Paraneoplastic cerebellar degeneration

Abstract

Paraneoplastic neurological syndromes (PNS) are an extensive group of neurologic disorders that occur in patients with cancer and can affect any part of the nervous system. Paraneoplastic cerebellar degeneration (PCD) is one of the most common PNS. PCD can be associated with any cancer, although it most commonly occurs in the setting of small cell lung cancer, gynecologic and breast cancer, and Hodgkin lymphoma. PCD can be associated with autoantibodies binding to intracellular or plasma-membrane neural antigens. Most of the antibodies that can be associated with PCD can also be detected in other PNS, but each antibody predicts the presence of specifi c types of cancers. Thus the detection of a specifi c antibody should guide the cancer search. Early diagnosis of PCD is critical because it offer the unique opportunity to discover an occult and potentially treatable neoplasm. In this chapter the immunobiology and the clinical characteristics of PCD are presented.