Paraneoplastic disorders of the central nervous system: Update on diagnostic criteria and treatment

Although the discovery of antineuronal antibodies has facilitated the diagnosis of paraneoplastic neurological disorders (PNDs), the recognition and treatment of these disorders remain a challenge. Some antineuronal antibodies have a more syndrome-specific association than others, and some syndromes evoke a paraneoplastic etiology more frequently than others. Because antineuronal antibodies may occur in cancer patients without PND, their detection does not necessarily imply that a neurological disorder is paraneoplastic. This review analyzes these issues and suggests a diagnostic strategy based on criteria derived from clinical and immunological findings and the presence or absence of cancer. We provide an update on the clinical features and treatment of classic PND of the central nervous system, with the proposal of a general treatment strategy. In addition, we analyze the evidence of a hypothetically effective antitumor immunity in patients with PND, which if confirmed would have implications for treatment of the cancer and PND.