Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus

Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most com monly consisting of seizures, psychiatric disturbances, or focal central ne rvous deficits. This is a new case of neuromyelitis optica or Devic's syndr ome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromye litis optica during pregnancy in a patient with systemic lupus erythematosu s. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated wi th a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and th ree years later at the same thoracic level. Management by bolus administrat ion of a steroid and cyclophosphamide resulted in remission again. There ha ve only been around a dozen reports in the literature of patients who had b oth Devic's neuromyelitis optica and SLE. Magnetic resonance imaging is con tributive to diagnosis and therapeutic follow-up, showing spinal cord lesio ns with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated m ortality.