Abstract
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 cellbased 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.
| Translated title of the contribution | Seronegative neuromyelitis optica spectrum - The challenges on disease definition and pathogenesis |
|---|---|
| Original language | Portuguese |
| Pages (from-to) | 445-450 |
| Number of pages | 6 |
| Journal | Arquivos de Neuro-Psiquiatria |
| Volume | 72 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - 2014 Jun |
Keywords
- Antibody
- Aquaporin-4
- Differential diagnosis
- Myelin oligodendrocyte glycoprotein
- Myelitis
- Neuromyelitis optica
- Optic neuritis
ASJC Scopus subject areas
- Clinical Neurology
- Biological Psychiatry