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与未成熟卵巢畸胎瘤相关的副肿瘤性脑炎中的EFA6A样抗体:病例报告

EFA6A-like antibodies in paraneoplastic encephalitis associated with immature ovarian teratoma: a case report.

作者信息

Koide Reiji, Shimizu Toshio, Koike Kazunori, Dalmau Josep

机构信息

Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo 183-0042, Japan.

出版信息

J Neurooncol. 2007 Jan;81(1):71-4. doi: 10.1007/s11060-006-9200-7. Epub 2006 Jun 29.

Abstract

We describe a 19-year-old patient with paraneoplastic encephalitis associated with immature ovarian teratoma (OT), who presented with psychiatric symptoms, prolonged disturbance of consciousness, refractory status epilepticus, central hypoventilation, and various abnormal involuntary movements. Immunological characterization of the patient's serum and cerebrospinal fluid (CSF) demonstrated the presence of an autoantibody that colocalized with EFA6A, a brain-specific protein involved in the regulation of dendritic development of hippocampal neurons. Despite the severity of the symptoms, the patient showed significant neurological improvement following removal of the tumor and chemotherapy. This case suggests that physicians should rule out an OT in young women with encephalitis who present with the subacute-onset of psychiatric symptoms. Antibodies that colocalize with EFA6A are a valuable marker for early diagnosis of a potentially reversible paraneoplastic encephalitis associated with OT.

摘要

我们描述了一名19岁患有与未成熟卵巢畸胎瘤(OT)相关的副肿瘤性脑炎的患者,该患者出现精神症状、意识长期紊乱、难治性癫痫持续状态、中枢性通气不足以及各种异常不自主运动。对患者血清和脑脊液(CSF)的免疫学特征分析显示存在一种自身抗体,该抗体与EFA6A共定位,EFA6A是一种参与调节海马神经元树突发育的脑特异性蛋白。尽管症状严重,但患者在肿瘤切除和化疗后神经功能有显著改善。该病例表明,对于出现亚急性精神症状的脑炎年轻女性,医生应排除OT。与EFA6A共定位的抗体是早期诊断与OT相关的潜在可逆性副肿瘤性脑炎的重要标志物。

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