Published online by Cambridge University Press: 21 November 2024
Also called Devic’s disease, after French neurologist Eugene Devic who first discovered and described the disease in 1894, neuromyelitis optica (NMO) is a highly aggressive spectrum of neuroinflammatory disease characterized by recurrent attacks of myelitis and optic neuritis. Occurring more frequently in women than in men (3:1) and usually in adulthood, the optic neuritis of NMO can be bilateral or unilateral, and the myelitis associated with NMO is typically longitudinally extensive, stretching to at least three vertebral segments of the spinal cord continuously. Though the majority of neural injury of NMO is associated with the inflammation of the spinal cord and optic nerves, recent studies have shown that patients with NMO also often have involvement of the brain including the area postrema, which causes intractable nausea and vomiting, and the hypothalamus, which causes endocrinopathies. Also, lesions of the cerebral hemispheres can be seen, producing a host of possible symptoms from focal neurological deficits to seizures, though they can also be asymptomatic.
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