神经系统的副肿瘤性或自身免疫性疾病

定义

神经系统的副肿瘤性疾病和自身免疫性疾病是由于对中枢神经系统（如脑病、共济失调、脊髓炎）或周围神经系统（周围或自主神经病、神经肌肉接头病变或肌病）的神经元或胶质细胞的定向免疫攻击所致。在副肿瘤方面，这种攻击是由来自于系统性癌症的体表神经抗原启动的潜在有效肿瘤免疫反应的结果。在称为自身免疫的非副肿瘤性疾病中，尽管越来越多的证据表明至少在某些病例中存在感染性触发因素，但其病因仍不清楚。 这些疾病通常是多灶性的，导致神经系统多个层面的损伤和症状。经常发现有个人或家族的自身免疫病史。可发现伴随神经和非器官特异性（甲状腺过氧化物酶[TPO]抗体）自身抗体。神经自身抗体谱可以预测特定的癌症类型，并且可能与特定的神经表型相关。排除其他病因（如感染）很重要。对免疫治疗的反应可能支持诊断。

Paraneoplastic and autoimmune disorders of the nervous system result from a targeted immune attack on neurons or glial cells in the central (e.g. encephalopathy, ataxia, myelitis) or peripheral nervous systems (peripheral or autonomic neuropathies, neuromuscular junction disorders or myopathy). In the paraneoplastic context, this attack is a consequence of a potentially effective tumour immune response initiated by onco-neural antigens derived from a systemic cancer. In the non-paraneoplastic context termed autoimmune the etiology remains elusive though increasing evidence indicates a preceding infectious trigger in at least some cases. These disorders are commonly multifocal causing injury and symptoms arising from involvement at many levels of the nervous system. A personal or family history of autoimmunity is often found. Accompanying neural and non-organ specific (thyroid peroxidase [TPO] antibodies) autoantibodies may be found. The neural autoantibody profile may be predictive of a specific cancer type and may be associated with a particular neurological phenotype. Exclusion of alternative etiologies (e.g. infections) is important. Response to immunotherapy may support the diagnosis.

子条目 共 6 条

• 8E4A.0中枢神经系统（脑或脊髓）的副肿瘤性或自身免疫性疾病
• 8E4A.1周围神经或自主神经系统副肿瘤性或自身免疫性疾病
• 8E4A.2副肿瘤性或自身免疫性神经肌肉接头疾病
• 8E4A.3副肿瘤性或自身免疫性肌肉疾病
• 8E4A.Y其他特指的神经系统的副肿瘤性或自身免疫性疾病
• 8E4A.Z未特指的神经系统的副肿瘤性或自身免疫性疾病