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REVIEW Dean M. Wingerchuk, MD, MSc, FRCP(C) 1,2 diseases has been long debated. Many investigators have Background: Neuromyelitis optica (NMO) is an uncommon CNS considered it simply a severe variant of multiple sclerosis demyelinating syndrome often mistaken for severe multiple sclero- (MS), since an optic-spinal syndrome may occur if the burden sis (MS). Several clinical, laboratory, and neuroimaging character- of white matter lesions falls disproportionately on those istics may accurately distinguish NMO from MS early in the disease structures, whereas others have argued that it represents a course. distinct disease. Some clinicians promoted an intermediate Review Summary: NMO is usually a relapsing disorder associated position, suggesting that NMO is a form of acute dissemi- with early, severe, attack-related residual disability. It is associated nated encephalomyelitis peculiarly restricted to expression in with a highly specific antibody marker, NMO-IgG, which targets the optic nerve and spinal cord. water channel aquaporin-4. Revised NMO diagnostic criteria require optic neuritis, acute myelitis, and 2 of the following 3 characteris- tics: disease-onset brain magnetic resonance imaging (MRI) that is nondiagnostic for MS, contiguous spinal cord MRI lesion extending Most patients with neuromyelitis optica, over 3 or more vertebral segments, and NMO-IgG seropositive status. Symptoms referable
The Neurologist – Wolters Kluwer Health
Published: Jan 1, 2007
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