Paraneoplastic syndromes

Introduction
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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. Many patients have 1 or more of an ever-growing list of antineuronal or “onconeural” autoantibodies. 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 article, the author 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.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Anesthesia
References cited
Contributors