Objective:

To assess the demographic, clinical, and outcome data in patients with isolated optic neuritis (ON) across a large pediatric and adult cohort.

Background:

The association of ON with aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies has led to improved diagnosis and treatment. However, autoantibody-negative ON remains common.

Design/Methods:

A retrospective chart review of University of Utah and Primary Children’s Hospital patients with diagnosis codes ICD-9 377.30 (optic neuritis unspecified), ICD9 377.39 (other optic neuritis), or ICD-10 H46 (optic neuritis) and at least 2 ophthalmologic evaluations between February 2011 and July 2023 was conducted. Only isolated ON without other brain or spinal demyelinating lesions were evaluated. 

Results:

Of 111 patients (19 children, 92 adults), 9/106 (8.5% with testing) were AQP4-IgG positive, and 35/96 (36.5% with testing) were MOG-IgG positive. Sixty-seven had other-ON of which 2 were ultimately diagnosed with recurrent isolated ON (RION), 2 with chronic relapsing inflammatory ON (CRION), 2 with multiple sclerosis, 1 with collapsing response-mediator protein-5-ON, and 1 with seronegative neuromyelitis optica spectrum disorder, during a median follow-up of 15 months. Five of them started long-term immunosuppressive therapy. No RION or CRION had preceding infections; they had the first recurrence of ON within 3 months. At presentation, AQP4-ON (75%) and MOG-ON (48.8%) had more severe vision loss (<20/200) compared to other-ON (23.6%) (P<0.01.) At the last follow-up, AQP4-ON (25%) had more severe visual (<20/200) acuity compared to MOG-ON (0%) and other-ON (1.4%) (P<0.01). Pediatric cases compared to adults frequently had bilateral ON (36.8% vs. 16.3%, P=0.04), preceding infection (47.4% vs. 20.7%, P=0.01), and disc edema (100% vs. 64%, P<0.01).  

Conclusions: