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副瘤性神经系统综合征(paraneoplastic neurological syndromes,PNS)是一种与恶性肿瘤密切关联的神经系统疾病,通常要排除其他原因。由于神经系统的每个层面均可受累,从而可导致神经系统的各部位损害出现广泛的神经系统症状和体征。自从发现特异性抗神经元胞核、胞浆及胞膜自身循环抗体后,对该病的认识及诊断得到很大提高,证实自身免疫作用导致PNS发生。本文就该病的临床表现、临床诊断标准、发病机制及癌肿与自身免疫的相关性作一阐述。
Paraneoplastic neurological syndromes (PNS) is a neurological disease that is closely associated with malignant tumors, and other causes are usually excluded. Because every aspect of the nervous system can be affected, various parts of the nervous system can cause damage to a wide range of neurological symptoms and signs. Since the discovery of specific anti-neuronal nucleus, cytoplasm and membrane itself circulating antibodies, the understanding and diagnosis of the disease has been greatly improved, confirmed that autoimmune effects lead to PNS. This article describes the clinical manifestations of the disease, clinical diagnostic criteria, pathogenesis and the correlation between cancer and autoimmunity.