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Publisher Summary Neuromyelitis optica (NMO) is an inflammatory, immune-mediated disease that targets the optic nerves and spinal cord. Clinically, NMO is characterized by severe attacks of optic neuritis (ON) and/or transverse myelitis. The clinical onset is often abrupt and may produce a prodrome that can include headache and low-grade fever. Upper respiratory symptoms or gastrointestinal symptoms often precede the neurologic events. Usually, the first clinical manifestation of NMO is ON, which is seen in 56–76% of patients. The attacks of ON can be recurrent or simultaneously bilateral. europathologically, NMO lesions demonstrate perivascular and parenchymal leukocyte accumulation, hemorrhagic exudation, and finally, fibrosis and tissue necrosis. The underlying pathophysiology of NMO is not completely understood; however, there is strong evidence to support a role for humoral immunity. The differential diagnosis of NMO is quite extensive given the number of physiologic etiologies that can lead to ON, transverse myelitis, or a combination of the two. In constructing a differential for NMO, one must first include diseases falling into the spectrum of inflammatory demyelinating diseases. These include isolated ON, isolated transverse myelitis, acute demyelinating encephalomyelitis, Balo's concentric sclerosis, relapsing myelitis, and benign MS. |
