An 8 Year Old Girl with Chronic Inflammatory Optic Neuritis (CRION): the Youngest Reported Case to Date (P7.015)

Abstract

OBJECTIVE: To report the youngest known patient with chronic relapsing inflammatory optic neuritis (CRION). BACKGROUND: CRION is a distinct clinical entity from other inflammatory demyelinating diseases including multiple sclerosis (MS), neuromyelitis optica-immunoglobulin G (NMO-IgG) spectrum disease, and idiopathic relapsing optic neuritis. CRION patients have increased steroid dependence, with progressive disease and severe visual disability. Three to 5[percnt] of ON patients relapse, increasing their conversion risk to MS or NMO-IgG, particularly with positive cerebrospinal fluid (CSF) oligoclonal bands (OCBs) and brain/spine demyelinating lesions, or NMO-IgG, respectively. Mean CRION onset is 36 years, with the previously youngest reported case a 14 year old girl. DESIGN/METHODS: Case report reviewing clinical, laboratory, and radiology records obtained from the Cleveland Clinic electronic medical record. RESULTS: SG was an 8 year old, normally developed, left handed girl presenting with bilateral eye pain, headache, photophobia, lethargy, and bilateral vision loss (< 20/200 OU). Fundoscopy revealed bilateral optic nerve pallor, edema, absent pupillary reflexes, and MRI revealed gadolinium-enhancing optic nerves bilaterally, and subcortical T2 hyperintense, enhancing lesions. She was diagnosed with ADEM and treated with steroids, with rapidly improving vision (OD 20/20, OS 20/40); CSF and baseline OCT exams were normal, but VEPs demonstrated bilaterally prolonged P100s. Upon steroid taper her vision rapidly worsened, prompting multiple high dose steroid courses, and IVIG x 2, with incomplete recovery of vision (scotoma and OD 20/20, OS 20/50). Her longest steroid-free period was 2 months. Repeat MRI revealed normal brain/spine, but on-going T2 hyperintense optic nerves and chiasm. Her RNFL thickness decreased OD 37 μm (44[percnt]), OS 35 μm (41[percnt]). NMO-IgG, ANA, and infectious serologies were negative on repeated testing. CONCLUSIONS: To our knowledge, this is the youngest reported case of CRION, a rare but progressively disabling demyelinating disease requiring further study in both pediatric and adult populations.

Disclosure: Dr. Rossman has nothing to disclose. Dr. Rensel has nothing to disclose.