Background: Paraneoplastic neurologic syndromes (PNS) constitute a rare group of disorders resulting from damage to the nervous system in the setting of cancer physically unrelated to the tumor site. PNS are believed to result from an autoimmune attack of normal neuronal tissue, spurred by similar neuronal antigens ectopically expressed by tumor cells.
Review summary: The most common PNS are reviewed and also their association with specific onconeural antibodies, some directly pathogenic, others whose role in the disease process is less clear-cut. This diversity in pathogenesis is likely due to the relative role of humoral versus cellular immunity in PNS. Virtually any cancer may result in PNS but certain tumors, small cell lung cancer, gynecologic cancers (breast and ovarian), thymoma, and plasma cell tumors are more frequently encountered. In most instances, immunosuppressive therapy is unhelpful and outcome is poor.
Conclusions: PNS have diverse presentations, affecting both the central and peripheral nervous system and commonly, it is the PNS, not cancer that is the presenting symptom. Only subsequently, after onconeural antibodies are discovered or cancer is found, is PNS diagnosed. Neurologists should familiarize themselves with these rare syndromes and treatment principles, as rapid detection and treatment of the underlying tumor offer the best chance for recovery or prevention of further neurologic deterioration.