Neuroimmunological diseases: update].

Department of Neurology, Kinki University School of Medicine.

Nihon rinsho. Japanese journal of clinical medicine 2013 May

Autoimmune mechanisms are involved in the pathogenesis of several neurological diseases, called as neuroimmunological diseases. Neuromyelitis optica(NMO) has long been considered as a subset of multiple sclerosis but the primary target of NMO has recently been shown as a water channel aquaporin 4(AQP4) localized on the astrocytes. Anti-AQP4 anti body is the main pathogenic factor in NMO. Antibodies to gangliosides and ganglioside complexes are involved in the pathogenesis of Guillain-Barré syndrome and Fisher syndrome. Efficacy of high-dose chemotherapy with autologous peripheral blood stem cell transplantation for POEMS syndrome has been reported. Presence of anti-MuSK antibodies and anti-Lrp4 antibodies was shown in some of the patients with anti-AChR antibody-negative myasthenia gravis. Several novel autoantibodies have been reported in some neuroimmunological diseases affecting central nervous system. Nationwide surveys have recently been performed for several neuroimmunological diseases in Japan.