACKGROUND
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis has been recently reported as autoimmune/paraneoplastic encephalitis, affecting mostly young females.
OBJECTIVE
To describe opsoclonus-myoclonus syndrome in association with anti-NMDAR antibodies.
DESIGN
Case report.
SETTING
Geneva University Hospital. Patient A 23-year-old woman with opsoclonus-myoclonus syndrome.
RESULTS
Two weeks after an episode of gastroenteritis, the patient developed symptoms of depression associated with psychomotor slowing, progressive gait instability, and opsoclonus-myoclonus. Cerebrospinal fluid examination showed mild lymphocytic pleocytosis and intrathecal IgG synthesis with oligoclonal bands. The patient's condition worsened rapidly to an akinetic mutism, followed by a period of agitation, delirium, and hallucinations. These gradually subsided; however, a frontal behavior and executive dysfunction persisted 5 months after symptom presentation. No tumor was found. Anti-NMDAR antibodies were found in the cerebrospinal fluid.
CONCLUSIONS
Opsoclonus-myoclonus may occur in patients with anti-NMDAR encephalitis. Prompt diagnosis of this disorder is important because after tumor removal and immunomodulatory therapies it has a relatively good prognosis.
背景
抗N-甲基-D-天冬氨酸受体（抗NMDAR）脑炎最近被报道为一种自身免疫性/副肿瘤性脑炎，主要影响年轻女性。
目的
描述与抗NMDAR抗体相关的眼阵挛-肌阵挛综合征。
设计
病例报告。
地点
日内瓦大学医院。患者为一名患有眼阵挛-肌阵挛综合征的23岁女性。
结果
在一次胃肠炎发作两周后，患者出现与精神运动迟缓相关的抑郁症状、进行性步态不稳和眼阵挛-肌阵挛。脑脊液检查显示轻度淋巴细胞增多以及伴有寡克隆带的鞘内IgG合成。患者病情迅速恶化至运动不能性缄默，随后出现一段时间的激越、谵妄和幻觉。这些症状逐渐消退；然而，在症状出现5个月后，额叶行为和执行功能障碍仍然存在。未发现肿瘤。脑脊液中发现抗NMDAR抗体。
结论
抗NMDAR脑炎患者可能出现眼阵挛-肌阵挛。及时诊断这种疾病很重要，因为在切除肿瘤并进行免疫调节治疗后，其预后相对较好。
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