Devic's neuromyelitis optica: Case report [Neuromielitis óptica de Devic: Reporte de caso]

Abstract

Devic's neuromyelitis optica is an infrequent clinical condition characterized by the combination, rarely simultaneous, of monophasic or recurrent episodes of optic neuritis and longitudinally extensive transverse myelitis. It is autoimmune and demyelinating in nature, and due to its recurrent behavior, it was considered in the past as an unusual variant of multiple sclerosis (MS). However, characteristics in clinical presentation and particularly very recent discoveries have permitted to better characterized Devic's disease and to distinguish it from MS. Specially, it has been described the autoimmune substrate of Devic's disease, characterized by the presence of autoantibodies against aquaporin-4 (AQP4). Here we present the case of a 49-year-old Hispanic white woman whose first event was an optic neuritis that appeared 5 years before definite diagnosis could be done, when an event of an anti-AQP4-positive acute transverse myelitis occurred. To our knowledge, this is the first description in the Spanish literature of a typical neuromyelitis optica case with anti-AQP4 evidence. Here it is also presented the imagenological and clinical evolution of this paradigmatic case, with a brief review on the diagnosis and management of this entity.