Skip to Main Content


Paraneoplastic neurologic disorders (PNDs) are cancer-related syndromes that can affect any part of the nervous system (Table 94-1). They are caused by mechanisms other than metastasis or by any of the complications of cancer such as coagulopathy, stroke, metabolic and nutritional conditions, infections, and side effects of cancer therapy. In 60% of patients, the neurologic symptoms precede the cancer diagnosis. Clinically disabling PNDs occur in 0.5–1% of all cancer patients, but they affect 2–3% of patients with neuroblastoma or small-cell lung cancer (SCLC) and 30–50% of patients with thymoma.

TABLE 94-1Paraneoplastic Syndromes of the Nervous System


Most PNDs are mediated by immune responses triggered by neuronal proteins ectopically expressed by tumors (e.g., SCLC and other cancers) or as a result of altered immunologic responses caused by some types of tumors such as thymomas or lymphomas. In PNDs of the central nervous system (CNS), many antibody-associated immune responses have been identified (Table 94-2). These antibodies react with neurons and the patient’s tumor, and their detection in serum or cerebrospinal fluid (CSF) usually predicts the presence of cancer. When the antigens are intracellular, most syndromes are associated with extensive infiltrates of CD4+ and CD8+ T cells, microglial activation, gliosis, and variable neuronal loss. The infiltrating T cells are often in close contact with neurons undergoing degeneration, suggesting a primary pathogenic role. T-cell–mediated cytotoxicity may contribute directly to cell death in these PNDs and probably underlies the resistance of many of these conditions to therapy.

TABLE 94-2Antibodies to Intracellular Antigens, Syndromes, and Associated Cancers

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.