Paraneoplastic nervous system syndromes

Recent progress in the understanding of the paraneoplastic neurologic syndromes has included further deliniation of the clinical syndromes and their treatment, attempts at standardization of the diagnostic nomenclature, investigations of pathogenetic mechanisms, and molecular characterization of the paraneoplastic antigens with implications as to their biologic relevance. Despite more than 30 years of investigation, the pathogenesis of these presumably autoimmune conditions remains unclear. Furthermore, no effective therapy has been identified for these conditions, with the possible exception of the opsoclonus-myoclonus ataxia and Lambert-Eaton myasthenic syndromes. Future investigations must focus on the disrupted genetic regulatory events involved in generation and propagation of the autoimmune response, antigen presentation and processing; target cell destruction, and consideration of alternative pathologic causes. Effective management strategies are most likely to develop from a better understanding of the disease pathogenesis, the development of animal model systems, and a creative look beyond the usual therapies employed in autoimmune conditions.