Background: Devic's syndrome or neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system associated with optic neuritis, myelitis involving 3 or more contiguous spinal cord segments, and seropositivity for NMO-IgG antibody.
Case: A 22-year-old African American woman P1G0 at 22 weeks of gestation presented with weakness for 1 week. The weakness initially started in the left lower extremity and then involved the other extremities. She also had horizontal diplopia, temporal headache, and arthralgias. On physical examination, she had a discoid rash behind the left ear, muscle strength 3/5 in the upper and 0/5 in the lower extremities, and hyporeflexia. She had lymphopenia, a highly positive antinuclear antibody, and SS-A/ SS-B antibodies. The magnetic resonance imaging of the patient showed abnormal cord signal within brain and cervical and thoracic spine. Salivary gland biopsy revealed mild lymphoplasmacytic inflammation. The NMO antibody was positive. A diagnosis of Devic's syndrome associated with probable systemic lupus erythematosus (SLE) was made. She was treated with pulse IV solumedrol and plasmapheresis for 4 days. The patient improved clinically with treatment, but the fetus developed bradycardia, which was treated with IV dexamethasone.
Discussion and conclusion: There is a debate about the relationship of NMO with autoimmune disorders, such as SLE or Sjogren syndrome. If clinically evident SLE or Sjogren or positive autoantibodies coexist with NMO signs and symptoms, the neurologic process could be an independent association due to NMO or may be a vasculitic complication of the systemic disease. Our case highlights these issues, difficulty in making a correct diagnosis, and choosing the appropriate management. Further case studies are needed to explore these important issues.