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Treatment options in paraneoplastic disorders of the peripheral nervous system

机译：周围神经系统副肿瘤性疾病的治疗选择

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Opinion statement: Paraneoplastic disorders of the peripheral nervous system (PNS) are the most frequent manifestation of paraneoplasia. As with the central nervous system, two categories of immune mechanisms are distinguished. On one side, antibodies toward intracellular antigens (HuD and CV2-CRMP5) occur with subacute sensory neuronopathy or sensorimotor neuropathy probably depending on a T cell mediated disorder (group 1). On the other side, the Lambert-Eaton myasthenic syndrome (LEMS) and peripheral nerve hyperexcitability (PNH) occur with antibodies to cell membrane antigens, respectively, the voltage gated calcium channel and CASPR2 proteins, which are responsible for the disease (group 2). Treatment recommendation mostly depends on class IV studies. Three lines of therapeutics can be proposed, namely tumor, immunomodulatory and symptomatic treatments. Cancer treatment is crucial since an early tumor cure is the best way to stabilize patients in group 1 and improve those in group 2. This implies the use of an efficient strategy for cancer diagnosis. With group 2 symptomatic treatment including 3,4 diaminopyridine for LEMS and carbamazepine for PNH may suffice to obtain good quality remission. Immunomodulatory treatments like IVIg and plasma exchange, which have a well-established efficacy in antibody dependent diseases, may be used as second line treatments. Rituximab, for which there is only little evidence in this context, may be kept in a third line for severe refractory patients. With group 1 patients, who frequently develop an evolving and disabling disorder, bolus of methylprednisolone and or IVIg may be recommended while searching for and treating the tumor. If the tumor is not found and the patient deteriorates, monthly pulses of cyclophosphamide may stabilize the patients. Antidepressants and antiepileptic drugs efficacious in the treatment of neuropathic pain are to be used as symptomatic treatment when necessary. The choice is then based on the cost effectiveness and tolerance of these drugs.
机译：意见陈述：周围神经系统（PNS）的副肿瘤性疾病是副肿瘤的最常见表现。与中枢神经系统一样，免疫机制分为两类。一方面，针对细胞内抗原（HuD和CV2-CRMP5）的抗体可能伴有亚急性感觉神经病或感觉运动神经病（取决于T细胞介导的疾病）（第1组）。另一方面，兰伯特-伊顿肌无力综合症（LEMS）和周围神经过度兴奋性（PNH）分别与导致该疾病的细胞膜抗原抗体，电压门控钙通道和CASPR2蛋白发生（第2组） 。治疗建议主要取决于IV类研究。可以提出三种疗法，即肿瘤疗法，免疫调节疗法和对症疗法。癌症治疗至关重要，因为早期肿瘤治愈是稳定第1组患者并改善第2组患者的最佳方法。这意味着使用有效的癌症诊断策略。对于第2组，对症治疗包括LEMS的3,4二氨基吡啶和PNH的卡马西平，足以获得良好的质量缓解。在抗体依赖性疾病中具有公认的功效的免疫调节治疗（如IVIg和血浆置换）可以用作二线治疗。在这种情况下，利妥昔单抗的证据很少，对于严重的难治性患者，可以将利妥昔单抗保留在第三行。对于经常发展为残疾的疾病的第1组患者，在寻找和治疗肿瘤时可建议推注甲基强的松龙和/或IVIg。如果未发现肿瘤并且患者恶化，则每月一次环磷酰胺的脉搏可使患者稳定。有效治疗神经性疼痛的抗抑郁药和抗癫痫药将在必要时用作对症治疗。然后根据这些药物的成本效益和耐受性进行选择。

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