A case of neuromyelitis optica (Devic's syndrome) in systemic lupus erythematosus. Clinicopathologic report and review of the literature

Abstract

An interesting case of systemic lupus erythematosus (SLE) is presented in which the clinical onset of myelopathy strongly suggested demyelinating disease. Later, intracranial hypertension developed on two occasions. Postmortem examination showed the systemic lesions of SLE. In addition, demyelinating lesions were found in the spinal cord and the optic nerve, the neuropathologic picutre of Devic's syndrome, a variant of multiple sclerosis. We question whether some of the cases showing necrotic myelopathy might have an autoimmune pathogenesis.