A mouse model for paraneoplastic neurological syndrome: From anti-tumor immunity to autoimmunity targeting neurons

Abstract

Paraneoplastic neurological disorders are rare human autoimmune diseases that can affect any part of the central nervous system (CNS). They are triggered by an efficient immune response against a tumor that expresses an onconeuronal antigen also found in the brain or spinal cord. One example is the so-called “anti-Hu syndrome”, in which patients harbor small cell lung cancer that expresses the onconeuronal antigen Hu, which is largely expressed by neurons. Despite high circulating antibody titers found in patients, indirect but growing data points to a major role of cell-mediated immunity in the anti-Hu syndrome.