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

Arquivos de Neuro-Psiquiatria

Douglas Kazutoshi Sato¹  Dagoberto Callegaro¹  Marco Aurélio Lana-peixoto¹  Ichiro Nakashima¹  Kazuo Fujihara¹ 

关键词: neuromyelitis optica;    aquaporin-4;    myelin oligodendrocyte glycoprotein;    antibody;    myelitis;    optic neuritis;    differential diagnosis;    neuromielite óptica;    aquaporina-4;    glicoproteína associada ao olidendrócito;    mielite;    neurite óptica;    diagnóstico diferencial;   

DOI  :  10.1590/0004-282X20140032

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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