Neuromyelitis Optica - 07/11/17

Résumé |
Neuromyelitis optica (NMO), formerly known as Devic disease, is an autoimmune astrocytopathic disease characterized by transverse myelitis and optic neuritis. Most patients demonstrate a relapsing course with incomplete recovery between attacks, resulting in progressive disability. The pathogenesis involves production of aquaporin-4 antibodies (AQP4-IgG) by plasmablasts in peripheral circulation, disruption of the blood-brain barrier, complement-mediated astrocyte injury, and secondary demyelination. The diagnosis relies on characteristic clinical manifestations in the presence of serum AQP4-IgG positivity or specific neuroimaging findings, and exclusion of alternative etiologies. Current treatment involves aggressive immunosuppression with pulse-dose steroids during acute attacks and long-term immunosuppression for attack prevention.
Le texte complet de cet article est disponible en PDF.Keywords : Neuromyelitis optica, Longitudinally extensive transverse myelitis, Optic neuritis, Area postrema syndrome, Aquaporin-4
Plan
| Drs S. Patterson and S.E. Goglin have no financial disclosures and no conflicts of interest. |
Vol 43 - N° 4
P. 579-591 - novembre 2017 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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