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

• Main Author: Sato,Douglas Kazutoshi
• Publication Date: 2014
• Other Authors: Callegaro,Dagoberto, Lana-Peixoto,Marco Aurélio, Nakashima,Ichiro, Fujihara,Kazuo
• Format: Article
• Language: eng
• Source: Arquivos de neuro-psiquiatria (Online)
• Download full: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2014000600445
• topic: neuromyelitis optica; aquaporin-4; myelin oligodendrocyte glycoprotein; antibody; myelitis; optic neuritis; differential diagnosis

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.