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[一名由乳腺癌诱发副肿瘤性边缘叶脑炎的患者出现嗜睡症状]

[A patient with paraneoplastic limbic encephalitis induced by breast cancer presenting with hypersomnia].

作者信息

Kawamura Nobutoshi, Kawajiri Masakazu, Ohyagi Yasumasa, Minohara Motozumi, Murai Hiroyuki, Kira Jun-ichi

机构信息

Department of Neurology, neurological Institute, Graduate School of Medical Sciences, Kyushu University.

出版信息

Rinsho Shinkeigaku. 2005 Aug;45(8):575-8.

Abstract

A 50-year-old woman developed gait disturbance and hypersomnia over a period of a month. General physical examination revealed axillary lymph node swelling. On neurological examinations she was fully orientated but hypersomnic; short term memory disturbance, horizontal gaze evoked nystagmus and ataxic gait were observed. Electroencephalography disclosed a tendency for easily decreasing vigilance with delta activities but normal dominant rhythm. Cerebrospinal fluid examinations showed increased protein amounts (109 mg/dl) without either pleocytosis or atypical cells. An echogram of the breasts revealed a tiny mass in the left side. Pathological studies on a biopsied lymph node and the mass in her left breast showed a mammillary duct carcinoma. Brain MRI was normal, and no anti-neuronal antibody was detected in sera by two dimensional immunoblotting using human brain crude antigens. She was diagnosed as having paraneoplastic limbic encephalitis (PLE) associated with breast cancer. Over 42 hours polysomnography showed long total sleep time (TST) with a high ratio of sleep stage 1/TST and no REM sleep abnormalities; this resembled a thalamic-hypothalamic damaged sleep pattern. At first she was treated with plasma exchanges, but no improvement was observed. Hormonal and chemotherapies produced partial resolution of her neurologic symptoms and there were signs of reduction of the breast mass. Most reported PLE cases with hypersomnia have been associated with testicular cancer and anti-Ma antibodies. The present case is an extremely rare example manifesting hypersomnia without either testicular cancer or anti-Ma antibody. Since anti-tumor therapy successfully ameliorated her neurologic symptoms, cell-mediated immunity against a common tumor and neuronal antigens rather than hormonal immunity may have played a role in the development of her PLE.

摘要

一名50岁女性在一个月内出现步态障碍和嗜睡。全身体格检查发现腋窝淋巴结肿大。神经系统检查显示她意识完全清醒但嗜睡;存在短期记忆障碍、水平凝视诱发眼球震颤和共济失调步态。脑电图显示有因δ波活动导致警觉性易降低的倾向,但主导节律正常。脑脊液检查显示蛋白量增加(109mg/dl),无细胞增多或异型细胞。乳房超声检查发现左侧有一个小肿块。对活检淋巴结和左侧乳房肿块的病理研究显示为乳腺导管癌。脑部MRI正常,使用人脑粗抗原进行二维免疫印迹法检测血清中未发现抗神经元抗体。她被诊断为与乳腺癌相关的副肿瘤性边缘叶脑炎(PLE)。超过42小时的多导睡眠图显示总睡眠时间长(TST),睡眠1期/TST比例高,且快速眼动睡眠无异常;这类似于丘脑 - 下丘脑受损的睡眠模式。起初她接受了血浆置换治疗,但未见改善。激素和化疗使她的神经症状部分缓解,且有乳房肿块缩小的迹象。大多数报道的伴有嗜睡的PLE病例与睾丸癌和抗Ma抗体有关。本病例是一个极其罕见的例子,表现为既无睾丸癌也无抗Ma抗体的嗜睡。由于抗肿瘤治疗成功改善了她的神经症状,针对常见肿瘤和神经元抗原的细胞介导免疫而非激素免疫可能在她的PLE发病过程中起了作用。

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