Paraneoplastic Neurologic Syndromes: Approaches to Diagnosis and Treatment

ABSTRACT There is increasing evidence that many paraneoplastic neurologic syndromes are immune-mediated. Because symptoms usually have an acute onset and precede the tumor diagnosis, the recognition of a neurologic disorder as a paraneoplastic syndrome is challenging. The poor response to treatment of many paraneoplastic neurologic syndromes has been attributed to the early, irreversible pathological changes involving the nervous system. This may account for the more frequent responses to therapy of some paraneoplastic neurologic syndromes affecting the peripheral nerves and neuromuscular junction compared with those that produce neuronal degeneration. This review focuses on the current therapeutic approaches to immune-mediated paraneoplastic neurologic syndromes, including prompt recognition of the disorder, detection and treatment of the associated tumor, and, in some disorders, immunosuppressive therapy.