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抗N-甲基-D-天冬氨酸受体抗体相关自身免疫性边缘叶脑炎与卵巢畸胎瘤:一种可治疗的副肿瘤性边缘叶脑炎形式

[Autoimmune limbic encephalitis with anti-NMDA receptor antibodies and ovarian teratoma: a treatable form of paraneoplastic limbic encephalitis].

作者信息

Henry C, Husson H, de Broucker T

机构信息

Service de neurologie, hôpital Delafontaine, 2, rue Pierre-Delafontaine, 93200 Saint-Denis, France.

出版信息

Rev Neurol (Paris). 2009 Jan;165(1):70-5. doi: 10.1016/j.neurol.2008.07.020. Epub 2008 Sep 21.

Abstract

INTRODUCTION

The first French-language case of limbic encephalitis due to anti-N-methyl-D-aspartate receptor to glutamate (NMDA-R) antibodies associated with an ovarian teratoma is reported.

CLINICAL CASE

A 35-year-old woman presented with a subacute severe anterograde memory deficit, psychiatric disturbances and generalized seizures associated with an ovarian teratoma. No abnormality was noticed on the two successive MRI. The cerebrospinal fluid showed mild lymphocytosis and elevation of protein concentration. The search for classical onconeuronal antibodies in the serum was negative. Total body computed tomographic scan disclosed a five centimeter long ovarian cyst. Pathology found an ovarian teratoma containing a small immature neuroepithelial component. Complete tumor resection associated with high doses of intravenous methylprednisolone and intravenous polyvalent immunoglobulins allowed her clinical state to improve as soon as three days after surgery. Full recovery was noted four months later. Serum anti-NMDA-R antibodies were positive.

CONCLUSION

Owing to the recent description of the association between anti-NMDA-R and limbic encephalitis and the frequent good prognosis reported in the available series and case reports, it is important to search for this association in the not so rare cases of limbic encephalitis when no other cause is disclosed.

摘要

引言

本文报道了首例由抗N-甲基-D-天冬氨酸受体(NMDA-R)谷氨酸抗体引起的边缘性脑炎伴卵巢畸胎瘤的法语病例。

临床病例

一名35岁女性,表现为亚急性严重顺行性记忆缺陷、精神障碍及全身性癫痫发作,伴有卵巢畸胎瘤。连续两次磁共振成像(MRI)均未发现异常。脑脊液显示轻度淋巴细胞增多及蛋白浓度升高。血清中经典肿瘤神经元抗体检测为阴性。全身计算机断层扫描显示一个5厘米长的卵巢囊肿。病理检查发现卵巢畸胎瘤含有少量未成熟神经上皮成分。完整的肿瘤切除联合大剂量静脉注射甲泼尼龙和静脉注射多价免疫球蛋白,使她在术后三天临床状态即有所改善。四个月后实现完全康复。血清抗NMDA-R抗体呈阳性。

结论

鉴于近期有关抗NMDA-R与边缘性脑炎之间关联的描述,以及现有系列研究和病例报告中报道的频繁良好预后情况,在未发现其他病因的边缘性脑炎病例中,寻找这种关联很重要,此类病例并不罕见。

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