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卵巢畸胎瘤相关副肿瘤性脑炎的临床分析

Clinical analysis of paraneoplastic encephalitis associated with ovarian teratoma.

作者信息

Tonomura Yasuyo, Kataoka Hiroshi, Hara Yasuo, Takamure Miwa, Naba Itirou, Kitauti Takanori, Saito Kozue, Ueno Satoshi

机构信息

Department of Neurology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, and Ikeda City Hospital, Osaka, Japan.

出版信息

J Neurooncol. 2007 Sep;84(3):287-92. doi: 10.1007/s11060-007-9372-9. Epub 2007 Apr 13.

Abstract

BACKGROUND

Recently, paraneoplastic encephalitis associated with ovarian teratoma has been described and related to an autoantibody.

METHODS

We describe four patients with ovarian teratoma-associated encephalitis (OTE) and compared their clinical pictures with those of 17 previously reported patients with OTE.

RESULTS

Clinically, OTE was characterized by the development of acute prominent psychiatric symptoms (20 of 21 patients), seizures (15 of 21 patients), and central hypoventilation (13 of 21 patients). Our patients had hypersalivation (three patients) and cardiac conduction problems (all patients); hypothermia was present in one patient. The mean time from the onset of OTE to tumor diagnosis was 19.6+/-22.1 weeks. Ventilatory support was required for 54.9+/-25.4 days on average. The white blood cell count in cerebrospinal fluid was 55.1+/-61.2/mm3. Twelve patients showed abnormalities on cranial MRI, involving areas such as the temporal regions (seven patients) or brainstem (four patients). In addition to tumor resection, 17 patients received some type of immunotherapy: 17 patients received corticosteroids, 10 received intravenous immunoglobulins, two received cyclophosphamide, seven received plasma exchange. Eighteen patients with OTE had neurological improvement, including 11 with full recovery.

CONCLUSIONS

OTE presents with cardiac conduction problems and hypersalivation in addition to psychiatric symptoms, seizures, and central hypoventilation.

摘要

背景

最近,已报道了与卵巢畸胎瘤相关的副肿瘤性脑炎,并发现其与一种自身抗体有关。

方法

我们描述了4例卵巢畸胎瘤相关性脑炎(OTE)患者,并将其临床表现与之前报道的17例OTE患者进行了比较。

结果

临床上,OTE的特征为急性显著精神症状(21例患者中的20例)、癫痫发作(21例患者中的15例)和中枢性通气不足(21例患者中的13例)。我们的患者有流涎过多(3例患者)和心脏传导问题(所有患者);1例患者出现体温过低。从OTE发病到肿瘤诊断的平均时间为19.6±22.1周。平均需要54.9±25.4天的通气支持。脑脊液白细胞计数为55.1±61.2/mm³。12例患者的头颅MRI显示异常,累及颞叶区域(7例患者)或脑干(4例患者)等部位。除肿瘤切除外,17例患者接受了某种类型的免疫治疗:17例患者接受了皮质类固醇治疗,10例接受了静脉注射免疫球蛋白治疗,2例接受了环磷酰胺治疗,7例接受了血浆置换治疗。18例OTE患者的神经功能有改善,其中11例完全康复。

结论

OTE除了有精神症状、癫痫发作和中枢性通气不足外,还伴有心脏传导问题和流涎过多。

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