Neuromyelitis optica (NMO), sometimes called Devic disease or opticospinal multiple sclerosis [MS]) is a severe, relapsing, autoimmune, inflammatory and demyelinating central nervous system disease that predominantly affects optic nerves and spinal cord. Aquaporin-4 receptor (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibody testing is used for diagnosis and evaluation of neuromyelitis optica (NMO), acute myelitis, spinal cord lesions, autoimmune encephalitis, or NMOSD. Therefore, early diagnosis and initiation of NMO-appropriate immunosuppressant treatment is important to optimize the clinical outcome by preventing further attacks. Skeletal muscle abnormalities with hyperCKemia have been reported in a few NMOSD patients. Recent reports indicate focal retinal vascular attenuation, inner nuclear layer thickening and microcystic edema in some NMO patients.