Tuesday MR with Big Lenie and Dr. Lewis: Neuromyelitis optica

A middle aged woman with a hx of severe bilateral glaucoma (legally blind), breast CA in remission who presented with acute proximal lower extremity weakness and subacute dysphagia admitted for statin-induced myositis due to elevated CK, eventually requiring intubation due to difficulty maintaining secretions, and found to have positive AQP4 Ab confirming the diagnosis of neuromyelitis optica.

  • NMO is an inflammatory CNS disorder characterized by severe, immune-mediated demyelination and axonal damage mainly affecting the optic nerves, spinal cord, brain and brainstem. It is distinguished from MS by the presence of AQP4 antibody.
  • Its incidence is 10 times higher in women than in men and more common in people of Asian, Hispanic, and African descent. Median age of onset is 32-41 years.
  • Main clinical features include optic neuritis, acute transverse myelitis, area postrema syndrome.
  • Evaluate with brain and spinal cord MRI, AQP4 Ab status, CSF analysis
  • Diagnosis: Pt has at least one core clinical characteristic; enhancements on brain and spinal cord MRI; positive AQP4 IgG serum (and often CSF) autoantibody status; and exclusion of alternative diagnoses.
  • Treatment: high dose IV methylprednisolone, plasma exchange, and immunosuppression treatment long-term disability and mortality rates are high
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