Answer to CC #12

Case challenge # 12 presented a 27 year old black female with descending numbness, paresthesias, and blurry vision. The exam confirms her complaints, and lumbar puncture reveals a lymphocytic pleocytosis. The MRI findings, seen below, reveals inflammation of the right optic nerve, but no brain parenchymal findings.

NMO MRI

What is the most likely diagnosis?

 
The correct answer is Devic’s Disease (Neuromyelitis Optica)!
  • Optic neuritis and cervical myelopathy
  • Can be difficult to distinguish from MS, but is a distinct entity
  • CSF may have neutrophilic pleocytosis, but negative for oligoclonal bands in up to 85% of cases
  • NMO-IgG binds to Aquaporin-4 channel in the astrocyte foot process at the blood-brain barrier. Testing for this antibody is both sensitive and specific.
  • No controlled trials evaluating therapy – usually IV steroids and PLEX, followed by systemic immuno-suppresion to prevent recurrent episodes.

Case challenge #13 (aka the Parkland Files!) will be posted next week!