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[一例伴副肿瘤性小脑变性和兰伯特-伊顿肌无力综合征的肺小细胞癌]

[A case of small cell carcinoma of the lung associated with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome].

作者信息

Seki Naomi, Mitsui Jun, Hoshino Masataka, Akishiro Masahiro, Ugawa Yoshikazu, Goto Jun, Tsuji Shoji

机构信息

Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.

出版信息

No To Shinkei. 2006 Jan;58(1):68-73.

Abstract

A 51-year-old male who showed severe ataxia, dysarthria, bilateral blepharoptosis, diplopia and nystagmus with the subacute onset was reported. The chest roentgenogram and CT scan revealed mass lesions at the hilus of the left lung. The tumor markers, NSE and ProGRP, were elevated; 12.8 ng/ml (< or = 10) and 140.7 pg/ml (< or = 46), respectively. The biopsy was performed surgically and the small cell carcinoma of the lung was confirmed pathologically. His cerebellar symptoms were considered to be caused by the paraneoplastc cerebellar degeneration. However, the blepharoptosis was peculiar. The electrophysiological studies were carried out The muscle strength test of the right APB muscle was 5. But the supramaximum stimulation of the right median nerve evoked only 2.0 mV of CMAP of the right APB muscle. The repetitive stimulation tests of the same nerve showed that 3 Hz stimulation resulted in 42% waning but 20 Hz stimulation evoked no waxing. The post-exercise test of the right APB muscle showed 73% increase of the CMAP. These findings indicated that he also suffered from Lambert-Eaton myasthenic syndrome. The titer of the antibody against the P/Q type voltage-gated calcium channel (VGCC) was remarkably elevated, 1,920 pM. None of the following antibodies were detected ; they included antibodies against acetylcholine receptor, Hu, Yo, Ri, Ma-2, CRMP-5, amphiphysin and glutamic acid dehydrogenase. The small cell carcinoma was treated with the combination of irinotecan hydrochloride and cisplatin, leading to the reduction of the mass lesions and the tumor markers. His cerebellar symptoms improved slightly but his blepharoptosis was unchanged. The titer of antibody against the P/Q type VGCC reduced remarkably to 451.8 pM. We reviewed reported cases associated with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome and discussed the relation between the paraneoplastic syndromes and autoantibodies.

摘要

报告了一名51岁男性,其亚急性起病,表现为严重共济失调、构音障碍、双侧上睑下垂、复视和眼球震颤。胸部X线片和CT扫描显示左肺门有占位性病变。肿瘤标志物NSE和ProGRP升高,分别为12.8 ng/ml(≤10)和140.7 pg/ml(≤46)。进行了手术活检,病理证实为肺小细胞癌。其小脑症状被认为是由副肿瘤性小脑变性引起的。然而,上睑下垂很特殊。进行了电生理研究。右拇短展肌肌力测试为5级。但右正中神经超强刺激仅诱发右拇短展肌复合肌肉动作电位(CMAP)2.0 mV。同一神经的重复刺激试验显示,3 Hz刺激导致波幅递减42%,但20 Hz刺激未诱发波幅递增。右拇短展肌运动后试验显示CMAP增加73%。这些发现表明他还患有兰伯特-伊顿肌无力综合征。抗P/Q型电压门控钙通道(VGCC)抗体滴度显著升高,为1920 pM。未检测到以下抗体:抗乙酰胆碱受体抗体、抗Hu抗体、抗Yo抗体、抗Ri抗体、抗Ma-2抗体、抗CRMP-5抗体、抗 amphiphysin抗体和抗谷氨酸脱氢酶抗体。肺小细胞癌采用盐酸伊立替康和顺铂联合治疗,导致占位性病变和肿瘤标志物降低。其小脑症状略有改善,但上睑下垂无变化。抗P/Q型VGCC抗体滴度显著降至451.8 pM。我们回顾了与副肿瘤性小脑变性和兰伯特-伊顿肌无力综合征相关的报道病例,并讨论了副肿瘤综合征与自身抗体之间的关系。

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