Neuromyelitis Optica (NMO)

Figure 1:  Sagittal T2 (top row left) and sagittal T1 post-contrast fat-saturated (FS) (top row right) images of the cervical spine demonstrate longitudinally extensive (long segment > 2 vertebral bodies in length) T2 hyperintense lesions in the spinal cord with ill-defined enhancement. Coronal T2-FS (bottom row left) and coronal T1 post-contrast FS (top row right) images of the orbits demonstrate an enhancing T2 hyperintense right optic nerve. The longitudinally extensive lesions in the cervical spinal cord, clinical and imaging findings of optic neuritis, and serology positivity for AQP-4 antibodies (NMO-IgG) are consistent with Neuromyelitis Optica.

AKA Devic’s Disease, Devic’s Syndrome, Devic’s Neuromyelitis Optica, Neuromyelitis Optica and its Spectrum Disorder (NMOSD)

Clinical Features

  • Peak Age: 32-41
  • Gender: F>M (5:1 to 10:1 F:M)
  • Etiology: Autoimmune mediated demyelination specifically targeting areas of increased Aquaporin-4 expression (spinal cord, periaqueductal gray matter, periventricular white matter). 
  • Epidemiology:
    • Africans, East Asians, and Hispanic predomination.
  • CSF:
    • Oligoclonal bands: negative
    • Protein: elevated
    • Cell count: Pleocytosis (20-80%); monocytes, lymphocytes and/or neutrophils.    
    • Diagnostic Criteria:
      • Complex and variable depending on result of IgG-AQP4 positivity.
      • Briefly, the presence of a single core clinical characteristic (optic neuritis, acute myelitis, area postrema syndrome, acute brainstem syndrome, n...