Centre de référence maladies rares "syndromes neurologiques paranéoplasiques", hospices civils de Lyon, hôpital neurologique, 59 boulevard Pinel, Lyon cedex 03, France.
Rev Neurol (Paris). 2011 Oct;167(10):729-36. doi: 10.1016/j.neurol.2011.08.001. Epub 2011 Sep 3.
Paraneoplastic neurological syndromes (PNS) are rare diseases defined so far by the presence of a neurological disorder associated with cancer in the absence of invasion of the nervous system by tumor cells. Discovery of circulating autoantibodies specific for these patients has revolutionized the diagnosis and understanding of these syndromes and demonstrated a role of the immune system in the neurological syndromes. Until recent years, we thought that these autoantibodies were only markers of the disease and had no role in the pathophysiology. The recent description of autoantibodies directed against membrane receptors or channels and playing a direct pathological role has transformed the concept of PNS. Especially, it appears that many patients may have a neurological syndrome and autoantibodies without cancer. This results in a classification based on the nature of the autoantibodies associated with neurological syndrome. In case of autoantibodies targeting intracellular antigens, cancer is almost always associated, the neurological disorders are mainly related to neuronal death, patients are rarely sensitive to immunomodulatory treatments and cellular immunity appears to play a major role. In contrast, patients with autoantibodies targeting membrane antigens (receptors, channels or receptor associated proteins) have rarely cancer, neurological disorders are related to a reversible neuronal dysfunction, patients are mostly sensitive to immunomodulatory treatments and it seems that humoral immunity and autoantibodies play a major role.
副肿瘤神经系统综合征（PNS）是目前定义为存在与癌症相关的神经系统疾病而无肿瘤细胞侵犯神经系统的罕见疾病。对这些患者特异性的循环自身抗体的发现，彻底改变了这些综合征的诊断和认识，并证明了免疫系统在神经系统综合征中的作用。直到最近，我们还认为这些自身抗体只是疾病的标志物，在病理生理学中没有作用。最近对针对膜受体或通道的自身抗体的描述及其直接的病理作用，改变了 PNS 的概念。特别是，许多患者可能患有神经综合征和自身抗体而无癌症。这导致了一种基于与神经综合征相关的自身抗体性质的分类。如果自身抗体针对细胞内抗原，几乎总是与癌症相关，神经障碍主要与神经元死亡有关，患者对免疫调节治疗的敏感性较低，细胞免疫似乎起主要作用。相比之下，针对膜抗原（受体、通道或受体相关蛋白）的自身抗体的患者很少发生癌症，神经障碍与神经元功能可逆障碍有关，患者对免疫调节治疗大多敏感，似乎体液免疫和自身抗体起主要作用。
