High mobility group box 1 (HMGB1) is a nuclear protein, and released from necrotic cells. Recently, It has been known that HMGB1 is released from monocyte/macrophage, neurons, and endothelial cells, and that HMGB1 is involved in sepsis, brain infarction, etc. We have reported that HMGB1 concentrations were elevated in cerebrospinal fluid (CSF) from patients with neuromyelitis optica (NMO) and multiple sclerosis (MS) and that the elevation was significant in CSF from NMO patients. Moreover, we have also reported that experimental autoimmune encephalitis (EAE) induced in C57BL/6 mice by immunization with myelin oligodendrocyte glycoprotein peptide (35-55) showed decrease of clinical and pathological severity by treatment with monoclonal anti-HMGB1 antibody. Thus, we think that HMGB1 is associated with pathophysiology in central nervous system in NMO and MS (especially in NMO), and that HMGB1 can be a target molecule for NMO and MS.