Devic syndrome: a diagnostic dilemma

Neuromyelitis optica (NMO, also known as Devic syndrome or Devic disease) is a rare clinical entity. Early recognition and prompt treatment can save patients from long-term disability, especially in themonophasic variant of disease. A 24-year-old African American female presented with high-grade fever for 1 day associated with frontal headache, photophobia, and 2 episodes of nonbloody vomiting. She had a history of nonitchy vesicular rash with sudden diminution of vision in the left eye 2 weeks ago. Over the next 24 hours, she developed progressively worsening weakness and numbness in her left arm and left leg, which later involved all limbs. Left eye vision was reduced to light perception with light and accommodation reflexes intact. Lumbar puncture showed lymphocytic pleocytosis with elevated protein. On day 2, neurological examination exhibited quadriparises with hypereflexia and clonus. Magnetic resonance imaging showed diffuse hyperintense signals in the spinal cord in cervical and lumbar regions. An assessment of neuromyelitis optica was made. Anti-NMO antibodies were negative. On day 3, she was intubated because of progressive dyspnea. Plasmapheresis resulted in rapid improvement in respiratory and neurological status. She was extubated on the second day and transferred to floor on day 4. Later, the patient was discharged.