Optic neuromyelitis optica is an idiopathic, autoimmune, severe inflammatory disease that can lead to astrocyte death and demyelination of the optic nerve and spinal cord. Optic neuromyelitis optica is most commonly seen in Asia and is more prevalent in women. Pathogenesis: Specific antibodies targeting aquaporin 4 (NMO-IgG) cause disruption of myelin integrity around the optic nerve and spinal cord, resulting in severe visual impairment and neurological signs. The clinical manifestations of optic neuromyelitis optica are varied and include unilateral or bilateral optic neuritis, transverse myelitis optica, both of which can be anterior or posterior. Visual impairment in optic neuromyelitis optica is generally more common bilaterally. Neuro-ophthalmologic signs include painful eye rotation, central vision impairment, color vision disturbance, and loss of retinal nerve fiber layer. Other neurological signs include: ataxia, paraplegia, sensory disturbances, and bladder disorders. Diagnostic screening for optic neuromyelitis optica: 1. If the acute optic neuritis is bilateral, painless, and unremitting, or if the patient has a prior history of autoimmune disease or is Asian, NMO-IgG antibody titer testing should be performed; 2. Imaging, MRI may reveal abnormal cervical spine lesions while the cranial brain is normal or not consistent with multiple sclerosis; 3. Cerebrospinal fluid WBC is elevated, protein is increased, and approximately 75% oligoclonal zone band is negative. Diagnostic criteria for optic neuromyelitis optica: Main criteria: 1) unilateral or bilateral optic neuritis; 2) complete or incomplete transverse myelitis with acute T2 image lesions longer than three vertebral segments and low signal lesions on T1 image; 3) absence of nodular disease, vasculitis, SLE, SS, etc. All primary criteria must be present, and time intervals may be available. Secondary criteria: 1. Normal brain imaging or not meeting Barkhof’s MRI diagnostic criteria (nonspecific T2 lesion that does not meet Barkhof’s criteria; dorsal lesion of the medulla oblongata that may or may not continue with the spinal cord lesion; hypothalamic and/or brainstem lesion; linear abnormal lesion of the periventricular/callosal body that is not ovoid and does not show Dawson’s finger); 2. Serum or cerebrospinal fluid Positive for NMO-IgG/AQP4 antibodies. At least one of the secondary criteria must be met. The prognosis for optic neuromyelitis optica is poor, and the condition is more critical due to more frequent and recurrent cycles of remission and recurrence of optic neuromyelitis optica. Visual impairment is more severe and, often, accompanied by paraplegia. Treatment of optic neuromyelitis optica: 1. Acute attacks: high-dose methylprednisolone intravenous shock therapy (1 g/day for 3-5 days) combined with immunosuppressive agents (azathioprine). Refractory, offensive lesions may be considered for hemodialysis treatment. 2. Relapse prevention: long-term application of prednisolone, azathioprine, and rituximab immunomodulatory therapy has been shown to be beneficial.