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[副肿瘤性神经综合征相关的神经病变]

[Neuropathy associated with paraneoplastic neurological syndrome].

作者信息

Mitsui Yoshiyuki, Kusunoki Susumu

机构信息

Department of Neurology, Kinki University School of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan.

出版信息

Brain Nerve. 2010 Apr;62(4):387-93.

Abstract

Paraneoplastic neurological syndromes (PNSs) are the remote effects of cancer on the nervous system. The peripheral nervous system is an important targets of PNS. The neuropathies associated with PNS are reviewed in this article. Among the various paraneoplastic neuropathies, the main classical syndorome of PNS is subacute sensory neuronopathy that involves the cell bodies of sensory neurons in the dorsal root ganglia. Its clinical symptoms include sensory ataxia. Onconeuronal antibodies such as anti-Hu and anti-CV2/CRMP5 antibodies are frequently associated with this syndrome. In contrast to this classical form of PNS, non-classical syndromes are considered as heterogeneous neuropathies. The clinical features of non-classical syndromes are variable and no evident association between a clinical phenotype and onconeuronal antibodies has been established. Early detection and treatment of cancer is an essential for management of PNS. Neuropathies with paraproteinemia are also important clinical entities of PNS. IgM M-protein is most likely to cause neuropathy. Patients with IgM paraproteinemic neuropathy is usually characterized by predominan distal and sensory impairment, prolonged distal motor latencies in nerve conduction studies, and the presence of anti-MAG and SGPG antibodies.

摘要

副肿瘤性神经系统综合征(PNS)是癌症对神经系统产生的远隔效应。周围神经系统是PNS的重要靶标。本文对与PNS相关的神经病变进行综述。在各种副肿瘤性神经病变中,PNS的主要经典综合征是亚急性感觉神经元病,累及背根神经节中的感觉神经元胞体。其临床症状包括感觉性共济失调。抗Hu和抗CV2/CRMP5抗体等肿瘤神经元抗体常与该综合征相关。与这种经典形式的PNS不同,非经典综合征被认为是异质性神经病变。非经典综合征的临床特征各异,且尚未确立临床表型与肿瘤神经元抗体之间的明显关联。早期发现和治疗癌症是PNS管理的关键。伴有副蛋白血症的神经病变也是PNS的重要临床类型。IgM M蛋白最易引发神经病变。IgM副蛋白血症性神经病变患者通常以远端和感觉障碍为主,神经传导研究显示远端运动潜伏期延长,且存在抗MAG和SGPG抗体。

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