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. 2018 Oct 31;15(1):302.
doi: 10.1186/s12974-018-1335-x.

Chronic Relapsing Inflammatory Optic Neuropathy (CRION): A Manifestation of Myelin Oligodendrocyte Glycoprotein Antibodies

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Free PMC article

Chronic Relapsing Inflammatory Optic Neuropathy (CRION): A Manifestation of Myelin Oligodendrocyte Glycoprotein Antibodies

Haeng-Jin Lee et al. J Neuroinflammation. .
Free PMC article

Abstract

Background: Key clinical features of chronic relapsing inflammatory optic neuropathy (CRION) include relapsing inflammatory optic neuritis (ON) and steroid dependency, both of which have been reported among patients with myelin oligodendrocyte glycoprotein antibodies (MOG-Abs). We investigated the relevance of the presence of serum MOG-IgG with the current diagnostic criteria for CRION among patients with idiopathic inflammatory optic neuritis (iON).

Methods: Retrospective reviews of a database prospectively collated between 2011 and 2017 from the tertiary referral center for multiple sclerosis and neuromyelitis optica were performed. Sixty-four patients with iON, who did not meet the diagnostic criteria for multiple sclerosis, neuromyelitis optica (NMO) spectrum disorder with/without NMO-IgG, or acute disseminated encephalomyelitis and who had no symptomatic central nervous system (CNS) lesions other than on the optic nerve, were included from a cohort of 615 patients with inflammatory demyelinating diseases of the CNS. Fulfillment of the current diagnostic criteria for CRION, assay results for the serum IgG1 MOG-Ab, and characteristics of CRION patients with MOG-IgG were compared to those of non-CRION patients with MOG-IgG.

Results: Twelve iON patients fulfilled the current diagnostic criteria for CRION, 11 patients were positive for MOG-IgG, and one patient was borderline. Among the other 52 iON patients not meeting the criteria for CRION, 14 had relapsing disease courses and 38 had monophasic courses, of which MOG-IgG positivity were 0% and 29%, respectively. CRION patients with MOG-IgG had more relapsing disease courses (first steroid-dependent worsening/relapse in 2.3 months, range 0.4-7.0) and poorer optical coherence tomography outcomes at follow-up than non-CRION patients with MOG-IgG. However, patients in the two groups did not differ in terms of age of onset, sex, or steroid treatment duration after initial attack.

Conclusions: CRION, according to the current diagnostic criteria, is a relapsing optic neuritis associated with MOG-IgG. Among iON patients with MOG-IgG, the absence of steroid-dependent attacks in the early stages of the disease may predict a long-term non-relapsing disease course and a more favorable outcome.

Keywords: Chronic relapsing inflammatory optic neuropathy; Multiple sclerosis; Myelin oligodendrocyte glycoprotein antibodies; Neuromyelitis optica; Optic neuritis.

Conflict of interest statement

Ethics approval and consent to participate

The study was approved by the Institutional Review Board of Seoul National University Hospital in Korea (approval number H-1012-080-344). The study protocol followed the tenets of the Declaration of Helsinki. All subjects signed written informed consent for use of material and clinical information for research purposes.

Consent for publication

Not applicable

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Flow chart of patients with optic neuritis. Patients were classified according to the results of clinical diagnosis and serological status of myelin oligodendrocyte glycoprotein antibodies. *The MOG-IgG result of one chronic relapsing inflammatory optic neuropathy patient, who did not test positive for MOG-IgG, was borderline
Fig. 2
Fig. 2
MOG-IgG positivity of each subcriterion for chronic relapsing inflammatory optic neuropathy in relapsing optic neuritis. The positive predictive value of steroid dependency for MOG-IgG was 91.7%, which is the highest predictive factor of MOG-IgG positivity
Fig. 3
Fig. 3
MOG-IgG positivity in chronic relapsing inflammatory optic neuropathy (CRION, n = 12), recurrent idiopathic optic neuritis (RION, n = 14), and monophasic idiopathic optic neuritis (monophasic iON, n = 38). MOG-IgG positivity was 92% in patients with CRION, 0% in patients with RION, and 29% in patients with monophasic iON

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