Paraneoplastic neurological syndromes: an update on diagnosis, pathogenesis, and therapy

Since the discovery of the first clinically relevant anti-neuronal antibody specific for a paraneoplastic aetiology in 1985, the number of such reactivities has grown at a rate of about one per year. Clinicians can now diagnose a paraneoplastic syndrome much more easily. This ability is especially important because, typically, the neurological symptoms occur before the cancer is diagnosed. Early tumour diagnosis is essential, because effective treatment of the cancer still seems to be the most efficient treatment option for the neurological symptoms. Immunomodulatory therapy should, nevertheless, be initiated as early as possible and seems especially helpful for peripheral syndromes and limbic encephalitis. The recent fundamental advances in understanding of the autoimmune pathology of these disorders, especially the role of cytotoxic T cells, should eventually lead to more effective treatment options.