Abstract
Neuromyelitis optica (NMO), also known as Devic's disease, is a devastating autoimmune disorder of the central nervous system and is clinically characterized by inflammation of the optic nerve, spinal cord, and, less frequently, brainstem. The discovery of antibodies against aquaporin-4 (NMO-IgG/AQP4-IgG) has definitively distinguished this disease from multiple sclerosis.
AQP4-IgG testing is crucial in differential diagnosis that also includes magnetic resonance imaging of the brain and spinal cord. Typically, spinal cord lesions exceeding the length of three vertebral segments are found.
Changes in magnetic resonance imaging of the brain do not exclude the diagnosis of NMO: by contrast, they are relatively common and are fairly typical. Optical coherence tomography provides important information, and quantifies the involvement of the retina and optic nerve.
Neurologic deficit in this disease is a result of disease relapse, and it is therefore crucial to establish the diagnosis early and initiate an appropriate treatment that can change the unfavorable prognosis. The main therapeutic strategies include aggressive treatment of relapses (with high-dose corticosteroids or plasmapheresis), followed by chronic therapy (first choice drugs include azathioprine and rituximab).