Autoimmune and paraneoplastic neurological disorders

Abstract

Autoimmune neurology is a rapidly emerging subspecialty that encompasses the diagnosis and treatment of neurological disorders with an autoimmune (paraneoplastic or noncancer associated) basis. Recently, there has been a dramatic rise in discoveries of neural-specific autoantibodies and their target antigens. Laboratory testing is now available for most of these neural-specific autoantibodies, which serve as diagnostic markers directing the physician toward specific cancer types and assisting in therapeutic decision-making. Autoimmune neurology intersects with many traditional neurological subspecialties including cognitive behavioral neurology, movement disorders, epilepsy, neurooncology, neuromuscular disorders, autonomic neurology, and demyelinating disorders. Antibodies targeting intracellular proteins (nuclear and intracytoplasmic enzymes, transcription factors, RNA-binding proteins) serve as markers of neural peptide-specific cytotoxic effector T-cell-mediated injury and are generally poorly responsive to immunotherapy. In contrast, antibodies targeting plasma membrane proteins (neurotransmitter receptors, ion channels, water channels, channel complex proteins) may act as pathogenic effectors and elicit greater response to immunotherapy.