Objective: To characterize a distinct disorder of the optic nerve that
manifests as recurrent episodes of acute, monocular disc edema and ma
cular star formation. Design: Description of the clinical features and
laboratory findings in a group of patients with this disorder. Settin
g: Referral center. Patients: Seven patients (average age, 27 years) w
ho experienced from two to seven attacks of neuroretinitis at interval
s ranging from 1 to 10 years (average interval, 2.7 years). Interventi
ons: Treatment with corticosteroids (oral in seven patients; intraveno
us in two patients) and azathioprine sodium (one patient). Main Outcom
e Measures: Visual acuity, color vision, Goldmann perimetry, and fundu
s photography. Results: Visual loss manifested as disc-related field d
efects with variable loss of acuity. Regardless of treatment, patients
did not experience significant improvement of optic nerve function fo
llowing each episode. Results of tests for specific inflammatory origi
ns were normal. Conclusions: The presence of disc-related field defect
s, poor recovery, and tendency to recur distinguish this disorder from
the typical benign form of idiopathic neuroretinitis previously descr
ibed.