We reviewed recent advances of therapeutic apheresis in neuroimmunological diseases including Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE) and myasthenia gravis (MG). The etiology of GBS and its related disease (CIDP, MFS and BBE) still remains controversial, but important advances have been made in the delineation of the mechanisms that lead to nerve damages. The infecting organisms induce humoral (antiganglioside antibody et. al.) and cellular immune responses that, because of the sharing of homologous epitopes (molecular mimicry), cross-react with ganglioside on the surface of peripheral nerves. Plasma exchange (PE) that removes these pathogenic humoral factors improves the early outcome in GBS. Also, high-dose intra venous immunoglobulin (IVIG), but not steroid therapy, has been shown to be at least as effective as plasma exchange. PE and IVIG carry individual advantages and disadvantages compared with each other. We need establishment of a standard protocol for the therapy of GBS based on clinical and molecular parameters. While PE is a fundamental therapy for GBS, PE can be applied for MG for a rescue therapy to treat the crisis of MG. In MG, extended thymectomy and high-dose corticosteroids therapies, in which symptoms improve after several weeks or months, are essential. Therefore, in myasthenia crisis, when patients necessitate effective therapy immediately, PE can be a rescue treatment with improvement occurring within days of treatment. But the beneficial effect of PE are temporary, lasting only weeks. The purpose of therapeutic apheresis is different from various neuroimmunologic disorders, and in future, based on clinical and molecular mechanism, we should use various treatments (PE, IVIG, corticosteroids, immunosuppressive agents and specific immunotherapy).