Optic neuritis and recurrent myelitis in a woman with systemic lupus erythematosus.

BACKGROUND: A 38-year-old woman with systemic lupus erythematosus presented with headaches and bilateral hearing loss. Brain MRI was initially suggestive of small-vessel disease developing in the context of neuropsychiatric systemic lupus erythematosus. Several months later, the patient developed optic neuritis, followed by recurrent attacks of myelitis. INVESTIGATIONS: MRI of the spine revealed multifocal regions of myelitis affecting the cervical spine. Serological evaluation revealed the presence of neuromyelitis optica-IgG antibodies. MRI of the brain was nondiagnostic for multiple sclerosis. DIAGNOSIS: Recurrent myelitis and optic neuritis, occurring in the context of neuromyelitis optica (also known as Devic's syndrome). MANAGEMENT: The patient had recurrent attacks of myelitis despite treatment with pulse cyclophosphamide. After initiation of rituximab, the patient experienced symptomatic improvement and had no further attacks of opticospinal disease.