부산대학교 도서관

Context:Paraneoplastic neurological syndromes (PNS) are rare neurological diseases that are remote effects of tumours and not caused by vascular, toxic or metabolic effects. Objective:Description of clinical presentation, diagnosis and evidence-based management of PNS.Material und methods:A systematic search of the literature was performed in Pubmed and was supplemented by a manual search.Results:Clinically, well characterized PNS are considered as classical PNS and must be differentiated from less well specified paraneoplastic neurological syndromes. The discovery of onconeuronal antibodies led to an important tool in the diagnosis of PNS, although their pathogenetic mechanism is not entirely known. With a recent cross-national prospective data survey, it was possible to obtain detailed information about these diseases and give more insight into some of the remaining questions. Another major achievement in recent years was the discovery of surface antibodies and associated syndromes which can present with very specific clinical conditions. Although frequently occurring in non-paraneoplastic conditions, some of these immune-mediated neurological disorders can also occur in a paraneoplastic context and therefore need special attention.Conclusion:Due to their often severe clinical course and the frequent appearance prior to tumour diagnosis, the recognition of PNS is of great importance for adequate management.