Neuromyelitis optica spectrum disorders

The disease concept of Neuromyelitis Optica Spectrum Disorders(NMOSD) has undergone a significant change over the last two decades including the detection of Myelin Oligodendrocyte Glycoprotein(MOG) antibody in patients who are seronegative for aquaporin-4 antibody. Aquaporin-4 antibody positive NMOSD is now regarded as an immune astrocytopathy. Conversely, MOG antibody associated disease is known to target myelin rather than astrocytes, leading to an NMOSD syndrome with distinct clinical and radiological features. Incorporation of clinical features like area postrema syndrome, brainstem syndrome, diencephalic syndrome and cortical manifestations as core clinical characteristics into the revised diagnostic criteria has widened the clinical spectrum of NMOSD. With the development of these criteria, it is possible to make the diagnosis at an earlier stage so that effective immunosuppression can be instituted promptly for a better long-term prognosis. Newer therapeutic agents have been introduced for aquaporin-4 seropositive NMOSD disease; however, challenges remain in treating seronegative disease because of limited treatment options.