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[副肿瘤性神经系统综合征。自身抗体的诊断及发病机制意义]

[Paraneoplastic neurologic syndromes. Diagnostic and pathogenetic significance of autoantibodies].

作者信息

Kaiser R

机构信息

Neurologische Klinik und Poliklinik, Albert-Ludwigs-Universität, Freiburg.

出版信息

Nervenarzt. 1999 Aug;70(8):688-701. doi: 10.1007/s001150050498.

Abstract

Paraneoplastic neurologic syndromes (PNS) are rare disorders of the nervous system that cannot be ascribed to metastases or to destruction of vital systemic organs by the tumor or its treatment. Most frequently, PNS occur in association with small-cell lung, breast or ovarian carcinomas. The most frequent PNS is Lambert-Eaton myasthenic syndrome, which at the same time has the highest predictive value concerning an underlying carcinoma. PNS have been classified mostly under anatomical aspects but now are increasingly categorized according to specific antibodies. In certain neurologic syndromes (sensible and autonomic neuropathy, cerebellitis, limbic encephalitis, Opsoclonus-Myoclonus syndrome, Stiff-man syndrome, neuromyotonia, subacute amaurosis) specific autoantibodies are highly predictive for a neoplasm. Pathogenic relevance of these autoantibodies has been demonstrated only for antibodies against (1) voltage gated calcium channels (VGCC) in Lambert-Eaton myasthenic syndrome, (2) voltage gated potassium channels (VGKC) in neuromyotonia, and (3) recoverin in cancer associated retinopathy. The present article deals with the neurological symptoms of PNS, associated tumors, and the relevance of specific antibodies for the diagnosis and pathogenesis of PNS.

摘要

副肿瘤性神经系统综合征(PNS)是一种罕见的神经系统疾病,不能归因于肿瘤转移或肿瘤及其治疗对重要全身器官的破坏。最常见的情况是,PNS与小细胞肺癌、乳腺癌或卵巢癌相关。最常见的PNS是兰伯特-伊顿肌无力综合征,同时它对潜在癌症具有最高的预测价值。PNS大多根据解剖学方面进行分类,但现在越来越多地根据特定抗体进行分类。在某些神经系统综合征(感觉性和自主性神经病、小脑炎、边缘叶脑炎、眼阵挛-肌阵挛综合征、僵人综合征、神经性肌强直、亚急性黑矇)中,特定自身抗体对肿瘤具有高度预测性。仅在以下抗体中证明了这些自身抗体的致病相关性:(1)兰伯特-伊顿肌无力综合征中针对电压门控钙通道(VGCC)的抗体,(2)神经性肌强直中针对电压门控钾通道(VGKC)的抗体,以及(3)癌症相关性视网膜病变中针对 recoverin 的抗体。本文探讨了PNS的神经症状、相关肿瘤以及特定抗体在PNS诊断和发病机制中的相关性。

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