Autoimmunity plays a pivotal role in a wide range of neurological disorders, affecting the peripheral and central nervous system. Removal of autoantibodies by different therapeutic apheresis modalities (PEX, DFPP, IA) remains the major treatment option in certain neurological diseases (e.g., acute inflammatory demyelinating polyradiculoneuropathy, acute myasthenia gravis, resistant acute disseminated encephalopathy). Despite the introduction of novel immunosuppressive agents (IVIG, RTX), which have also improved the outcomes in autoimmune neurological conditions, PEX is of particular importance in acute severe episodes of the diseases, where rapid clinical improvement is needed. The aim of this chapter is to demonstrate the effectiveness of therapeutic apheresis in neurological pathology in the light of modern treatment options.