Neuromyelitis optica, also known as Devic disease, affects either the optic nerves or spinal cord, or both. It may be misdiagnosed as multiple sclerosis and vice versa. -ed.
Neuromyelitis optica, also known as Devic disease, is a CNS inflammatory disease. It is a devastating disease and leaves many patients with permanent vision loss and paralysis. Neuromyelitis optica is thought to be caused by abnormal activity of B cells, although studies suggest an important role of T cells as well. Most neuromyelitis optica patients have elevated levels of an IgG antibody to a water channel called aquaporin-4 (NMO-IgG). This antibody has been hypothesized to be disease-causing because it can fix complement and, thus, can cause tissue destruction. However, not all patients have the anti-aquaporin-4 antibody. Hence, other pathological mechanisms must exist that are responsible for tissue destruction. Patients presenting with neuromyelitis optica must be treated urgently with aggressive measures, as described later. Also, these patients must be recognized early in the disease course. Disability in neuromyelitis optica is relapse driven. Hence, early treatment at the onset of the first relapse is important. The authors review literature regarding the pathology of neuromyelitis optica and available treatment options, including their own observations. They explain the diagnostic criteria, treatment, and prognosis of neuromyelitis optica. New updates include a review of the sensitivity and specificity of current available lab tests, prevalence of coexisting autoimmunity, clinical presentations beyond optic neuritis and transverse myelitis, imaging characteristics of neuromyelitis optica lesions, mechanisms of pathogenesis, and current treatments.