Document details

Seronegative Neuromyelitis Optica Spectrum - The challenges on disease definition and pathogenesis

Author(s): Sato,Douglas Kazutoshi ; Callegaro,Dagoberto ; Lana-Peixoto,Marco Aurélio ; Nakashima,Ichiro ; Fujihara,Kazuo

Date: 2014

Origin: Oasisbr

Subject(s): neuromyelitis optica; aquaporin-4; myelin oligodendrocyte glycoprotein; antibody; myelitis; optic neuritis; differential diagnosis


Description

Neuromyelitis optica spectrum disorders (NMOSD) are characterized by severe optic neuritis and/or longitudinally extensive transverse myelitis, and some brain lesions are also unique to NMOSD. Serum autoantibodies against aquaporin-4 (AQP4) are detected in most cases of NMOSD. However, some patients with NMOSD remain seronegative despite repetitive testing during attacks with highly sensitive cell-based assays. The differential diagnosis of NMOSD is not restricted to multiple sclerosis and it includes many diseases that can produce longitudinally extensive myelitis and/or optic neuritis. We review the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSD with antibodies against myelin oligodendrocyte glycoprotein.

Document Type Journal article
Language English
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