Paraneoplastic disorders.
Paraneoplastic disorders.
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DOI：
10.1212/01.con.0000464180.89580.88
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发表时间：
2015-04-01
期刊：
影响因子：
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通讯作者：
Lancaster, Eric
中科院分区：
文献类型：
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作者：
Lancaster, Eric
综述目的：副肿瘤性疾病是与特定癌症风险相关的自身免疫性疾病，以特异性自身抗体为标志。它们引起中枢和外周神经系统的各种临床综合征。最近的发现：在外周神经系统中，对突触或轴突蛋白的自身免疫长期以来被认为与特定的癌症有关。在这些疾病中，以重症肌无力为代表，抗体是直接毒性的，免疫治疗是恢复的规则。相比之下，典型的副肿瘤综合征涉及较高的癌症风险、细胞内蛋白（例如Hu蛋白）的自身抗体、靶向CNS或外周神经系统的T细胞依赖性疾病机制以及对治疗的不良反应。在发现N-甲基-D-天冬氨酸（NMDA）受体抗体后，出现了一组新的且不断扩大的疾病，涉及中枢神经系统突触和神经元膜蛋白的自身抗体以及对免疫治疗的良好反应。最后一组疾病涉及细胞内突触蛋白的抗体，如谷氨酸脱羧酶65（GAD 65），目前还不清楚这些疾病是否涉及抗体或T细胞机制。神经科医师应认识与副肿瘤相关的临床症状，利用自身抗体等检测确诊，了解疾病的病理基础，并及时给予适当的治疗。
PURPOSE OF REVIEW: Paraneoplastic disorders are autoimmune diseases associated with risks for specific cancers and marked by specific autoantibodies. They cause diverse clinical syndromes of the central and peripheral nervous systems.RECENT FINDINGS: In the peripheral nervous system, autoimmunity to synaptic or axonal proteins has long been recognized to associate with specific cancers. In these disorders, typified by myasthenia gravis, the antibodies are directly toxic, and recovery with immunotherapy is the rule. In contrast, the classic paraneoplastic syndromes involve a higher risk of cancer, autoantibodies to intracellular proteins (eg, Hu proteins), T-cell-dependent disease mechanisms targeting the CNS or peripheral nervous system, and a poor response to treatment. Following the discovery of N-methyl-D-aspartate (NMDA) receptor antibodies, a new and expanding group of disorders involving autoantibodies to CNS synaptic and neuronal membrane proteins and a favorable response to immunotherapy emerged. A final group of disorders involves antibodies to intracellular synaptic proteins, such as glutamic acid decarboxylase 65 (GAD65), and it is unclear whether these diseases involve antibody or T-cell mechanisms.SUMMARY: Neurologists should recognize the clinical syndromes associated with paraneoplastic disorders, utilize autoantibody and other testing to confirm the diagnosis, understand the pathologic basis of the diseases, and promptly give appropriate therapies.