Current therapies for neuromuscular manifestations of paraneoplastic syndromes.
The neuromuscular manifestations of paraneoplastic disorders result in diverse syndromes that may also occur in patients without cancer. In general, treatment of these disorders is the same whether or not there is an underlying malignancy. However, when the disorder is believed to be paraneoplastic, the main concern should be prompt detection and treatment of the tumor, as this has been shown to offer the best chance for neurologic stabilization or improvement. The paraneoplastic neuromuscular disorders can be divided into two main categories: those that are directly mediated by antibodies and those that are believed to result from other immune-mediated mechanisms, including cytotoxic T-cell responses with or without association with specific antibodies. For disorders in which the antibodies are pathogenic, therapy is aimed at removing the antibodies. For the other disorders, adjuvant therapies are for the most part empiric and include a variety of immunosuppressant and immunomodulatory agents.
Department of Neurology, Section of Neuro-Oncology, 3 West Gates, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA.
Myrna R
MR
Rosenfeld
Josep
J
Dalmau
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000906:Antibodies / Q000378:metabolism; D001323:Autoantibodies / Q000378:metabolism; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009468:Neuromuscular Diseases / Q000209:etiology / Q000276:immunology / Q000628:therapy; D020361:Paraneoplastic Syndromes, Nervous System / Q000150:complications / Q000276:immunology / Q000628:therapy; D010951:Plasma Exchange
D000305:Adrenal Cortex Hormones; D000906:Antibodies; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents