Paraneoplastic cerebellar degeneration associated with noncutaneous Merkel cell carcinoma.

Abstract

Paraneoplastic cerebellar degeneration (PCD) is a rare condition that often heralds an underlying tumor. The syndrome is often associated with small cell carcinoma of the lung, breast cancer, gynecologic cancers, and Hodgkin disease. Treatment options are not well defined and improvement is often limited. Diagnosis can often be aided by detection of antibodies to various cerebellar proteins, including Yo, voltage-gated calcium channels (VGCC), and the Delta/Notch-like epidermal growth factor–related receptor (the target antigen of the Tr immune response). Current therapeutic goals are aimed at suppression or removal of immune-mediated activity, resection of primary tumor, and symptomatic treatment. We report a case of PCD with VGCC autoantibodies and noncutaneous metastatic Merkel cell carcinoma.