A 28-year-old woman has a 6-month history of relapsing-remitting episodes of optic neuritis, transverse myelitis, and area postrema syndrome (intractable hiccups and vomiting). MRI brain shows no demyelinating plaques typical of MS. She is anti-AQP4 antibody positive. The CORRECT diagnosis and recommended acute treatment is:
- A Neuromyelitis optica spectrum disorder (NMOSD); high-dose IV methylprednisolone and plasma exchange for severe attacks ✓
- B Multiple sclerosis; natalizumab
- C Neurosarcoidosis; oral prednisolone
- D Anti-MOG antibody disease; fingolimod
Explanation
Anti-AQP4 (aquaporin-4) antibody positivity with involvement of the area postrema (intractable nausea/hiccups — a signature of NMO), optic neuritis, and longitudinally extensive transverse myelitis confirms NMOSD. Acute attacks are treated with high-dose IV methylprednisolone (1 g/day for 3–5 days); plasma exchange is indicated for attacks that are severe or steroid-unresponsive. Maintenance with eculizumab, inebilizumab, or satralizumab (newer biologics) or rituximab/azathioprine reduces relapse risk. MS disease-modifying therapies (natalizumab, fingolimod) may worsen NMOSD.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.