Introduction and overview of immunopathological mechanisms and future directions.

ABSTRACT

Paraneoplastic neurological disorders represent a significant part of the field of autoimmune neurology. Most neural autoantibodies discovered to date are associated with underlying malignancy and in that context are considered paraneoplastic antibody biomarkers. These autoantibodies can be divided into two major categories those that target intracellular proteins (not pathogenic) and those that target plasma membrane proteins (pathogenic). Disorders accompanied by the former are mediated primarily by neural peptide-specific cytotoxic T-cells, are commonly associated with cancer, and are poorly responsive to immunotherapy. Disorders accompanied by the latter represent antibody-mediated diseases and are generally more responsive to immunotherapy. Areas of significant unmet need in the context of paraneoplastic neurological disorders include novel therapeutic options, as FDA-approved therapies are lacking. This chapter provides a brief overview of immunopathological mechanisms and potential future therapeutic targets. Our contributing authors and their chapters are also introduced.