Autoantibody-mediated neurological diseases

© 2020 Autoimmune neurological diseases are an expanding group of phenotypically characteristic, potentially treatable syndromes. A number of these conditions are mediated by autoantibodies. The last decade has identified distinctive clinical clues to help diagnose these diseases, and increased clinical experience regarding the effectiveness of immunotherapies. Awareness of these conditions among non-specialists – as well as neurologists and psychiatrists – is crucial as early immunotherapy improves outcomes. To discuss the most common antibody-associated neurological conditions, we focus on autoimmune myasthenic syndromes and neuromyotonia in the peripheral nervous system, and their rapidly expanding central nervous system counterparts, which include neuromyelitis optica and autoimmune encephalitis. We also discuss other related conditions, including stiff person syndrome and classical paraneoplastic neurological syndromes. We discuss strategies for identification, differential diagnosis, investigation and treatment. Although individually these conditions are uncommon, cumulatively they form a significant group of treatable diseases that frequently present to general medicine and are important for all physicians to recognize promptly.