Acute Disseminated Encephalomyelitis Followed by Recurrent or Monophasic Optic Neuritis

Abstract

Acute disseminated encephalomyelitis (ADEM) is typically a monophasic demyelinating disease in young children. When relapses occur after ADEM, chronic diseases such as multiple sclerosis and neuromyelitis optica are suspected. A new clinical phenotype, ADEM followed by recurrent or monophasic optic neuritis in pediatric patients, has been proposed. We present the case of a young female with encephalopathy and polyfocal neurologic signs meeting criteria for ADEM, who subsequently had three relatively similar such relapses with new silent brain lesions. One year after her initial presentation, she developed relapses only affecting the optic nerves with substantial resolution of all other brain and spinal cord lesions on MRI. She did not have aquaporin-4 antibodies in the serum or cerebrospinal fluid. Similarly, oligoclonal bands were absent from the CSF during multiple attacks. Her clinical phenotype, laboratory findings, and imaging are consistent with ADEM followed by recurrent or monophasic optic neuritis. Collaborative outcome studies are needed to guide therapy and determine the prognosis of this rare disorder.