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副肿瘤性神经病

Paraneoplastic neuropathy.

作者信息

Koike Haruki, Sobue Gen

机构信息

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

出版信息

Handb Clin Neurol. 2013;115:713-26. doi: 10.1016/B978-0-444-52902-2.00041-2.

Abstract

Recent progress in serological screening of paraneoplastic antibodies and in diagnostic imaging techniques to detect malignancies has enabled a broadening of the concept of paraneoplastic neurological syndromes by integrating nonclassic clinical features. The peripheral nervous system is frequently involved in patients with paraneoplastic syndrome and may be seen alone or in combination with involvement of other areas of the nervous system. Destruction of dorsal root ganglion cells due to lymphocytic infiltration, especially with CD8-positive cytotoxic T cells, has been postulated to mediate the classic syndrome of subacute sensory neuronopathy. However, the motor and autonomic nervous systems are frequently affected. Indeed, patients can develop clinical features compatible with Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, or brachial plexopathy. Other forms of paraneoplastic neuropathy are vasculitic neuropathy, autoimmune autonomic ganglionopathy, and chronic intestinal pseudo-obstruction. Various onconeural antibodies, including anti-Hu, anti-CV2/CRMP-5, and anti-ganglionic acetylcholine receptor antibodies, are associated with neuropathy. Somatic neuropathy is the most common manifestation in patients with anti-Hu and anti-CV2/CRMP-5 antibodies, while anti-ganglionic acetylcholine receptor antibody is associated with autonomic neuropathies. A whole-body fluorodeoxyglucose positron emission tomography scan may be useful to detect malignancy in patients with unremarkable conventional radiological findings. Recognition and diagnosis of paraneoplastic neuropathy is important, as neuropathic symptoms usually precede the identification of the primary tumor, and treatment at an earlier stage provides better chances of good outcomes.

摘要

近年来,副肿瘤性抗体血清学筛查及检测恶性肿瘤的诊断成像技术取得了进展,通过整合非典型临床特征,拓宽了副肿瘤性神经综合征的概念。副肿瘤综合征患者的外周神经系统常受累,可单独出现,也可与神经系统其他部位受累同时出现。淋巴细胞浸润,尤其是CD8阳性细胞毒性T细胞浸润导致背根神经节细胞破坏,被认为是亚急性感觉神经元病经典综合征的发病机制。然而,运动和自主神经系统也常受影响。事实上,患者可出现与吉兰-巴雷综合征、慢性炎症性脱髓鞘性多发性神经病或臂丛神经病相符的临床特征。其他形式的副肿瘤性神经病变包括血管炎性神经病、自身免疫性自主神经节病和慢性肠道假性梗阻。多种肿瘤相关性神经元抗体,包括抗Hu、抗CV2/CRMP-5和抗神经节乙酰胆碱受体抗体,都与神经病变有关。抗Hu和抗CV2/CRMP-5抗体患者中,躯体神经病变是最常见的表现,而抗神经节乙酰胆碱受体抗体与自主神经病变有关。对于传统放射学检查结果无异常的患者,全身氟脱氧葡萄糖正电子发射断层扫描可能有助于检测恶性肿瘤。认识和诊断副肿瘤性神经病变很重要,因为神经病变症状通常先于原发性肿瘤的发现,早期治疗能提供更好的良好预后机会。

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