Neuromyelitis optica: Diagnosis and treatment

Neuromyelitis optica (NMO) is an inflammatory disorder of the central nervous system (CNS) characterized by optic neuritis and transverse myelitis. NMO is also known as Devic’s disease, after a case report by French neurologist Eugène Devic and his colleagues in the late nineteenth century. NMO has been considered a variant of multiple sclerosis (MS) and called opticospinal MS in Japan, where its prevalence is much higher than in Western countries. In 2004 however, an autoantibody, NMO-IgG (anti-aquaporin 4 antibody), was detected in the serum of patients with NMO, but not in patients with MS, indicating that NMO is independent of MS. Recent studies of NMO have contributed to a growing understanding of the disease that includes NMO spectrum disorders (NMOSD). In this chapter, we describe the clinical and laboratory characteristics of NMO/NMOSD and their treatments. While corticosteroids and/or plasmapheresis are treatments for NMO/NMOSD, novel therapeutic approaches are being developed through research elucidating the pathomechanisms of NMO.