Paraneoplastic neurological syndromes (PNS) are very rare, remote effects of malignancies. Well-characterised antibodies against intracellular neuronal antigens are well-known in association with distinct tumors and with classical and non-classical syndromes. In this review article the current aspects of classification, pathophysiology, underlying tumors, antineuronal autoantibodies and diagnostic and therapeutic aspects of syndromes affecting the central nervous system are summarized.