Paraneoplastic syndromes

Edward J Dropcho MD (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released March 16, 1998; last updated July 8, 2015; expires July 8, 2018

Overview

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.

Historical note and terminology

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).

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