Paraneoplastic syndromes

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By Edward J Dropcho MD

Paraneoplastic neurologic syndromes can affect any part of the central or peripheral nervous system. These disorders are uncommon compared to other neurologic complications of systemic cancer, but affected patients often have severe and irreversible neurologic morbidity. Because most paraneoplastic syndromes are the presenting feature of the associated neoplasm, neurologists must be able to recognize and diagnose these syndromes promptly. In this clinical article, Dr. Edward Dropcho, of the Indiana University Medical Center, provides an overview of clinical features, autoimmune aspects, and management of patients with known or suspected paraneoplastic disorders.

Key Points

  • Neurologic paraneoplastic disorders are relatively rare but often disabling complications of a variety of systemic neoplasms, most notably small-cell lung carcinoma in adults and neuroblastoma in children.
  • Most, if not all, paraneoplastic disorders are believed to be caused by an autoimmune reaction against shared "onconeural" antigens, though the exact pathogenesis of most syndromes remains unclear.
  • Paraneoplastic disorders may present as any of a wide variety of clinical syndromes and are often a diagnostic challenge.
  • Prompt diagnosis and treatment of paraneoplastic disorders increases the likelihood of a more favorable neurologic outcome.