Voltz Raymond
Department of Palliative Medicine, University Hospital, Kerpener Str. 62, 50924, Köln, Germany.
J Neurol. 2006 Sep;253 Suppl 5:V33-8. doi: 10.1007/s00415-006-5005-z.
Combining clinical and immunological information, a neurological syndrome can now be diagnosed as a "definite" or "possible" paraneoplastic syndrome according to the newly suggested diagnostic criteria of the PNS Euronetwork. Differentiated diagnosis of a paraneoplastic syndrome is essential for differential therapy in patients. According to the response to IVIG therapy, paraneoplastic disorders may be subgrouped in group A, a clinical response is the rule (prototype Lambert-Eaton myasthenic syndrome), and in group B, IVIG may be helpful in single patients and is indicated in specific clinical settings (prototype anti-Hu associated neurological syndromes). The mode of action of IVIG may range from direct anti-idiotype effect to indirect effects on the cellular part of the pathogenesis of paraneoplastic syndromes. Due to the therapeutic relevance, it is therefore important to diagnose a PND as early as possible, and start immunotherapy including IVIG immediately.
结合临床和免疫学信息，根据PNS欧洲网络新提出的诊断标准，一种神经综合征现在可被诊断为“确诊”或“可能”的副肿瘤综合征。副肿瘤综合征的鉴别诊断对患者的鉴别治疗至关重要。根据对静脉注射免疫球蛋白（IVIG）治疗的反应，副肿瘤性疾病可分为A组，通常会有临床反应（典型的兰伯特-伊顿肌无力综合征），以及B组，IVIG可能对个别患者有帮助，且在特定临床情况下适用（典型的抗Hu相关神经综合征）。IVIG的作用方式可能从直接抗独特型效应到对副肿瘤综合征发病机制细胞部分的间接效应。因此，由于治疗相关性，尽早诊断副肿瘤性神经系统疾病并立即开始包括IVIG在内的免疫治疗很重要。
