Paraneoplastic Diseases

Paraneoplastic diseases, a group of neurologic disorders, are associated with malignancies in various visceral organs. Notably, they occur in the absence of metasis to the nervous system (the remote effects of malignancies). An estimated 1% to 3% of patients with various cancers develop paraneoplastic complications. These may present concurrently with the malignancy or may follow it, and importantly, may also precede it. Paraneoplastic diseases can be associated with almost any malignancy, but are most commonly associated with small-cell cancer of the lung and cancers of ovaries and breast. The diseases can

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Cerebellar degeneration associated with a hepatocellular carcinoma that metastasized to the pancreas and the lung. The cortex shows extensive Purkinje cell loss and Bergmann astro-cytosis (HE).

figure 1 1.48

Cerebellar degeneration associated with a hepatocellular carcinoma that metastasized to the pancreas and the lung. The cortex shows extensive Purkinje cell loss and Bergmann astro-cytosis (HE).

affect the central and peripheral nervous systems, retina, and neuromuscular junction.

Paraneoplastic diseases are immune-mediated; antibodies initiated by the tumor react with antigens expressed by both the tumor and neurons. The presence of specific antibodies in the serum and CSF of these patients points to a particular malignancy—a helpful clue to the clinician, especially when the primary tumor is not clinically evident. About 50% to 60% of patients carry antibodies.

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