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Paraneoplastic neurologic syndromes are heterogenous disorders that can occur in the setting of various types of cancer (1). Paraneoplastic neurologic disorders are commonly caused by immune-mediated mechanisms triggered by an underlying tumor and have to be distinguished from neurological symptoms related to direct tumor invasion, infection, vasculopathy, ischemia, metabolic disturbances, or treatment-related toxicities.

Abnormal antibody or T-cell-mediated responses can target any part of the central, peripheral or autonomic nervous system to cause a diverse range of neurological symptoms. In general, the incidence of paraneoplastic syndromes is less than 1% in the general cancer population, but may be more frequently seen in specific types of cancer, such as small cell lung cancer (SCLC), cancers of the ovary and breast, and thymoma.

The diagnosis of a paraneoplastic neurologic syndrome is primarily clinical. Classical paraneoplastic syndromes (Table 65-1) may develop before the diagnosis of cancer, in a patient with known cancer, or in a patient considered to be in cancer remission. While an extensive search for an underlying tumor is warranted in classical paraneoplastic syndromes, such as paraneoplastic cerebellar degeneration (PCD) or Lambert-Eaton myasthenic syndrome, it may be noteworthy that several neurologic syndromes designated as "paraneoplastic" may also be seen in non-neoplastic autoimmune diseases.


Since paraneoplastic syndromes often herald the ...

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