SYNDROME OF NEUROMYELITIS OPTICA: A CASE REPORT WITH NECROPSY FINDINGS

Abstract

Neuromyelitis optica (Devic''s disease) was first described in 1870 by Albutt. The syndrome is characterized by symptoms referable to the optic nerves and spinal cord. The course and symptomatology will depend on the acuteness and on the order in which the various structures are involved. The question has not been resolved as to whether this is a disease entity or is an acute form of multiple sclerosis with a specific localization. The case report is of a 33-yr.-old male who had a rather acute onset with initial symptoms referable to the spinal cord followed by signs of optic tract involvement. There were sensory changes in the lower extremities followed in 3 weeks by optic tract involvement with progression in 3 days to almost complete amaurosis. The process in the spinal cord initiated by sensory changes, advanced rapidly and produced the picture of a complete transection of the cord with bilateral paralysis, total sensory loss and absence of sphincter control. Death was due to bulbar paralysis. Since the age of 9 yrs., he had had diabetes mellitus, which at times had not been adequately controlled. There was a history of a luetic infection for which treatment had apparently been adequate. The necropsy findings consisted of a diffuse demyelinizing process in the spinal cord and in the right cerebral hemisphere accompanied by a marked phagocytic glial reaction without gliosis. The spinal cord in addition revealed areas of softening and cavitation.