Objective: To characterize a distinct disorder of the optic nerve that manifests as recurrent episodes of acute, monocular disc edema and macular star formation.
Design: Description of the clinical features and laboratory findings in a group of patients with this disorder.
Setting: Referral center.
Patients: Seven patients (average age, 27 years) who experienced from two to seven attacks of neuroretinitis at intervals ranging from 1 to 10 years (average interval, 2.7 years).
Interventions: Treatment with corticosteroids (oral in seven patients; intravenous in two patients) and azathioprine sodium (one patient).
Main outcome measures: Visual acuity, color vision, Goldmann perimetry, and fundus photography.
Results: Visual loss manifested as disc-related field defects with variable loss of acuity. Regardless of treatment, patients did not experience significant improvement of optic nerve function following each episode. Results of tests for specific inflammatory origins were normal.
Conclusions: The presence of disc-related field defects, poor recovery, and tendency to recur distinguish this disorder from the typical benign form of idiopathic neuroretinitis previously described.