Introduction: Paraneoplastic neurological syndromes (PNS) refer to a set of neurological disorders associated with neuronal degeneration in some patients with systemic cancers. These disorders are not related to the tumor mass or metastasis.
Current knowledge and key points: Evidence argues for an autoimmune reaction against tumor cells which expresses antigens normally present in neurons. A high percentage of patients with PNS harbors high titers of anti-neuronal autoantibodies in their serum and cerebrospinal fluid. In addition to their clinical interest in diagnosis and pathophysiology, these autoantibodies provide a unique opportunity to identify genes encoding previously undiscovered neuronal proteins which are also expressed by tumor cells. These "onconeural" antigens have been classified in four groups: neuromuscular junction proteins, nerve terminal/vesicle-associated proteins, neuronal RNA binding proteins, or neuronal signal transduction proteins.
Future prospects and projects: All of these proteins would play a major role in the neuronal maturation and homeostasis, and for some of them in cellular proliferation. Better understanding of the exact role of these proteins would in turn permit better understanding of the mechanisms of neuronal degeneration and tumor cell proliferation in PNS.