Optic-spinal multiple sclerosis : clarification of pathogenesis, establishment of new disease entity and treatment

视脊髓多发性硬化症（OSMS）是一种顽固性神经系统疾病，其特征是复发性严重视神经炎和横切性脊髓炎，本质上与视神经脊髓炎（NMO）相同。本研究在发现OSMS/ nmo特异性自身抗体aquaporin 4 （AQP4）抗体的基础上，明确了其包括纵向延伸性脊髓炎（bbbb3椎节段）在内的临床特征，确立了AQP4抗体的致病性和星形细胞病这一新的临床实体，并证实了血浆置换和免疫抑制对该病的治疗效果。

Optic-spinal multiple sclerosis (OSMS) is an intractable neurologic disease characterized by recurrent severe optic neuritis and transverse myelitis, and is essentially identical to neuromyelitis optica (NMO). Based on the discovery of aquaporin 4 (AQP4) antibody, an OSMS/NMO-specific autoantibody, the present study clarified its clinical features including longitudinally extended myelitis (>3 vertebral segments), established pathogenicity of AQP4 antibody and the new clinical entity as astrocytopathy, and proved therapeutic efficacy of plasma exchange and immunosuppression in the disease.