Paraneoplastic syndromes

Historical note and nomenclature
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By Edward J Dropcho MD

The term "paraneoplastic" was originally applied to any disorder associated with neoplasia but not caused by direct tumor invasion or metastasis. In current usage, neurologic paraneoplastic disorders refer to nonmetastatic disorders that are not attributable to toxicity of cancer therapy, cerebrovascular disease, coagulopathy, infection, or toxic and metabolic causes.

Classic publications in the 1950s and 1960s delineated the clinical and pathologic features of several neurologic paraneoplastic syndromes (Eaton and Lambert 1957; Brain and Wilkinson 1965; Henson et al 1965; Corsellis et al 1968). Paraneoplastic disorders are far less common than nervous system metastases and are relatively rare compared to other nonmetastatic neurologic complications of systemic cancer, but they are worthy of consideration for several reasons:

  1. In most patients with paraneoplastic disorders, the neurologic symptoms are the presenting feature of an otherwise undiagnosed tumor, and patients see a neurologist first. It is the neurologist's task to identify the disorder as paraneoplastic and to initiate the appropriate search for the tumor.
  2. Among patients with a known diagnosis of cancer, the paraneoplastic syndromes are an important part of the differential diagnosis of neurologic dysfunction.
  3. Paraneoplastic disorders often cause severe and permanent neurologic morbidity.
  4. Prompt recognition of a paraneoplastic disorder maximizes the likelihood of successful tumor treatment and a favorable neurologic outcome.

A consortium of European centers published a set of criteria for diagnosing "definite" or "possible" paraneoplastic neurologic disorders based on the clinical syndrome, presence of onconeural antibodies, and tumor association (Graus et al 2004).

In This Article

Historical note and nomenclature
Clinical manifestations
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited